Roentgenographic

Changes in the Arthritides (Part I)

By Howard

L. Steinbach

RHEUMATOID

and Pamela

S. Jensen

ARTHRITIS

R

HEUMATOID ARTHRITIS (RA) is a subacute or chronic nonsuppurative, inflammatory polyarthritis affecting many joints and periarticular structures. The arthritis is most frequent in the peripheral joints, in which it is usually distributed almost symmetrically. Exacerbation and remission associated with systemic abnormalities characterize the disease. The minimal incidence in Great Britain is approximately 5% in females rising to about 16% in females between the ages of 65 and 74 yr. The disease is three times as common in females of all ages, and tends to be more severe than in males. The rheumatoid factor usually is present.

Roentgenographic

Features

The disease commonly affects the metacarpophalangeal and metatarsophalangeal joints, the proximal interphalangeal joints of the hands, wrists, toes, knees, elbows, ankles, and shoulders as well as hips, temporomandibular joints, cervical spine, and sacroiliac joints. As lesions become advanced they tend toward a symmetrical distribution. The following early changes appear at all sites. Soft tissues. Diffuse edema of the extremities is uncommon. Distention of the joint capsule by fluid and synovial thickening indicates active disease. This is seen on roentgenograms as a thickening of the deep soft tissues with displacement outward of the subcutaneous fat. Bursae at various sites, but particularly the prepatellar and olecranon bursae, become distended early. The gastrocnemiusP semimembranosus bursae which often communicate with the knee joint may distend or rupture extending for varying distances below or above the knee joint. This reduces the pressure and fluid volume in the knee joint leading to less severe damage of the intraarticular structures. When the hip is involved, the iliopsoas bursa, which often communicates with the hip joint, may become distended producing a palpable mass which may be seen on roentgenograms displacing the cecum, ureter, or other viscera. Rheumatoid nodules can be readily identified roentgenologically. Osteoporosis. Osteoporosis may be generalized due to debility, muscle atrophy from disuse or neurologic disturbance, or inactivity. There may also be loFrom the Department of Radiology, University of California School of Medicine; Department of Radiology, French Hospiral. San Francisco, Cal$ 94119; and the Department of Radiology, Yale University School of Medicine. New Haven, Corm. 06510. Howard L. Steinbach, M.D.: Chief of Radiology, French Hospital, San Francisco, Calif; Clinical Professor of Radiology. Stanford University School of Medicine,Stanford, Calif; Clinical Professor of Radiology and Medicine. University of California. San Francisco School of Medicine, San Franci.wo. Catty Pamela S. Jensen, M.D., Assistant Professor of Radio1og.v. Yale University School of Medicine. 333 Cedar St.. New Haven, Conn. 06510. 0 1975 by Grune & Stratton, Inc. Seminars in Arthritis and Rheumatism, Vol. 5. No. 2 (November). 1975

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calized osteoporosis associated with the joint disease. Osteoporosis is visible initially in spongy bone because of the more rapid resorption due to a larger surface area than compact bone. Thus, it is initially seen adjacent to the joints where cancellous bone is located and subsequently recognized as thinning of the cortex of the shaft secondary to endosteal resorption. The bones distal to the site of disease are also osteoporotic. This may occur as a single or multiple layers of Periosteal bone formation. bone adjacent to an inflamed joint. If associated with tenosynovitis it involves longer segments of the diaphysis. This usually develops early, often before erosive lesions become visible, and ultimately the periosteal bone becomes incorporated with the cortex. Destructive lesions begin at the margins of joints at the Destruction of bone. junction of articular cartilage, capsular and ligamentous attachments, and the synovial reflection. The earliest roentgen sign is an erosion of the cortical or subchondral bone producing a blurring or roughness resulting in the disappearance of the sharp bone outline. Cup-shaped defects often having overhanging margins develop later. The margins are irregular during active destruction and may become smooth with regression of the disease. Large pockets with smooth borders may appear some distance from the joint surface. These are often filled with synovial fluid in communication with the joint which may fill with fibrous tissue and, rarely, partially calcify. Extraarticular erosive lesions develop at the insertions of ligaments, tendons, and muscles. These are common at the plantar and dorsal surfaces of the calcaneus, crest of the ilium, lateral margin of the scapula, greater trochanter of the femur, inferior and distal surfaces of the clavicle and lower cervical spinous processes. Similar lesions develop along the posterior and dorsal surfaces of the second to ninth ribs about 1-5 cm from the costovertebral junction. The lesions may be bilaterally symmetrical or predominately one sided. These lesions may develop from pressure by the medial border of the scapula or inflammation at the attachment of the costocervicalis muscles. Narrowing of articular cartilages. This is evident by a narrowed space between bones compromising the joint. It is an early sign in the interphalangeal Fig. 1. Almost symmetrical distention of the proximal interphalangeal joint with fluid and synovial thickening. The erticular cartilage is of normal width. and there is no significant osteoporosis. A small irregular marginal erosive lesion is visible at the base of the middle phalanx. There is also distention of the distal interphelengeal joint which is asymmetrical. Superfrciel marginal erosion of the lateral surface of the middle phalanx is present. The marginal bone erosion may precede cartilage narrowing. Capsular distention indicates active disease. Fig. 2. Early RA of the middle Cnger with symmetric capsular distention, marginal erosion, and artitular cartilage narrowing of the proximal interphalangeal joint. The erosion is typical cup-shaped with an irregular border and overhanging edges. The articular cartilage is narrower adjacent to the destructive lesion which is unusual. Minimal involvement of the corresponding joint of the third finger with osteoporosis of all bones has developed. Fig. 3. Early RA in a 63-yr-old female. Capsular distention of the first and fifth carpometacarpal joints without other abnormalities is outlined by arrows. The soft tissue adjacent to the distal radius and ulna is thickened as a result of tenosynovitis. A shallow erosion of the ulnar styloid process is associated with periosteal bone formation where the fibers of the extensor carpi ulnaris tendon sheath insert. The radioulnar joint is narrowed end periosteal bone has formed along the adjacent ulnar surface. Fig. 4. Thickened soft tissue adjacent to the ulnar styloid process is associated with superficial irregular erosion of the cortical bone.

