British Journal of Rheumatology 1990;29:303-305 CASE REPORT
ARTHRITIS MUTILANS, TUMORAL CALCINOSIS, RAYNAUD'S PHENOMENON AND SJOGREN'S SYNDROME BY M. MONTEAGUDO*, J. LIMA*, M. L. BRAVOf, F. GARCfA-BRAGADO*, E. LIENCEf, M. VILARDELL* AND M. C. ARNALf * Internal Medicine Service, t Rheumatology Section, Hospital General Valle de Hebrdn, Barcelona, Spain
KEY WORDS:
Arthritis, Calcification, Sicca syndrome, Respiratory function, Bone resorption.
normal: serum complement, rheumatoid factor, VDRL, antinuclear antibodies, RNP, Sm, SS-A/Ro SS-B/La and anticentromere antibodies. Chest radiographs showed an interstitial pattern in both lung bases. The tests of respiratory function showed a restrictive ventilatory impairment with a moderate reduction of TLco. Study of the oesophagus by barium meal and manometry was normal. The nail bed capillaries of thefingerswere also normal. An EMG study did not show myositis and the observed polyphasic pattern was possibly due to glucocorticoid treatment. Schirmer's test showed hyposecretion of tears (right eye 3 mm, left eye 4 mm, after 5 min). A biopsy of the minor salivary glands showed a lymphocytic infiltrate with destruction and atrophy of glands. Multiple soft tissue calcifications were present on the radiographs. They were more marked in both thighs (Fig. 1) and had a periarticular distribution particularly evident over the right shoulder, carpus and interphalangeal joints (Fig. 2) and back (Fig. 3). The hands had severe metacarpophalangeal and interphalangeal deformities with periarticular calcification and bone resorption.
SOFT tissue calcification is a relatively common manifestation in connective tissue diseases, especially in progressive systemic sclerosis (PSS) and the juvenile form of dermatomyositis[l]. It is much less common in systemic lupus erythematosus (SLE). Soft tissue calcification is also seen in hyperparathyroidism, sarcoidosis and hypervitaminosis D. Osteolysis of the distal interphalangeal joints is a typical manifestation of psoriatic arthritis, and less typical in other diseases like rheumatoid arthritis (RA) [2], multicentric reticulohistiocytosis and sarcoidosis [3]. Recently it has been described in patients with Sjogren's syndrome (SS) [4] and in a patient with tumoral calcinosis [5]. We report here a female patient with a 25-y.ear history characterized by arthritis mutilans involving both hands, Raynaud's phenomenon, tumoral calcinosis, lung fibrosis, and SS.
CASE REPORT In 1965 our patient was 36 years of age and had the first symptoms of symmetrical polyarthritis involving small and large joints with morning stiffness. There was progressive involvement of both hands with severe deformities and periarticular and soft tissue calcification. Raynaud's phenomenon appeared 20 years ago and xerostomia, xerophthalmia and fever 10 years later. When she was 60 years old she was admitted to our hospital. She had a fever (38.5°C) and physical examination showed severe deformities of both hands with subluxation, swan neck and opera glass deformities of some fingers. There were no anatomical nor functional abnormalities of the other joints. Hard and non-tender masses were present in her back, shoulders, arms, dorsum of hands and thighs corresponding to areas of calcification seen on radiographs. There were no skin lesions, sclerodactyly or telangiectasiae. She had no exposure to vinyl chloride. There were crackles in both lung bases. Laboratory data showed ESR 127 mm/h, white blood cell count 8.2 x 109/l, creatinine 7.1 mmol/1, serum uric acid 244 (imol/1, calcium 2.17 mmol/1, phosphate 1.16 mmol/1, creatine kinase 504IU/1, LDH342IU/1, aldolase 5.9IU/1, angiotensin converting enzyme 5.4 IU/1. The following tests were either negative or Submitted 29 October; accepted 5 November 1989. Correspondence to Dr E. Lience, Rheumatology Section, Hospital General Valle De Hebr6n, Paseo Valle Hebr6n S/N, 08035 Barcelona, Spain.
FIG. 1.—Large masses of soft tissue calcium in thighs. 303
Downloaded from http://rheumatology.oxfordjournals.org/ at University of Iowa Libraries/Serials Acquisitions on July 2, 2015
SUMMARY We report a female patient who over 25 years developed a progressive deforming arthropathy involving both hands without anatomical or functional abnormalities of other joints, Raynaud's phenomenon, lungfibrosisand Sjogren's syndrome. She had multiple soft tissue and periarticular calcification particularly evident in the lower extremities, shoulders, hands, fingers and back.
