MALAWIMEDJ.2003;15(l )14-16

REVIEW ARTICLE & CASEREPORT

Ankylosing spondylitis

in

A

Malawian.

Both his hips were ankylosedat 70 degreesof flexion anc degreesofabduction/ adduction(fig2). tls z Supine position showhg

maximum

amount of hip extension possible. Hip*

were ankylosed at 70 degrees flexion and neutral adduction / abduction

Mkandawire, N.C. Abstract I report a case of ankylosing spondylitis in a Malawian patient. I believe this is the first reported case of ankylosing spondylitis in a Malawian. Ankylosing spondylitis is rare in black Africans when compared with the Caucasian population. The clinical features ofthis diseaseare also different in these different racial groups. I will present a brief clinical history and a review of ankylosing spondylitis with special reference to the black African. Case Mr D.M. Date of Birth 27th June 1938 A 63 year old man presentedto QECH In July 2001 complaining of back and neck pain associatedwith loss of range of motion in the whole of his spine and hips rapidly getting worse since January 2001. He had not been able to walk since December 2000. He had difficulty squatting on a pit latrine and with perineal hygiene due to loss of motion in the hips. The history of his illness dates back to 1988 when he first noticedpain and stiffnessinhis cervicalspine.By 1989pain and stiffnesshad progressedto involve the thoracic and lumbar spine forcing him to quit his job as a clerk in the civil service.Between 1989 and 1998 his symptoms were quiescent.Towards the end of 1998 the back symptoms relapsedand he also had severepain and limitation of range of motion in the shoulder,hip and knee joints. He had difliculties with walking and startedusing a walking stick. Progressively loss of joint range of motion and fixed deformities in the spine,hips and, to a lesserextent, kneesresulted in complete loss of ability to walk by December 2000. He has no significant past medical, family, and social history. On examination he had complete loss of range of motion of the cervical, dorsal and lumbar spine. He had limitation of chest wall expansion.He could standbut both his hips and kneeswere in a flexed position (frg la and 1b).

He had partial loss of range of motion in the shoulderswith ths right one more involved than the left one. The left shoulderherl about 50% of normal range of motion preserved, whereas the right was estimated as having 30%oof normal range of motim preserved(fig 3) Fig 3 Showing functional range of motion possible at the shoulderjoints.

His knees were in a flexed attitude with a range of motion from 45 to 130 degrees.He could not walk. Full blood count, and erythrocyte sedimentationrate were normal. X-rays of the cervical, dorsal and lumbar spines showed 'bamboo spine'. His sacroiliacand hip classicalfeaturesof a joints were ankylosed.The shouldersshowed some degenerative changes.The knee x-rays were normal (see figs 4,5,6). Fio 4

frg la

fig lb

Standing posture showing fixed flexion deformities of the hips and knees.