Fig. 5. Lateral view of a wrist demonstrating a localized erosive defect of the ulna with an irregular surface and overhanging margins. Fig. 6. Early RA of a foot which is characterized by periosteal bone formation along the shafts of the third and fourth proximal phalanges and the distal end of the fourth metatarsal bone. Osteopomsis is severe and of relatively recent origin as indicated by the irregular endosteal resorption of the proximal phalanges. Erosive lesions about the first metatarsophalangeal joint are due to rheumatoid arthritis. Fig. 7A and 6. Slight cartilage narrowing along the upper surface of the hip joint is associated with blurring of the adjacent subchondral bone of the femoral head. Two years later the cartilage destruction has progressed and involves the entire articular surface. Subchondral erosive lesions of the femoral heed and scetebulum have developed. In regions of contect of bone some sclerosis has formed. Early protrusion of the acetebulum is indiceted by the acetabular margin located mediel to the pelvic wall. 170

Fig. 8. The right hip joint resembles that of Fig. 78 but the acetabular protrusion is more advanced. More advanced protrusion is visible on the left side. The typical protrusion associated with rheumatoid arthritis as seen here is inward and upward displacement which distinguishes it from the idiopathic form where displacement is predominantly medially and associated with a coxa vara deformity. In advanced Still’s disease the femoral head usually is displaced laterally. Minimal erosive changes of the sacroiliacjoints as shown here or their absence is usual. The surrounding sclerosis and fusion seen in AS, psoriatic arthritis,or Reiter’s syndrome rarely occurs. Fig. 9. Typical bilateral hip changes are associated with a large sharply demarcated cyst-like lesion extending in the right femoral neck almost to the intertrochanteric region. At surgery this was seen to communicate with the joint and contained synovial fluid. 171

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Fig. 10A and B. Frontal and lateral views of the right knee with uniform cartilage narrowing involving hyaline cartilage of the femoral, tibial, end femoral petellar surfaces as well as the menisci. Erosive lesions extend from the posterior femoral condyles proximally beyond the margin of the artitular cartilage. A depression in the anterior surface of the femur above the patella is a mechanical defect produced by contact with a patella devoid of cartilage when the knee is extended. Fig. 11. Typical RA of the shoulder with uniform cartilage narrowing and subchondral erosive lesions of the humerel heed and glenoid. Almost complete destruction and atrophy of the rotator cuff. which normally occupies the space between the proximal humerus and acromion process, has produced an upward subluxation with the humerus coming in contact with the acromion. The disrup-

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joints of the hands and feet, the carpal, tarsal, knee, and hip joints. Initially the narrowing is uniform but in advanced stages with bone destruction, it becomes irregular. Deformities. Subluxations are multiple with diastasis of the acromioclavicular, sternoclavicular, distal radioulnar, and carpal joints. Dorsal dislocation of the distal ulna occurs. Subluxation of the odontoid and of multiple cervical vertebrae is common. Volar subluxation or dislocation and ulnar deviation of the fingers are common, as are subluxation, dorsal extension, and lateral deviation of the toes. Superior subluxation of the humeri associated with narrowing or destruction of the rotator cuff may be bilaterally symmetrical. A tendency to multiple subluxations with minimal destructive lesions exists in some patients. Rheumatoid spondylitis has features differentiating it from Still’s disease, ankylosing, psoriatic, and Reiter’s spondylitis. The lesions are almost exclusively in the cervical spine and are characterized by instability and dislocation. There is erosive arthritis of the apophyseal joints and destructive lesions of the corresponding disc in the vicinity of the neurocentric or Luschka joints at the posterolateral disc margin. The inflammatory process erodes the annulus posteriorly plus the adjacent bony surfaces. Eventually the entire disc narrows. This may be differentiated from osteoarthrosis by the absence of osteophytes, initial predominent posterior involvement of the disc, narrowing of the upper as well as lower cervical discs, subluxations, and erosions or fusion of the apophyseal joints. Osteoarthrosis is characterized by narrowing of discs predominantly below the fourth vertebral body, osteophytes, and subluxations that are minimal and limited to the lower cervical spine. In RA the atlanto-axial subluxation may be of two types. Most commonly the atlas is subluxated anteriorly which can best be seen on lateral roentgenograms with the neck in flexion. Vertical or backward subluxation is less common and is produced by bone and cartilage destruction causing the odontoid and even the body to pass into the foramen magnum. The apophyses fuse in the upper cervical spine in about 17% of rheumatoid patients. Erosions of the vertebral endplates are present in 15%. Hands. The most common lesions are in the distal ulna, the first, second, and third metacarpophalangeal joints, carpal, proximal interphalangeal, carpometacarpal, and radiocarpal joints. Soft tissue swelling of the extensor carpi ulnaris tendon adjacent to the distal ulna is often evident. Effusions and synovial thickening are well visualized within the proximal interphalangeal joints where the capsule is outlined by subcutaneous fat. The distention is usually symtion of the rotator cuff has also led to atrophy of the humeral tuberosities. Another common finding is the diastasis of the acromioclavicular joint and partial destruction of the distal inferior surface of the clavicle. The smooth cortical margin of the distal clavicle indicates inactivity and partial repair of the lesion. Fig. 12. Characteristic rheumatoid changes of the cervical spine consist of more extensive narrowing dorsalry of the intervertebral discs between the fourth, fifth. and sixth vertebral bodies. The discs are destroyed initially adjacent to the neurocentric or Luschka joints and in this case are associated with destruction of the adjacent surfaces of the fifth and sixth vertebral bodies dorsally. There are also erosions of the fifth and sixth apophyses. The typical disc narrowing and erosive lesions of the neurocentric joints in the absence of osteophytes exclude osteoarthrosis, ankylosing spondylitis. and Still’s disease. The posterior subluxation and destruction of the odontoid process as well as erosion of the tip of the seventh cervical spinous process can be present in any of the rheumatoid variants.

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Fig. 13A. 8, and C. Extensive destructive lesions with minimal reaction of adjacent bone may evolve in multiple joints as demonstrated in the hip, shoulder, elbow. Other joints had similar destructive lesions. This disease has been called arthritis mutilans and is more common in seronegative patients. This patient was a male with typical clinical findings of rheumatoid arthritis. He had less disability then would be expected from the roentgen appearance. This appearance is more common in females. Similar destructive lesions are seen with psoriatic arthritis or Reiter’s syndrome.

metrical, but may be asymmetrical. A lateral roentgenogram of the carpal region often shows considerable soft tissue thickening from capsular distention. Narrowing of the cartilage is common at the radioulnar joint followed in frequency by the intercarpal joints surrounding the capitate, scaphoid, trapezium, first three metacarpophalangeal joints, and middle two interphalangeal joints. Narrowing of the carpal joints is usually uniform, distinguishing it from osteoarthrosis which develops primarily in the joint between the trapezium and scaphoid. Other joints articulating with the carpal bones may be narrowed simultaneously.

Fig. 14. Fusion of the fifth proximal interphalangeal joint, also present in the other hand. is unusual in RA. When present it should suggest the possibility of psoriatic arthritis. Fusion of the carpal joints is common in all of the rheumatoid variants. The smooth articular margins of the other visible joints and absence of capsular distension indicate inactivity. Despite inactive disease, the severe osteoporosis persists, indicating irreversibility of severe osteoporosis. Fig. 15. Bilateral lymphedema of both hands, wrists, end forearms in a 56-y-old men is an uncommon finding. Typical almost symmetric lesions consist of uniform cartilage narrowing, marginal cup-shaped irregular erosions about all of the joints, but most obvious on the radial surface of the metacarpal heads, and ulnar displacement of the first proximal phalanges. Atypical findings are the multiple advanced erosive lesions of the distal interphalangeal joints and relative sparing of the carpal, carpometacarpal, radiocarpal, and radioulnar joints.

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Fig. 16A and B. Hand of a 46-yr-old male with severe chronic disease of all the carpal, carpometacarpal, radiocarpal. and radioulnar joints. The triangular cartilage has been destroyed allowing the distal ulna to contact the carpal bones. There is considerable destruction of the proximal carpal bones. The articular cartilage of the third metacarpophalgeal joint is narrowed uniformly. There is slight ulnar deviation of the fifth finger. The second through the fifth metacarpal bones am short with the third and fifth disproportionately so. This indicates that the disease had its onset before growth had ceased during the juvenile period, with the inhibition of growth resulting. (B) Roentgenogram obtained 5 yr later. Ulnar deviation of the second through the fourth fingers and volar subluxation of the second, third, and fourth proximal phalanges at the metacarpophalangeal joints has appeared. The proximal phalanx of the thumb is now displaced medially.