304
BRITISH JOURNAL OF RHEUMATOLOGY VOL. XXIX NO. 4
DISCUSSION Among the different causes of arthritis mutilans are RA, psoriatic arthritis, multicentric reticulohistiocytosis and sarcoidosis [3]. She exhibited no evidence of these, nor did she have other causes that could explain the presence of calcinosis [6]. The syndrome of tumoral calcinosis [7] explains the presence of painless hard masses with normal serum calcium and phosphate levels [8]. This has been described in a patient with acro-osteolysis [5] and has been reported in association with hyperphosphataemia [9].
REFERENCES
FIG. 3.—Chest X-ray shows calcification in the right shoulder and over the dorsum.
1. Blane CE, White SJ, Braunstein EM, etal. Patterns of calcification in childhood dermatomyositis. Am J Roentgenol 1989;142:397-400. 2. Ebong WW, Kolande TM. Tumoral calcinosis associated with rheumatoid arthritis and sickle cell anemia. Centr Afr J Med 1989;30:107-10. 3. Harris ED Rheumatoid arthritis: the clinical spectrum. In: Kelley WN, Harris ED, Ruddy S, Sledge EB, eds. Textbook of rheumatology. Philadelphia: WB Saunders, 1989;928-57. 4. Shuckett R, Russell ML, Gladman DD. Atypical erosive osteoarthritis and Sjogren's syndrome. Ann Rheum Dis 1989;45:281-8. 5. Frame B, Herrera LF, Mitchell DC, Fine G. Massive osteolysis and tumoral calcinosis. Am J Med 1971; 50:408-12. 6. Resnick D, Niwayama G, eds. Diagnosis of bone and joint disorders. Philadelphia: WB Saunders, 1989; 3102-80. 7. Lafferty FV, Reynolds ES, Pearson OH. Tumoral calcinosis. Am J Med 1965;38:105-18. 8. Mitnick PD, Goldfarb S, Scatopolsky E, Lemann JJR, Gray RW, Agus ZS. Calcium and phosphate
Downloaded from http://rheumatology.oxfordjournals.org/ at University of Iowa Libraries/Serials Acquisitions on July 2, 2015
FIG. 2.—Calcification of carpus. Multiple periarticular interphalangeal and metacarpophalangeal calcification. Arthritis mutilans.
However, coexistence with other connective tissue diseases may explain this clinical picture but there were no symptoms nor electromyographic changes suggestive of inflammatory muscle disease. We think that the increased levels of creatine kinase and aldolase may be due to the presence of calcined masses in or around the muscles. Progressive systemic sclerosis and CREST syndrome, in which soft tissue calcification is a common manifestation and in which severe osteolysis is occasionally seen [10], are unlike the illness of our patient because, despite a prolonged clinical course, there was no skin or systemic involvement. Furthermore, the anticentromere and other antibodies were negative. The patient did not fulfil the diagnostic criteria for SLE, a condition that occasionally is accompanied by soft tissue calcification [11]. Hydroxyapatite-associated destructive arthropathy [12] in the hands may be considered, but it was unlikely because of widespread florid non-periarticular and periarticular calcinosis without destructive changes except for the hands. There were no symptoms suggestive of hyperparathyroidism and the PTH levels were normal [13]. Shuckett et al. [4] reported five cases of SS with erosive osteoarthritis, but unlike our patient, none of the patients developed soft tissue calcifications. Tumoral calcinosis has been reported in association with acroosteolysis, and SS has been observed in patients with acro-osteolysis [14] but there is no patient like ours with tumoral calcinosis, Raynaud's phenomenon, acro-osteolysis, lung fibrosis and Sjogren's syndrome. In conclusion, we think that our patient had an unclassifiable connective tissue disease. We cannot rule out that she may have a special form of primary Sjogren's syndrome with tumoral calcinosis.
MONTEAGUDO ETAL.: PROGRESSIVE DEFORMING ARTHROPATHY metabolism in tumoral calcinosis. Ann Intern Med 1980;92:482-7. 9. Wilber J, Slatopolsky E. Hyperphosphatemia and tumoral calcinosis. Ann Intern Med 1968;68: 1044-9. 10. Brawer AC, Resnick D,KartinC,«a/. Unusual articular changes of the hand in scleroderma. Skeletal Radiol 1979;4:119-23. 11. Weinberger A, Kaplan J, Myers A. Extensive soft tissue calcification in systemic lupus erythematosus. Ann Rheum Dis 1979;38:384-6.