I

REVIEW ARTICLE & CASEREPORT Africa, HLA 827 was present in only one patient (2). In sub SaharaAfrica, the prevalence of HLA 827 in the general population is thought to be between 3Yoand 6%. Theseestimatesare from researchin Senegaland Gambia (3). In the Gambia studies no case of ankylosing spondylitis was reportedupon screeningthe HLA B27 positivepopulationi.e. an HLA B2l positive black African has a much lower chance of having ankylosing spondylitis compared to a HLA B27 positive Caucasian. It may be postulated that there is presenceof some non HLA B27 protective factor reducing the prevalenceof ankylosing spondylitis in the black African population (3). Altematively the HLA B27 subtype present in black Africans is not associatedwith ankylosing spondylitis. This rarity of HLA B2l and the lack of correlation between HLAB27 and spondyloarlhropathies may explain the rarity of spondyloarthropathies in black Africans. Consequently,HLAB27 cannot be used as a diagnostic aid in spondyloarthropathiesin African blacks (2). Pathology There is progressive ankylosis of the involved joints. In the spine, the apophysealjoints and costovertebraljoints become fused. There is ossification of the intertebral discs, intervertebral ligaments, anterior longitudinal ligaments, and posterior longitudinal ligaments resulting in a rigid spine, the so called 'bamboo spine'. Involvement of the sacroiliacjoints is usually present early in the diseaseprocessand results in ankylosing of thesejoints. In synovialjoints thereis synvoitiswith accumulation of inflammatory cells (lymphocytes, plasma cells and histiocytes). This is followed by villous Hyperplasia, formation of a panuus, and destruction of the articular cartilage and ankylosis of subchondral bone. Inflammatory induced joint destruction and soft tissueossificationresult in ankylosisofthe axial skelel,.lanaqementconcentrated pain on relief with analgesicsand ton and large peripheral joints. Enthesopathy,which is inflam':rr inflamatory medications.Physiotherapyof the upper limbs mation at the inserlion of tendons and ligaments into bone, is ,rd knees was done to maintain functional range of motion in common and may be associated with the formation of bony :iese joints. The aim was to make him an independentwheel_ spurs at these sites. : :iair ambulatory patient. Discussion \nkvlosing Spondylitis (AS) in a chronic inflammatory condi_ rltrn of unknown etiology. It is usually grouped together with -,rher seronegative sponyloarthropathiessuch as Reiter's syn_ Jrome, psoriatic arthritis, arthritis of inflamatory bowel disease rnd undifferentiated spondyloarthropathies.It presents as an rntlammatory polyarthritis principally affecting the vertebral ;Lrlunm, sacroiliac joints and large peripheral joints such as the hips. shouldersand rarely the knees. trn the Caucasianpopulation, the prevalence rate of AS is esti_ mated at between 67.7 and 197 per 100,000people. The inci_ denceofAS in Caucasiansis estimatedat I to 2:2000. The male to female ration is 5:1. This compareswith a male to female ration of 1:3 in rheumatoid arthritis. There is a stuongassociation of AS with the class I antigen HLA-827, which is present in 80% to 98oh of affectedwhite patients compared with only g% of the generalpopulation. HLAB27 has twenty different alleles (subfypes)numbered one to twenty. Subtype number five HLA 821*05 is the allele which is most clearly and consistently asso_ ciated with ankylosing spondylitis and spondyloarthropathies (1). AS is very rare in black Africans and no prevalencerates could be found in the literature. In the few cases of AS reported in black Africans, HLA B27 is rarely an associatedfinding. In seventeen casesof ankylosing spondylitis reported in Central and Southern

Clinical Features The onsetof the diseaseis usually betweenthe agesof 16 to 30 years in theHLAB2T positive population. Age of onset is much older in the HLA 827 negalive population and in Black Africans. Patients may present with constitutional features as fatigue, weight loss and general malaise. Early morning stiffness, back pain and sacroilitis are common early features.Chest pain may occur as a result of involvement of the costoverlebraland costotransversejoints.Thereis progressiveloss ofrange ofmotion of the spine and the chest cavify. Late in the disease,fixed spinal deformities such severe kyphosis may develop. In the lumbar spine kyphotic deformity may interfere with the patient's ability to see the horizon, and also causescompressionof the abdominal viscera by the rib margin, compromising respiration, which is highly dependenton diaphragmatic excursion. In the cervical spine, kyphosis can progress to the point where the patient is unableto look forward becauseof a'chin on chestdeformity'. The hips and shoulders are the most commonly affected extraaxial joints and pain in thesejoints is the primary symptom in l5Yo of patients. Involvement of large peripheral joints such as hips and shoulderspresentwith joint pains and loss of range of motion. Fixed deformities may develop late in the diseaseresulting in hip flexion and adduction contractures. Extra articular involvement may result in anterior uveitis, iritis and iridocyclitis. Eye diseaseis the most common extra-articular manifestation of AS and occurs h 25ohto

REVIEWARTICLE

& CASE REPORT

Pain relief is achievedby administrationof analgesicand nt -steroidal anti-inflammatory drugs Function and range motion are maintainedby adheringto a regime ofphysiothe.;:and exercises. Analgesics and non-steroidalanti-inflammat.'. medication are crucial in maintenanceofrange of motion. fun:tion and adherenceto exerciseregimes Physiotherapyand exrcise regimes should include breathing exercises to mainra:: chest cavity expansion and posture training to avoid develoP ment of deformjty such as spinal kyphosis In the case presented,DM had complete ankf/losis of the hiF: fixed flexion and adductiondefomities resulting in loss u'f with Diagnoses Differential and handicapwith activities such astoileting. His enrire mobility of diagnosis of the differential phase of the disease, early In the already completely ankylosed The emphasisof maais diseases such spine include rheumatic would ankylosing spondylitis pain reliefwith anti-inflanmatory medicationsard was agement AS: upper limb range of motion He ha-' physiotherapy to maintain a Rheumatoidarthritis chair mobility ifhis upper limt' wheel potential for independent aReiter's syndrome is maintained. function o Reactiveafthdtis secondaryto gastrointestinaland Surgeryoccasionallyis indicatedto correct flxed severekyphotgenitourinaryinfections. ic deformities ofthe spine.In such cases,extensionosteotomres postue. Total joim Other inflammatory condition suchas psoriaticafihritis and gout of the spine are necessary to improve be indicated to may and shoulders the hips of feplacement are normally quite easily distinguished from ankylosing range of motion. Joint and restore function pain and relieve spondylitis. joints have become In sub-SaharanAfrica, the pandemicof HIV/AIDS has resulted replacementsare best done before the major 'HIV fully ankylosedit are already his hips Because fully ankylosed. RelatedArthropathies' in an increasein the incidence of joint relatively contraindicated are replacements that These primarily are reactive type arthritis or spondy- is felt