The ulnar styloid is eroded along its medial horder at the attachment of fibers from the extensor carpi ulnaris tendon sheath, at the fovea of the ulna which is a notch at the base of the styloid process representing the medial margin of the radioulnar joint, and at the tip of the styloid process where capsular fibers insert. Other common sites of erosion in the carpal region are the lateral surface of the scaphoid, trapezium, capitate, and medial surface of the triangular bone. The metacarpal heads are marginally eroded less frequently than the metatarsal heads and more often than the phalanges. This is particularly true of the first three metacarpophalangeal joints. This is best seen along the radial surface. A common site of erosion is the base of the proximal phalanx of the thumb beneath the sesamoid bone. Interphalangeal lesions are less common and involve mostly the middle finger and base of the distal phalanx of the thumb at its ventroulnar aspect where the flexor pollicis longus inserts. The erosions of the proximal interphalangeal joints appear in profile at the medial and lateral corners of adjacent phalanges and en face adjacent to areas of capsular attachment. Large cystic defects may develop, particularly in the central part of the distal radius, although they occur in other areas as well. Friction and compression defects take place where adjacent bones are forced into direct contact by complete destruction of articular cartilage or subluxations of a joint. These differ from erosions caused by inflammation in that their base is broad, smooth, and sclerotic with no overhanging edges. When the articular car-

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Fig. 17. Multiple marginal erosions of the third through fifth proximal interphalangeal joints and the second metacarpophalangeal joint ara visible. The margins of the defects are smooth indicating some repair. However, capsular distension of the involved joints indicates continuing inflammation. There is also tenosynovitis adjacent to the distal ulna with soft tissue swelling and ulnar styloid erosion. The typical erosion in the middle of the radius through which the line passes is common in RA. The carpal bones are rotated in a radial direction with the scaphoid nesting in the concavity of the radius and the lunate displaced toward the ulna. The normal refationship of the fingers persists as demonstrated by the vertical line which passes through the shaft of the radius and extends through the third metacarpal and finger. Compare the position of the carpal bones with Fig. 15 in which rotation is not apparent. Carpal rotation develops with many types of arthritis and is not specific for rheumatoid arthritis. The erosive lesions of the comers of the scaphoid and trapezium es well as the waist of the scaphoid are typical in location. They are adjacent to capsular and ligamentous insertions. Fig. 18. Mechanical friction defects are visible in the interphalangeal joint of the thumb. As usual. the base of the distal bone (distal phalanx) is saucer-shaped. expanded, and smooth. The adjacent proximal bone (proximal phalanx) tapers and also has smooth discrete borders. The space between the two bones is widened and probably filled with fibrous tissue and fibrocartilage. The proximal interphalangeal joints are irregular in contour with smooth borders and absence of capsular distension indicates absence of inflammation.

tilage of one bone is in contact with the periosteal surface of another bone due to subluxation or dislocation, the defect is in the latter bone. In the finger joints the base of the distal bone is usually eroded and sometimes expanded, and the adjacent proximal bone is tapered. Many deformities of the hand distinguish RA from similar arthritides. Ulnar deviation and volar subluxation of the proximal phalanges may occur simultaneously or independently and are particularly characteristic. Similar deformities develop with Jacoud’s arthritis, systemic lupus erythematosus (SLE), and progressive systemic sclerosis (PSS), but it is unusual with Reiter’s syndrome, psoriatic arthritis, ankylosing spondylitis (AS), and Still’s disease. It may

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Fig. 19A and 9. A soft tissue mass was palpable in the right lower quadrant and on this excretory urogram it displaces the right ureter medially. Uniform narrowing of the articular cartilage of the hip is visible. (B) A barium enema shows displacement of the cacum and ileum upward by the mass. Surgery exposed a distended iliopsoas bursa communicating with the diseased hip joint. The iliopsoas bursa communicates with the hip joint in about one-third of humans and may do so on either side. A distended bursa may develop with any type of arthritis involving the hip in which excess synovial fluid is formed. Fig. 20. Arthrogram of the knee demonstrates passage of contrast material into a small popliteal cyst which has ruptured forming a large pocket that extends to the middle of the distal extremity. A valve is present at the communication between the knee joint and the gastrocnemius-semimembranosus bursa allowing the fluid to pass from the joint into the bursa and cyst but not in a revarse direction. This decompresses the joint and is usually associated with minimal cartilage destruction and erosive changes within the joint.

take place in the absence of cartilage narrowing or marginal erosions and often begins with the fifth finger subsequently involving the others in sequence. Rotation of the carpal bones with the proximal row moved medially and the distal row laterally is common in RA and other arthritides. A more specific finding is diastasis of the joint between the scaphoid and lunate, or less commonly, between the scaphoid and trapezium. Diastasis of the radioulnar joint is common and, with destruction of the triangular cartilage of the wrist, the ulna may be subluxated distally making contact with the triangular or lunate bones.

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The proximal phalanx of the thumb is frequently displaced toward the ulna and the distal phalanges may be angulated in the opposite direction. Hyperflexion of the distal interphalangeal joints may be associated with hyperextension of the proximal interphalangeal joints, or the two may be reversed. Bony ankylosis is frequent in the carpal joints, but infrequent in the interphalangeal joints and rare in the metacarpophalangeal joints. The possibility of psoriatic arthritis should be suspected when the fingers are ankylosed. Arteriograms show occlusive lesions of the digital arteries in some patients. These lesions are more common in males and are usually at the level of the proximal phalanx. Collateral vessels through arcuate arteries usually are extensive. Calcification of vessels about the ankles or hands and wrists may develop particularly in patients treated for long periods with corticosteroids. STILL’S DISEASE (JUVENILE

RHEUMATOID

ARTHRITIS)

The criteria for the diagnosis of Still’s disease (JRA) proposed by Ansell and Bywaters require that: (1) the arthritis begins before the age of 16 yr; (2) at least four joints are involved for a period of 3 mo with either pain and swelling, pain and limitation of motion, or limitation and swelling; (3) if fewer joints are affected, biopsy of the synovium should show changes compatible with rheumatoid arthritis; and (4) other diseases are excluded. By this definition, other diseases, such as arthritis associated with psoriasis, ulcerative colitis, regional en-

Fig. 1. There is distension of the capsule with fluid, cartilage narrowing, and subchondral erosion of the lateral femoral condyle and tibia1 plateau on the right side. Osteoporosis is present. The femoral, tibial, and fibular epiphyses are enlarged and have accelerated maturation compared to the normal left knee.

Fig. 2A and B. (A) Anterior-posterior projection of both knees; (B) Lateral projection of the right knee. Distension of the joint capsule and right suprapatellar bursa due to fluid and synovial thickening. The bones are osteoporotic with preferential involvement of the epiphyses. The distal femoral and proximal fibular epiphyses are slightly enlarged. A characteristic deformity of the patella consisting of a concave inferior border is present. This is also seen in hemophilia and paralysis.