305
12. Resnick D. Calcium hydroxyapatite crystal deposition disease. In: Resnick D, Niwayama G, eds. Diagnosis of bone and joint disorders. Philadelphia: WB Saunders, 1981;1575-97. 13. Godfrey RS, Faddis DM, Daroca PS Jr, Sutherland CM. Tumoral calcinosis and hyperparathyroidism. J Ca State Med Soc 1987;139:26-8,31-2. 14. Fox RI, Howell FV, Bone PC, Michelsen P. Primary Sjogren's syndrome: clinical and immunopathologic features. Semin Arthritis Rheum 1984;14: 77-105.
1990 Sept Sept Oct Nov Nov
6-7 26-28 12 15-16 23
1991 Feb
8-9
April Sept
12 18-20
BSR Paediatric Course. BATH (Dr A. Clarke). BSR Annual General Meeting. IMPERIAL COLLEGE, LONDON. BSR Basic Rheumatology Course. CAMBRIDGE (Dr B. Hazelman). BSR Rehabilitation Course. NORTHWICK PARK (Dr A. Frank). French Rheumatology Society Meeting. PARIS. BSR Basic Rheumatology Course: Clinical examination and radiology of joints. NOTTINGHAM (DrM. Doherty). BSR Spring Meeting and Heberden Round. OXFORD (Dr A. Mowat). BSR Annual General Meeting. IMPERIAL COLLEGE, LONDON.
EDUCATIONAL VISITS TO UK RHEUMATOLOGY CENTRES As a trainee in Rheumatology (Registrar, Senior Registrar or equivalent), you may wish to broaden your experience by visiting other rheumatology units. Many centres around the UK are willing to host such visits. Details of these rheumatology units are available from the British Society for Rheumatology. The organization of the visit is then up to the visitor. Doctors from both the UK and overseas are welcome to contact the British Society for Rheumatology for further details: UK Rheumatology Visits The British Society for Rheumatology 3 St Andrew's Place Regent's Park London NW1 4LE Further information about these events from Ms L. Johnson, British Society for Rheumatology, 3 St Andrew's Place, Regent's Park, London NW1 4LE.
Downloaded from http://rheumatology.oxfordjournals.org/ at University of Iowa Libraries/Serials Acquisitions on July 2, 2015
ANNOUNCEMENTS AND CALENDAR FOR 1990-91
ARTHRITIS MUTILANS, TUMORAL CALCINOSIS, RAYNAUD'S PHENOMENON AND SJOGREN'S SYNDROME BY M. MONTEAGUDO*, J. LIMA*, M. L. BRAVOf, F. GARCfA-BRAGADO*, E. LIENCEf, M. VILARDELL* AND M. C. ARNALf * Internal Medicine Service, t Rheumatology Section, Hospital General Valle de Hebrdn, Barcelona, Spain
KEY WORDS:
Arthritis, Calcification, Sicca syndrome, Respiratory function, Bone resorption.
normal: serum complement, rheumatoid factor, VDRL, antinuclear antibodies, RNP, Sm, SS-A/Ro SS-B/La and anticentromere antibodies. Chest radiographs showed an interstitial pattern in both lung bases. The tests of respiratory function showed a restrictive ventilatory impairment with a moderate reduction of TLco. Study of the oesophagus by barium meal and manometry was normal. The nail bed capillaries of thefingerswere also normal. An EMG study did not show myositis and the observed polyphasic pattern was possibly due to glucocorticoid treatment. Schirmer's test showed hyposecretion of tears (right eye 3 mm, left eye 4 mm, after 5 min). A biopsy of the minor salivary glands showed a lymphocytic infiltrate with destruction and atrophy of glands. Multiple soft tissue calcifications were present on the radiographs. They were more marked in both thighs (Fig. 1) and had a periarticular distribution particularly evident over the right shoulder, carpus and interphalangeal joints (Fig. 2) and back (Fig. 3). The hands had severe metacarpophalangeal and interphalangeal deformities with periarticular calcification and bone resorption.
SOFT tissue calcification is a relatively common manifestation in connective tissue diseases, especially in progressive systemic sclerosis (PSS) and the juvenile form of dermatomyositis[l]. It is much less common in systemic lupus erythematosus (SLE). Soft tissue calcification is also seen in hyperparathyroidism, sarcoidosis and hypervitaminosis D. Osteolysis of the distal interphalangeal joints is a typical manifestation of psoriatic arthritis, and less typical in other diseases like rheumatoid arthritis (RA) [2], multicentric reticulohistiocytosis and sarcoidosis [3]. Recently it has been described in patients with Sjogren's syndrome (SS) [4] and in a patient with tumoral calcinosis [5]. We report here a female patient with a 25-y.ear history characterized by arthritis mutilans involving both hands, Raynaud's phenomenon, tumoral calcinosis, lung fibrosis, and SS.