patients.Rareli the patient may have aofiitis and myocarditis. In late stageprogressivedisease,the patient may develop amyloidosis, which can causefatal renal failure. Black Africans with ankylosing spondylitis commonly present with late onset diseaseafter the age of fifty years. History of familial occunenceis rare in Africans and extm articular tissues are rarely involved. This pattem ofpresentationis similar to that seen in Caucasianswith HLA 827 negative ankylosing spondylitis (2).

loarlhropathiesother than ankylosing spondylitis. HIV/AIDS should thereforebe consideredin the sexually active age group presentingwith rheunatic arthropathies(4). I vestigations In the early phaseof the diseaseroutine investigationthat may need to be done, depending on clinical presentation, may include: . FBC . ESR o Rheumatoidfactor a HLA B27 (as discussedabove this is not a useful diagnosticaid in black Africans suspectedofhaving ankylosing spondylitis.) . HIV If rcactive adhritis from gastrointestinalor genitourinary infection in suspected,appropdateinvestigationsuch as: o Stool analysis o Urinalysis a Urethral swabs a Vaginal swabs may be necessary. X-rays of the appropriatejoints are important. Early sacroilitis 'fuzziness' of the sacroiliacjoint. Later frank obliteris seenas ation of the sacroiliacjoint signifies complete ankylosis. The vertebralcolumn shows evidenceof"squared off'vertebrae and bridging syrdesmophytesdue to calcification olthe intervefiebral discs,anterior longitudinal ligaments,posteriorlongitudinal ligamentsand intervertebralligaments. This gives the classical 'bamboo spine'. appearanceof hips may show evidenceofjoint destruction Largejoints suchas ankylosis of the joint. frank and, in late stages,

Summary - Ankylosing spondylitis is rare in black Africans.The incidence of HLAB27 is also rare in Black Africans and the association of HLA B27 and ankylosing spondylitis in black Africans is not strong. - Cliriical featuresofankylosing spondylitis in Black Africans are similar to the pattem seenin Caucasianswith HLA 827 negative ankylosing spondylitis. - Early diagnosis and initiation of treatment with analgesicsnon-steroidalanti-inflammatory medicationsandphysiotherapy is impofiant ifjoint range of motion and function are to be preserved.Results ofjoint surgery are maximised if operations are done beforejoints become ankylosed. - Further investigationsare neededto show the relationship of clinical features ol ankylosing spondylitis and spondyloafihropathiesand the different subtypes(alleles) of HLA B27 and their correlation with different racial populations and geographic regions ofthe World. N.C. Mkandawire, ofMedicine College Dep1. or Surgery, 2000lul: ofHLA 827 cur OpinRheumatol L Kian MA. Update:thc tsenty subtypes 1 2 ( 4 )2: 3 5 E Africa in sub Saharan 2. MiyajawaM. ODilnkltanO. Klun MA Spondyloanhopathies 2000Juli l2(4): 281 6 cur oDi. Rhcumatol in WcstAfricans L BrownMA, JepsonA. YoungA. ct al Ankvlosingspondvlitis for a non HLA B27prorecrileeff.ct Ann zurcumDis 1997Jani56(l): evidence 68 70 in AfricanblacksScminAithritisRheum1994 4. AdebajoA, DavisP Rlcutiaric discase I 3: 9 - 5 3 Oc| 2,1(21

Management The principle aims of managementare pain relief and maintenance ofjoint range of motion and function. l6

MalawiMedicaL JounMl / Ankylosins in a Malawian-

Ankylosing spondylitis in a Malawian.

I report a case of ankylosing spondylitis in a Malawian patient. I believe this is the first reported case of ankylosing spondylitis in a Malawian. An...
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