Fig. 3. There is extreme narrowing of the femoral shaft with relative expansion of the distal femoral metaphysis and epiphysis. The articular surface of the apiphysis is not widened pmducing a bulbous contour. Similar, less marked changes are present in the proximal tibia. The articular cartilage has been almost completely destroyed and the subchondral bone is eroded irregularly. Severe osteoporosis is present. Fig. 4. There is severe osteoporosis of the right femur and tibia which preferentially involves the epiphyses. Remaining trabeculaa along the lines of stress am thickened. The lateral erticular cartilage is narrowed. There is e transverse fracture of the distal femur without displacement. This is the most common site of fracture in patients not receiving corticosteroid medication. In those receiving steroids. the vertebrae are most often the site of fracture. 180

Figs. 3 and 4.

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Fig. 5. There is widening of the left hip joint due to cartilage thickening which appears in the early stages of Still’s disease. Subsequently the cartilage becomes thin. Fig. 6. This is the typical appearance of the hip in Still’s disease, but also occurs with hemophilia and paralysis or severe muscular weakness in childhood. The acetabulum is shallow and slopes more than in the normal hip. The femoral head is displaced laterally. The femoral head is underdeveloped medially end the medial portion of the epiphyseal cartilage is inclined downward. The lateral margin of the femoral head protrudes beyond the femoral neck. There is a valgus deformity of the femoral neck, enlarged lesser trochanter, and small greater trochanter. The subchondral surfaces of the femoral head and acetabulum are irregular and sclerotic. The bones of the obturator ring are slender. The obturator foramen is large.

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Fig. 7. Osseous synostosis of the hip is an uncommon complication of Still’s disease. Fig. 8. With the neck extended the odontoid process is in normal position. The articular facets of the second, third, fourth, and fifth vertebrae have fused. The third intervertebral disc is slightly narrow and the fourth and fifth vertebral bodies have diminished height. The spinal canal appears wide. Fig. 9A and 8. (A-neutral; S-flexion). With the cervical spine in neutral position the position of the odontoid is normal. With flexion the odontoid is subluxated posteriorly due to laxity or rupture of the transverse ligament. The margin of the odontoid process is smooth.

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Fig. 10A and 6. (A) Right hand and wrist of a L-yr-old girl with bilateral, symmetric arthritis of hands and wrists. The articular cartilages of the carpal joints are narrowed uniformly. The subchondral surfaces of the carpal bones are irregular, have angulated margins and are deformed. The adjacent margins of the radius and ulna are angulated toward each other. The bone age of the carpal bones is markedly accelerated. The carpal bones have rotated. The epiphyses of the metacarpal heads and proximal phalanges have angulated margins. (6) The same patient at age 7 yr. Growth of the carpal bones has increased with continued deformity. The articular cartilage is thicker. Discrete marginal erosions are not present. The deformity of the adjacent radial and ulnar margins has increased. The third and fourth metacarpals are now short because of diminished endochondral ossification and premature fusion of the distal epiphyses is impending. The involved epiphyses are deformed with sharp angulations laterally and medially replacing the usual rounded margins.

teritis, acute dermatomyositis (AD), PSS, SLE, and AS are excluded. Patients partially fulfilling the criteria often develop symptoms and signs of these other diseases. Polyarthritis characteristic of adult seropositive RA develops in a small number of children. These children are usually older, often reporting the onset of their disease in the second decade. They characteristically develop a positive rheumatoid factor and widespread, progressive arthritis. Rarely do nodules of the adult rheumatoid type and digital vasculitis appear in children. The incidence has been tabulated at 0.06% in school children studied over a 5yr period. The age of onset of seronegative arthritis is earlier than that of acute rheumatic fever, usually between the first and third years of life and only occasionally before the age of 1 yr. The disease is about twice as common in girls. Familial occurrence has been noted. Some form of polyarthritis is present in a substantial number of relatives of the patients with juvenile rheumatoid arthritis. AS is more common in male relatives, sacroiliitis occurs three times more often than in controls. Erosive arthritis of the hands has been noted more often than expected in female relatives. One family has been described in which five of seven siblings had JRA. The disease may begin as an acute illness with high fever, rash, and arthralgia; as an acute polyarthritis with varied systemic manifestations; or as arthritis of one or two joints, usually affecting the knee or ankle. Iridocyclitis or pericarditis may be the presenting feature. Lymphadenopathy and splenomegaly are com-

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Fig. 11. Swelling of the right wrist in a 29-mo-old girl is associated with periosteal new bone formation along the radial side of the third metacarpal diaphysis. Bone maturation is normal. Fig. 12. The right hand of a 17-yr-old who had the onset of arthritis at the age of 3 yr. There is no evidence of inflammatory disease or bone destruction. The second, third, and fourth distal phalanges are short due to premature epiphyseal closure. The fourth metacarpal bone is short, and there is an osseous synostosis of the capitate and third metacarpal bone. The carpal bones are rotated.

mon. Leukocytosis with a high percentage of polymorphonuclear cells may simulate leukemia. The joints most commonly involved are the knees, ankles, carpals, feet, hands, cervical spine, and hips in that order. In contrast to adult RA, the small joints of the hands and feet are less often involved and the cervical spine and hips are often affected early. Onset of the disease with involvement of a single joint occurs in about 33% of the patients. The arthritis may remain confined to one joint or may subsequently involve others. Polyarthritis occurs in 33% of patients, usually developing within the first year. The cervical spine is involved clinically in about 75% of patients but roentgenologically in about 33%. This occurs earlier than in adult RA or other variants. Disturbances of growth and maturation are frequent and may be generalized or localized to regions of joint involvement. The bone age is only minimally retarded even when severe deficits in height are present. The appearance and fusion of epiphyses is accelerated frequently about diseased joints. This accelerated maturation also affects uninvolved epiphyses distal to the arthritic joints. Although growth of a bone may be accelerated initially, early fusion of the epiphyseal growth cartilage results in shortening. The most common localized growth disturbance is brachydactyly usually seen in older children. Fractures are frequent with Still’s disease and commonly involve the femur in

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girls whose disease began before 5 years of age. Children develop fractures, usually of the spine. Roentgenographic