CASE REPORT In 1965 our patient was 36 years of age and had the first symptoms of symmetrical polyarthritis involving small and large joints with morning stiffness. There was progressive involvement of both hands with severe deformities and periarticular and soft tissue calcification. Raynaud's phenomenon appeared 20 years ago and xerostomia, xerophthalmia and fever 10 years later. When she was 60 years old she was admitted to our hospital. She had a fever (38.5°C) and physical examination showed severe deformities of both hands with subluxation, swan neck and opera glass deformities of some fingers. There were no anatomical nor functional abnormalities of the other joints. Hard and non-tender masses were present in her back, shoulders, arms, dorsum of hands and thighs corresponding to areas of calcification seen on radiographs. There were no skin lesions, sclerodactyly or telangiectasiae. She had no exposure to vinyl chloride. There were crackles in both lung bases. Laboratory data showed ESR 127 mm/h, white blood cell count 8.2 x 109/l, creatinine 7.1 mmol/1, serum uric acid 244 (imol/1, calcium 2.17 mmol/1, phosphate 1.16 mmol/1, creatine kinase 504IU/1, LDH342IU/1, aldolase 5.9IU/1, angiotensin converting enzyme 5.4 IU/1. The following tests were either negative or Submitted 29 October; accepted 5 November 1989. Correspondence to Dr E. Lience, Rheumatology Section, Hospital General Valle De Hebr6n, Paseo Valle Hebr6n S/N, 08035 Barcelona, Spain.
FIG. 1.—Large masses of soft tissue calcium in thighs. 303
Downloaded from http://rheumatology.oxfordjournals.org/ at University of Iowa Libraries/Serials Acquisitions on July 2, 2015
SUMMARY We report a female patient who over 25 years developed a progressive deforming arthropathy involving both hands without anatomical or functional abnormalities of other joints, Raynaud's phenomenon, lungfibrosisand Sjogren's syndrome. She had multiple soft tissue and periarticular calcification particularly evident in the lower extremities, shoulders, hands, fingers and back.
304
BRITISH JOURNAL OF RHEUMATOLOGY VOL. XXIX NO. 4
DISCUSSION Among the different causes of arthritis mutilans are RA, psoriatic arthritis, multicentric reticulohistiocytosis and sarcoidosis [3]. She exhibited no evidence of these, nor did she have other causes that could explain the presence of calcinosis [6]. The syndrome of tumoral calcinosis [7] explains the presence of painless hard masses with normal serum calcium and phosphate levels [8]. This has been described in a patient with acro-osteolysis [5] and has been reported in association with hyperphosphataemia [9].
REFERENCES
FIG. 3.—Chest X-ray shows calcification in the right shoulder and over the dorsum.
1. Blane CE, White SJ, Braunstein EM, etal. Patterns of calcification in childhood dermatomyositis. Am J Roentgenol 1989;142:397-400. 2. Ebong WW, Kolande TM. Tumoral calcinosis associated with rheumatoid arthritis and sickle cell anemia. Centr Afr J Med 1989;30:107-10. 3. Harris ED Rheumatoid arthritis: the clinical spectrum. In: Kelley WN, Harris ED, Ruddy S, Sledge EB, eds. Textbook of rheumatology. Philadelphia: WB Saunders, 1989;928-57. 4. Shuckett R, Russell ML, Gladman DD. Atypical erosive osteoarthritis and Sjogren's syndrome. Ann Rheum Dis 1989;45:281-8. 5. Frame B, Herrera LF, Mitchell DC, Fine G. Massive osteolysis and tumoral calcinosis. Am J Med 1971; 50:408-12. 6. Resnick D, Niwayama G, eds. Diagnosis of bone and joint disorders. Philadelphia: WB Saunders, 1989; 3102-80. 7. Lafferty FV, Reynolds ES, Pearson OH. Tumoral calcinosis. Am J Med 1965;38:105-18. 8. Mitnick PD, Goldfarb S, Scatopolsky E, Lemann JJR, Gray RW, Agus ZS. Calcium and phosphate
Downloaded from http://rheumatology.oxfordjournals.org/ at University of Iowa Libraries/Serials Acquisitions on July 2, 2015
FIG. 2.—Calcification of carpus. Multiple periarticular interphalangeal and metacarpophalangeal calcification. Arthritis mutilans.