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Features

The lesions differ from adult RA in degree, are milder, less destructive, and more readily repaired by the regeneration of tissues. There is a tendency for involvement of larger joints, a limited number of joints, and initial asymmetry. Narrowing of cartilage and erosion of bone develop late in the course of the disease. Osteoporosis of the acute type with mottling of cancellous bone is common but transient. Severe osteoporosis may appear in chronic disease, with diffuse bone reduction and thinning of the cortices. Initially, osteoporosis may be predominant in the subchondral bone or beneath the epiphyseal growth cartilage. Periosteal bone is formed more frequently in children than adults. It appears early and may be limited to the site of a capsular attachment or it may extend along the shaft of a bone where inflamed tendon sheaths insert. The new formed bone may become incorporated in the cortex thereby thickening it or concomitant resorption of the endosteal surface may produce a wide bone with a large medullary cavity and a thin cortex. In other cases the bone is narrowed because of constriction of the medullary cavity, although the length of the bone and thickness of the cortex are relatively normal. Cervical spine abnormalities are often detected by the second year of the disease, beginning with loss of the normal lordosis, narrowing and irregularity of apophyseal joints, failure of growth of vertebral bodies and atlanto-axial subluxation. The apophyseal joints may fuse and occasionally the intervertebral discs narrow and adjacent vertebral bodies fuse. The most common location for apophyseal lesions is between the second and third vertebrae followed by the third to fourth and fourth to fifth vertebrae. The joints between the fifth and seventh cervical vertebrae usually are normal. In patients with cervical spine disease sacroiliitis usually develops. Lesions affecting the spine between the cervical region and sacroiliac joints are uncommon and when they do appear, are minimal. Disease of the sacroiliac joints affects from 7% to 24% of patients. (AS occurs in children and is considered a separate entity). The sacroiliac lesions are mild and usually consist of erosions of the iliac surface without adjacent sclerosis or synostosis. Lesions of the hips differ from those in adults because of early onset, minimal destruction, and lateral subluxation. Growth disturbance results in a large lesser trochanter and small greater trochanter associated with a valgus deformity of the neck. The inner aspect of the femoral head is less developed and the lateral part may overlap the neck. Obliquity of the acetabular roof increases. Protrusion of the acetabulum is uncommon in Still’s disease, but common in adult RA. Osteoarthritis may develop in affected hip joints, usually after the age of 15. The knees may have a characteristic appearance with articular cartilage narrowing and enlargement of the distal femoral epiphyses associated with narrowing of the adjacent shaft. The femoral condylar enlargement, as with other epiphyses, is usually greater proximal to the joint surface producing a bulbous appearance. The inferior surface of the patella is often underdeveloped and its surface broad rather than tapered as in the normal patella. In some instances it is

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concave resembling the deformity seen in hemophilia and paralysis such as occurs with poliomyelitis. We have seen identical deformities of the fingers, hips, and knees including cartilage narrowing in patients with hemophilia, poliomyelitis, and Still’s disease. Still’s disease of the hands and wrists differs from adult RA in the following respects: cartilage narrowing and marginal erosions are late in appearing; the wrists, carpal, and distal interphalangeal joints are more commonly affected; periosteal bone appears more frequently about the proximal phalanges and metacarpals and in addition to capsular distension is the only roentgen finding of importance during the first year of the disease. It usually subsides within the year. Narrowing of cartilage and destruction of bone may develop early in the carpals and frequently is extensive. Many of the carpal bones may be destroyed completely, but more often, destruction is followed by ankylosis which may extend to the carpometacarpal and radiocarpal joints. Dense metaphyseal bands and metaphyseal rarefaction occasionally develop particularly in the distal radius and ulna. Calcification about joints and vessels has been described. In some cases this may follow corticosteroid medication. There may be generalized growth disturbances as a result of the systemic or chronic nature of the illness. Height is often temporarily decreased, but, as the disease regresses, growth resumes and bone length approaches normal levels because epiphyseal fusion is not associated with this generalized growth retardation. In the vicinity of joint disease, size and maturation of bones may be accelerated initially. Eventually growth ceases and the epiphyses and metaphyses fuse prematurely producing retarded growth. The most severe deformities arise when the onset of the disease is early and the disease is prolonged. Brachydactyly is the most common deformity. Often the epiphyses of the fingers grow more rapidly than the metaphyses, producing an expansion of the end of the bone. This is seen in the distal and proximal interphalangeal joints. Growth of metacarpals and phalanges diminishes and epiphyses fuse early in selected joints not affected by the inflammatory process if the disease is in a proximal joint. Arthritis of a wrist may be associated with shortening of uninvolved metacarpals and phalanges-the fourth, fifth, and third metacarpals, respectively, are most commonly affected.

Fig. 13A and S. The hands of a 21 -yr-old female whose carpal bones have fused with each other and with the middle three metacarpals and radius. Volar subluxation of the proximal phalanges, as shown here, is uncommon in Still’s disease. The short ulna and narrowed radius, and the ulna. metacarpal, and proximal phalangeal diaphyses with normal cortical thickness. but thin medullary canals indicate onset of the disease in childhood. Fig. 14. The right hand of a 19-yr-old female who had the onset of arthritis relatively late. The disease was active at the time the roentgenogram was obtained. There has been considerable destruction of the carpals. distal radius, and ulna with carpal synostoses. The subluxation of the proximal phalanx of the thumb and destructive lesions of the fourth and fifth proximal interphalangeal joints resemble the lesions of adult AA. However, distal interphalangeal joint erosions of this magnitude are uncommon in the adult disease and the short third metacarpal bone indicates that the disease began before the bone had achieved its normal length. Fig. 15. A rare abnormality of the wrist in children with Still’s disease is the development of radiolucent bands in the distal metaphyses of the radius and ulna. The lesions appear destructive and irregular. They involve the zone of provisional calcification and to a varying extent the underlying metaphyseal bone. Superffcially they resemble rickets but are limited to the radius and ulna, are not as svmmetrical, and are less uniform.

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The distal ulna is often short. If this shortening results from wrist disease, the disturbance of growth may affect primarily the side adjacent to the radius producing disproportionate shortness and sloping of its radial side. The adjacent radial epiphyseal cartilage may be affected also resulting in tilting towards the ulna. ANKYLOSING

SPONDYLITIS

Ankylosing spondylitis (AS) is a progressive inflammatory disease of the spine and sacroiliac joints, with less frequent involvement of the peripheral joints. It is usually self-limiting and has been reported in 0.08% of the general population. The onset of the disease usually occurs between the age of 16 and 40 yr, most commonly between 17 and 25 yr. It may begin in childhood initially appearing as Still’s disease, with the spondylitis developing subsequently. It is predominantly a disease of males, with the ratio cited as from 4: 1 to 10: 1. Most patients show HLA27 antigens in their serum. Roentgenographic

Features

The roentgen appearance is similar at all sites of involvement during the various stages of development of the disease. In almost all patients the sacroiliac joints are affected. A detailed account of the sacroiliac joints will illustrate features found at other regions. Initially, the subchondral compact bone on the iliac side of the sacroiliac joint loses its sharp margin and becomes blurred. The normal articular cartilage on this side of the joint is thinner and fibrous which may account for the earlier and more extensive bone changes laterally. Irregular lesions develop producing a ragged border creating the illusion of widening of the joint. This is probably secondary to irregular progression of endochondral ossification, with replacement of the subchondral bone plate and calcified cartilage by porous trabecular bone. Simultaneously, the adjacent bone increases in density because of the appositional deposition of lamellar bone and the formation of woven bone at the capsular attachment. A similar radiographic appearance is subsequently seen on the sacral side of the joint. As the disease becomes quiescent the sclerotic bone assumes a normal density. Obliteration of the joint is seldom complete. Ankylosis of the capsule in thin shells of bone anteriorly and posteriorly may be complete. The disease is approximately symmetrical in the two sacroiliac joints. Asymmetry has been described in the literature, but many of the cases may have been psoriatic arthritis, Reiter’s syndrome, or Still’s disease, in which asymmetry or unilateral involvement is common. Lesions similar to those in the sacroiliac joints develop, usually later, at other sites about the pelvis adjacent to regions where ligaments or tendons insert. These include the iliac crests, ischial tuberosities and spines, symphysis pubis, and greater and lesser trochanters. Similar abnormalities are seen subsequently about the shoulder at the adjacent surfaces of the acromion and clavicle, coracoid process, and greater tuberosity of the humerus. Other sites include the sternal angle, sternoclavicular joint, costovertebral and costotransverse joints, transverse and posterior spinous processes and the dorsal and plantar surfaces of

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Fig. 1A and B. Anteroposterior view of the sacroiliac joints reveals sclerosis of the ilium adjacent to the sacroiliac articulations. The disease involves the lower two-thirds of the bone bilaterally and is more extensive inferiorly. The sacroiliac joints reveals sclerosis of the ilium adto two-thirds of the space between the sacrum and ilium appear widened, an illusion produced by the resorption of the subchondral bone plate and calcified cartilage. and replacement with porous trabecular bone. Above the true sacroiliac joint the bone surfaces appear normal. The process appears symmetrical. fBj Oblique view of the left sacroiliac articulation best demonstrates the apparent widening of the joint. The sclerotic process is occurring at the insertion of the sacroiliac ligaments into the ilium and extends to its upper border.

the calcaneus. The lesions tend to be bilaterally symmetrical, whereas similar lesions in Reiter’s syndrome and psoriatic arthritis are more often asymmetrical. The lesions consist of superficial erosion of the cortical surface and sclerosis of the adjacent bone. Swelling of the adjacent soft tissue is present during the inflammatory phase. As the inflammation subsides the erosion fills in with bone

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Fig. 2A and 6. Lateral view of the second, third, and fourth lumbar vertebral bodies shows early resorption of the upper anterior comer of the fourth vertebral body with underlying sclerosis. There is less resorption of the corresponding upper and lower corners of the third vertebral body. The bodies have a concavity anteriorly. fB1 Four years later there has been considerable further resorption of all visible anterior comers associated with increasing density of the underlying bone. Because of the bone removal at these corners the anterior surfaces of the vertebral bodies are no longer concave. The intervertebral disc between the third and fourth vertebrae demonstrates some interval narrowing. Fig. 3. Lateral lumbar spine showing corner changes of the third, fourth, and fifth vertebral bodies. Early ossification is extending from the upper anterior corner of the fifth vertebral body through the anterior portion of the intervertebral disc to the inferior border of the fourth body. This is a syndesmophyte. Another syndesmophyte is beginning to form along the inferior surface of the third vertebral body. The lumbar spine has lost its normal lordotic curve and bone has formed, earlier than usual, in the capsules of the apophyseal joints producing synostosis.

Fig. 4A and 6. Further advanced AS with straightening of the spine, apophvseal joint involvement, osteoporosis, and syndesmophytes producing ankylosis at all levels. (6) On the frontal projection, syndasmophytes are seen projecting laterally adjacent to the vertebral discs. Upon close inspection. it is apparent that the inner borders of the syndesmophytes actually extend vertically through the outer part of the discs. Subsequent apposition of bone laterally has produced the appearance of bulging. On the lateral roentgenogram, the syndesmophytes do not bulge. Superimposed on the inner borders of the syndesmophytes end immediately adjacent to the lateral borders of the pedicles are bilateral vertical lines extending throughout the length of the lumbar spine. These have the appearance of railroad tracks and represent the ossificetfon of the apophvseal joint capsules posteriorly. Fig. 5A and B. Ankylosis of the thorecic spine except et the level of the errovv where there is an interruption of the syndasmophytes anteriorly and en irregular radiolucent horizontal line of bone resorption. Sclerosis of the adjacent vertebral body surfaces is also apparent. This has resulted from a localized fracture through a fused portion between two adjacent vertebral bodies. fB) Two years later the roantgenogram demonstrates healing of the fracture.

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Fig. BA and 6. Cervical spine of the same patient as Fig. 2. In 1969 corner resorption and sclerosis are present resulting in the loss of the normal anterior vertebral body concavity. Syndesmophytes ar8 beginning to form along the inferior anterior borders of the upper two vertebral bodies. The posterior joints app8ar normal. (B) Four years later syndesmophytas extend between the upper three vertebral bodies and dorsally between the upper two bodies. These two Vertebra8 are now narrower in their anterior posterior dimension. The spine is straightened and the bones are osteoporotic. The intervertebral discs have narrowed.

that often extends beyond the original surface into the inserting ligament, tendon, or capsule. This new hone is initially irregular in contour with a ragged or fuzzy border which, with subsequent remodeling, becomes smooth. The earliest lesions of the spine are at the thoracolumbar and lumbosacral transitions. The process then ascends the spine usually involving the cervical region last. Most often the initial lesions are seen at the upper and lower anterior corners of the vertebral bodies on the lateral radiograph. The corner is eroded and a small triangular area of reactive sclerosis is visible adjacent to it. The erosion of the corners converts the original anterior concave surface of the vertebral body to a straight or convex surface. The intervertebral disc is also affected and may become narrowed or calcified. With repair, as at other sites, bone formation in the outer fibers of the intervertebral discs extends from one vertebral body to another. These bone formations are known as syndesmophytes and extend through the disc. In time additional apposition of bone develops along the lateral surfaces producing a bamboo appearance on the frontal radiograph. Unlike RA, the cervical lesions are not often associated with instability and subluxations of the lower five vertebrae. The odontoid is often subluxated, but bone destruction is usually less than in RA. Ankylosis primarily affects the vertebral bodies and less often the apophyseal joints when the disease begins later than 20 yr of age. This is in contrast to RA and Still’s disease in which apophyseal fusion is present if ankylosis of the bodies occurs. If the onset of AS is before the age of 20 and particularly in females, apophyseal lesions prevail. Underdevelopment of vertebral bodies is not uncommon when the disease begins in childhood, but somewhat similar narrowing of vertebral bodies may develop due to bone

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Fig. 7. A lateral cervical spine with the neck flexed demonstrates dorsal subluxation of the odontoid process. There is little resorption of the odontoid and no other evidence of AS is apparent. Fig. 8. The left foot of a patient with AS shows swelling of the soft tissues of the great toe and distension of the metatarsophalangeal joint capsule. Destruction of the cortex with underlying sclerosis and some extension of new bone into the adjacent soft tissue is seen at the base of the first proximal phalanx and at the medial margin of the distal first metatarsal. The articular cartilage of the metatarsophalangeal joint is narrowed minimally. The pathologic changes appear to be primarily the result of tenosynovitis. Fig. 9. There is sclerosis of bone with extension into the adjacent tendinous insertions of the greater trochanters. ischial tuberosities. and left acetabulum. The amount of asymmetry is unusual. The sacroiliac joints are fused. Uniform narrowing of the cartilages of both hips without significant bone resorption is present and is typical.