However, coexistence with other connective tissue diseases may explain this clinical picture but there were no symptoms nor electromyographic changes suggestive of inflammatory muscle disease. We think that the increased levels of creatine kinase and aldolase may be due to the presence of calcined masses in or around the muscles. Progressive systemic sclerosis and CREST syndrome, in which soft tissue calcification is a common manifestation and in which severe osteolysis is occasionally seen [10], are unlike the illness of our patient because, despite a prolonged clinical course, there was no skin or systemic involvement. Furthermore, the anticentromere and other antibodies were negative. The patient did not fulfil the diagnostic criteria for SLE, a condition that occasionally is accompanied by soft tissue calcification [11]. Hydroxyapatite-associated destructive arthropathy [12] in the hands may be considered, but it was unlikely because of widespread florid non-periarticular and periarticular calcinosis without destructive changes except for the hands. There were no symptoms suggestive of hyperparathyroidism and the PTH levels were normal [13]. Shuckett et al. [4] reported five cases of SS with erosive osteoarthritis, but unlike our patient, none of the patients developed soft tissue calcifications. Tumoral calcinosis has been reported in association with acroosteolysis, and SS has been observed in patients with acro-osteolysis [14] but there is no patient like ours with tumoral calcinosis, Raynaud's phenomenon, acro-osteolysis, lung fibrosis and Sjogren's syndrome. In conclusion, we think that our patient had an unclassifiable connective tissue disease. We cannot rule out that she may have a special form of primary Sjogren's syndrome with tumoral calcinosis.
MONTEAGUDO ETAL.: PROGRESSIVE DEFORMING ARTHROPATHY metabolism in tumoral calcinosis. Ann Intern Med 1980;92:482-7. 9. Wilber J, Slatopolsky E. Hyperphosphatemia and tumoral calcinosis. Ann Intern Med 1968;68: 1044-9. 10. Brawer AC, Resnick D,KartinC,«a/. Unusual articular changes of the hand in scleroderma. Skeletal Radiol 1979;4:119-23. 11. Weinberger A, Kaplan J, Myers A. Extensive soft tissue calcification in systemic lupus erythematosus. Ann Rheum Dis 1979;38:384-6.
305
12. Resnick D. Calcium hydroxyapatite crystal deposition disease. In: Resnick D, Niwayama G, eds. Diagnosis of bone and joint disorders. Philadelphia: WB Saunders, 1981;1575-97. 13. Godfrey RS, Faddis DM, Daroca PS Jr, Sutherland CM. Tumoral calcinosis and hyperparathyroidism. J Ca State Med Soc 1987;139:26-8,31-2. 14. Fox RI, Howell FV, Bone PC, Michelsen P. Primary Sjogren's syndrome: clinical and immunopathologic features. Semin Arthritis Rheum 1984;14: 77-105.
1990 Sept Sept Oct Nov Nov
6-7 26-28 12 15-16 23
1991 Feb
8-9
April Sept
12 18-20
BSR Paediatric Course. BATH (Dr A. Clarke). BSR Annual General Meeting. IMPERIAL COLLEGE, LONDON. BSR Basic Rheumatology Course. CAMBRIDGE (Dr B. Hazelman). BSR Rehabilitation Course. NORTHWICK PARK (Dr A. Frank). French Rheumatology Society Meeting. PARIS. BSR Basic Rheumatology Course: Clinical examination and radiology of joints. NOTTINGHAM (DrM. Doherty). BSR Spring Meeting and Heberden Round. OXFORD (Dr A. Mowat). BSR Annual General Meeting. IMPERIAL COLLEGE, LONDON.
EDUCATIONAL VISITS TO UK RHEUMATOLOGY CENTRES As a trainee in Rheumatology (Registrar, Senior Registrar or equivalent), you may wish to broaden your experience by visiting other rheumatology units. Many centres around the UK are willing to host such visits. Details of these rheumatology units are available from the British Society for Rheumatology. The organization of the visit is then up to the visitor. Doctors from both the UK and overseas are welcome to contact the British Society for Rheumatology for further details: UK Rheumatology Visits The British Society for Rheumatology 3 St Andrew's Place Regent's Park London NW1 4LE Further information about these events from Ms L. Johnson, British Society for Rheumatology, 3 St Andrew's Place, Regent's Park, London NW1 4LE.
Downloaded from http://rheumatology.oxfordjournals.org/ at University of Iowa Libraries/Serials Acquisitions on July 2, 2015
ANNOUNCEMENTS AND CALENDAR FOR 1990-91