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remodeling in adults. Localized destructive lesions associated with acute pain may develop in patients with advanced ankylosis. The end plates of adjacent vertebrae may develop extensive destructive changes due to localized excessive motion secondary to a fracture of the lamina or adjacent supporting structures, or at individual nonankylosed segments where excessive mobility occurs. In about 60% of patients the joints of the extremities are affected. The frequency is as follows: acromioclavicular joint, knee, hip, shoulder, tarsal, proximal interphalangeal joints of the fingers, metatarsophalangeal joints, ankle, and wrist. The lesions in general are mild consisting of a joint effusion or some artitular cartilage narrowing with minimal erosion, although ankylosis may supervene particularly in the hips, tarsals, and carpal joints. FELTY’S SYNDROME

In 1924, Felty described a syndrome in five patients with splenomegaly and neutropenia in addition to chronic RA. Felty’s syndrome occurs in 1% or less of patients who have RA. Females are affected twice as frequently as males. Although the majority are over the age of 50 yr at the time of diagnosis, cases in 20 and 30 yr olds have been reported. The etiology is unknown. Most believe the association of splenomegaly and neutropenia with chronic RA is not coincidental and represents a syndrome. In addition to chronic RA, splenomegaly, and neutropenia, other findings that may also be present include weight loss, rheumatoid nodules, increased skin pig-

Fig. 1A and B. Prominent splanomegaly and hepatomagaly ara demonstrated. The right hip joint has the typical appearance of RA with uniform cartilage narrowing and early protrusion of the acatabulum into the pelvic cavity. (BI Five years following splenactomy the protrusion of the hip has increased indicating continual activity of the arthritic process. The femoral head is irregular in contour. A layer of fibrocartilaga separates the femoral head from the acatabulum. The protrusion of the acetabulum is upward as well as inward. (Figs. 2.3. and 4 ara of the same patient).

Fig. 2. There is severe generalized osteoporosis, ulnar deviation, and volar subluxation of the fingers, erosive changes of the metacarpal heads, and ulnar displacement of the first digit of the right hand. The left hand is similarly affected. These abnormalities are characteristicof RA. The carpal joints are narrowed and destructive changes of the distal radius and ulna as well as radial rotation of the carpals are present. The synostosis of the second distal interphalengeal joint could be due to either RA or osteoatthrosis. The smooth shallow defect of the ulnar surface of the right proximal phalanx is due to mechanical pressure and friction from the adjacent metacarpal head. The patient. a 69-yr-old female. had RA for 20 yr prior to developing Fefty’s syndrome. Fig. 3. The toes are angulated laterally and the middle three also demonstrate dorsal subluxation. Erosive changes are minimal. The other foot is symmetrically involved.

Fig. 4. The cervical spine presents the typical findings of RA with extensive erosion of the odontoid, narrowing of the third. fourth, fifth, and sixth intenrertebral discs without osteophyte formation, erosion of the inferior anterior surface of the third and superior anterior surface of the fourth vertebral bodies and forward subluxation of the third vertebra. The apophyseal joints do not appear abnormal.

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mentation, lymphadenopathy, hepatomegaly, chronic leg ulceration, keratoconjunctivitis sicca and Sjbgren’s syndrome, peripheral neuropathy, anemia, and purpura. Recurrent infections are common and are often the cause of death. The RA is usually present a number of years before the diagnosis of Felty’s syndrome is made, although sometimes the syndrome may be present within the first years of the disease. There is no correlation between the extent or activity of the arthritis and the development of the syndrome. Often the arthritis is quiescent or “burnt-out” at the time of diagnosis. Rheumatoid factor is almost always present, often in high titers. LE cell preparations may be positive, but the percentage does not appear to be significantly higher than that which is usually seen with severe RA without Felty’s syndrome. Roentgenographic

Features

Radiographic findings of chronic RA with varying degrees of involvement and activity are present. Splenomegaly can ofterr be seen on the abdominal radiograph. Corticosteroids are often employed in the treatment and bone changes secondary to this therapy may also be present consisting of generalized osteoporosis, often accompanied by compression fractures in the spine.

Fig. 1. The fingers of the right hand show psoriatic arthritis limited to the distal interphalangeal joints. There is extensive destruction of bone adjacent to the joints of the second, third, and fourth fingers with a connective tissue filled space between the distal and middle phalanges of the third finger. Complete synostosis of the distal joint of the fifth finger and beginning synostosis of the third finger is evident. The bone adjacent to the arthritic process is sclerotic. Osteoporosis is absent. A thickened deformed third fingernail is visible. Fig. 2. Psoriatic arthritis of the radioulnar. radiocarpal. intercarpal. carpometacarpal. second metacarpophelangeal and proximal interphalangeal, and fifth distal interphalangeal joints. The features suggesting psoriatic arthritis are the extensive destruction of the proximal first metacarpal, the destruction of the lateral surface of the proximal fourth metacarpal, and the dense, closely adherent irregular periosteal bone formed about the proximal metacarpals and the distal end of the second proximal phalanx.

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PSORIATIC

ARTHRITIS

A subacute or chronic arthritis is often associated with psoriasis. The arthritis may follow, be synchronous with, or precede skin lesions. The joint lesions differ from RA in several respects. Psoriatic arthritis often preferentially affects the distal interphalangeal joints of the fingers and toes, is less commonly associated with osteoporosis, and is more often accompanied by sclerosis adjacent to erosive defects and extensive periostitis. It does not produce ulnar deviation, multiple subluxations, rheumatoid nodules, or a significantly elevated rheumatoid factor although some patients with psoriasis develop a disease similar to RA. Patients with psoriatic arthritis may develop specific lesions of the spine. Psoriatic arthritis is found in first degree relatives of patients about 50 times as often as in the general population. A multifactorial inheritance is likely. The disease has an equal sex distribution. The prevalence of arthritis in patients with psoriasis varies from 1.5% to 10% depending upon the criteria used for diagnosis. Many show HL-A27 antigens in their sera. Roentgenographic

Features

Psoriatic arthritis may involve almost any joint of the extremities and spine. Irrespective of its site and particularly when few joints are involved, the distribution

Fig. 3. The interphalangeal joints of the third, fourth, and fifth fingers are fused. There is tapering of the fourth and fifth proximal phalanges. The fingernails of the second and third fingers are thickened and the ungual tuft of the third distal phalanx has been eroded. These findings provide the diagnosis of psoriatic arthritis. The inactive arthritic process involving the metacarpophalangeal joints is not specific. Fig. 4. Oblique view of the thumb shows thickening of the nail and erosion of the underlying surface of the distal phalangeal tuft.

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Fig. 5. Erosion with sclerosis and tapering of the tips of the distal phalanges is seen in both great toes. The toenails are thickened. There are erosive lesions and sclerosisof both first distal interphalangeal joints of the great toes with dense periosteal bone present on the left. Similar lesions are present at the left fourth and fifth and right fourth distal interphalangeal joints and terminal tufts.

of lesions may be asymmetrical, or if bilateral, varied in extent. This asymmetry is greater than in RA. Osteoporosis is usually minimal or absent. Periostitis frequently is extensive at the site of ligamentous, capsular and muscular attachments to bone. It is often dense with irregular margins. It is seen along the dorsal and plantar surfaces of the calcaneus, the phalanges, tibia1 spines, patella, malleoli, distal radius and ulna, distal clavicle, and other bones. Localized lesions of joints may develop in some patients who have significant titers of rheumatoid factor. These lesions are usually typical of those seen in RA alone or represent a combination of both rheumatoid and psoriatic lesions. Patients who do not show a rheumatoid factor often have characteristic lesions. There is a predilection for involvement of the interphalangeal joints of the thumb and hallux and of the terminal interphalangeal joints of the fingers and toes. The fingers and toes are affected approximately equally. Bony ankylosis of the interphalangeal joints of the hands and feet, resulting in deformity, is common particularly in arthritis mutilans and spondylitis. Synostosis of fingers or toes is uncommon in RA, but occurs in the toes with Reiter’s syndrome. Severe destruction of bone producing a gap between adjacent sharply demarcated bony stumps

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Fig. 6. Extensive destruction about the interphelengaal joint of the big toe has left a connective tissue filled gap between the phalanges. Periosteal bone of the distal phalanx is “fluffy” and irregular. A less extensive lesion of the metatarsophalangaal joint with adjacent tenosynovitis has caused swelling of the entire toe, called a “sausage” toe. There is bony fusion of the third and fourth proximal interphalangeal joints as well as severe destruction of the metatarsophalangeal joints. The metetarsals taper distally. The diagnosis based on this roentgenogram alone is limited to psoriatic arthritis or Reiter’s syndrome. Fig. 7. The sacroiliac joints are asymmetrically effected end less advanced than one would expect if the large syndesmophyte between the first two lumbar vertebrae was due to AS. This syndesmophyte differs from those of AS because of the following features: it involves only tfre right side of the spine: it is thick and dense; it is continuous with the adjacent half of the vertebral bodies instead of being limited to the comers.

is suggestive of this disease, although similar lesions may be seen in Reiter’s syndrome and reticulohistiocytosis. The lesions are most frequer in the fingers and toes and are often followed by synostosis. Another characteristic lesion consists of destruction of the interphalangeal joints of the great toe with proliferation of bone at the base of the distal phalanx. Varying degrees of destruction and irregular bone proliferation may occur. Resorption of the tufts of the distal phalanges of the hands and feet is most extensive usually in the great toe and thumb. The lesion is associated with psoriasis of the adjacent nail and nail bed. The narrowed distal part of the bone is often sclerotic. Other nonspecific signs of psoriatic arthritis consist of osteolysis and tapering of metacarpals and metatarsals, and “mushrooming” or “cup-and-stem” deformity of the metacarpophalangeal, metatarsophalangeal and interphalangeal joints. Sacroiliitis and spondylitis appear in many cases of long-standing disease. Characteristic features which also appear in Reiter’s syndrome include: asymmetry of sacroiliac disease and syndesmophytes; extensive destruction and scle-

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Roentgenograms of the elbow and calcaneus of the seme patient show irregular Fig. 8A snd 6. erosion and periosteal bone formation with associated underlying sclerosis.The continuous process of bone erosion and fluffy periosteal reaction occurs in both psoriatic arthritis and Reiter’s syndrome.

rosis of the sacroiliac joint margins; and less frequent involvement of the symphysis pubis, crest of the ilium, ischial tuberosities, spinous and transverse processes and apophyseal joints. A straight spine and square vertebral bodies are less common. Progressive extension upward from the sacroiliac joints to the cervical spine is uncommon. The appearance of syndesmophytes varies from the marginal ones characteristic of ankylosing spondylitis to nonmarginal ones that begin from a broad base below or above the vertebral body corners often tapering in the form of a comma or inverted comma. Syndesmophytes may form in the annulus fibrosus without attachment to vertebral bodies. When marginal syndesmophytes are present they are usually fewer in number and asymmetrical in contrast to the symmetry and progressive involvement associated with ankylosing spondylitis. Occasionally the cervical spine alone may be affected without involvement of the lower portions of the spine. SJ6GREN’S

SYNDROME

Sjijgren’s syndrome is a chronic inflammatory disorder characterized by the triad of keratoconjunctivitis sicca, xerostomia (dry mouth), and connective tissue disease. The keratoconjunctivitis sicca and xerostomia (sicca complex) are the result of decreased lacrimal and salivary gland secretions, which is associated with lymphocyte and plasma cell infiltration and destruction of the acinar portion of the glands. The extensive multisystem nature of the syndrome became apparent recently. Females are affected nine times as often as males. In the majority the diagnosis is made between the ages of 40 and 60 yr. The time of onset of the first symptom usually antecedes the diagnosis by a number of years. Although the

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Fig. 1. Parotid sialogram demonstrates enlargement of the gland plus ductal dilatation, globule formation, and localized areas of cavitation. This patient has had RA for 20 yr. (See sections on RA. SLE. and PSS for the roentgen appearance of the arthritis associated with Sjiigren’s syndrome).

reported range is 5-77 yr, the diagnosis of Sjogren’s syndrome is rarely made in children and is uncommon before the age of 20 yr. There are reported cases of familial occurrence, which may be the reflection of a common environment, although predisposing genetic factors such as immunologic deficiencies may also play a role. The arthritis associated with this syndrome is often RA, less commonly SLE; these diseases may have predisposing genetic factors. The incidence is difficult to establish due to the insidious onset of the glandular dysfunction which often goes unnoticed by the patient. Keratoconjunctivitis was noted in 14% of patients with RA when specifically screened for this complication although no complaints referable to the eyes were noted. The disease occurs in 1 in 225 persons. The etiology is unknown. Immunologic abnormalities are present in the majority of patients. Rheumatoid factor, often in high titers has been reported in 75%-98% of patients, hypergammaglobulinemia in 56%-69%, and antinuclear antibodies in 48%-68% of patients with Sjogren’s syndrome. Although the characteristic features of Sjogren’s syndrome are keratoconjunctivitis sicca, xerostomia, and connective tissue disease, the associated

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manifestations are protean and the clinical presentations variable. The disease usually begins insidiously with only one symptom as the sole manifestation for many years. Ocular symptoms are the result of decreased tear formation and are often unrecognized by the patient. Associated lacrimal gland swelling is uncommon. Salivary gland dysfunction may also go unnoticed although the majority of patients note decreased salivary secretions resulting in a dry mouth, difficulty in swallowing, pain with fissure formation, serious dental problems, and recurrent salivary and parotid gland swelling. Xerostomia is rarely the sole presenting symptom. Most commonly it follows the ocular symptoms, although it may occur coincidental with ocular manifestations. Arthralgia or arthritis is observed in 50%~87% of patients, which is due to RA in 30%-57%. SLE, PSS, and AD occur less frequently. Joint symptoms usually precede the manifestations of Sjogren’s syndrome by 7-9 yr. Roentgenographic

Features

Sialography in patients with Sjiigren’s syndrome generally demonstrates abnormal salivary glands even in asymptomatic patients. Ductal dilatation with subsequent globule formation, cavitation, and finally destruction of the gland can be demonstrated. Radioisotope studies (sequential salivary scintigraphy) correlate with clinical manifestations and demonstrate delayed uptake in abnormal glands. Splenomegaly, hepatomegaly, pulmonary fibrosis, pulmonary nodules, bronchiectasis, and pleural disease may occur. There is a high incidence of pulmonary infections, particularly pneumonitis and bronchitis, which are usually recurrent and are probably due to the dryness of the respiratory tract plus the altered immunologic state. Osteomalacia may be associated with renal tubular acidosis. The lymphangiogram may be positive when malignant lymphoma develops. Radiographic findings associated with the connective tissue component of the disease usually are those of RA, less commonly SLE and PSS. (This article

will

and Rheumatism.)

be continued

in the February

1976

issue of Seminars

in Arthritis