Clinical spectrum of inflammatory myositis in South India--a ten year study.

OBJECTIVE: To describe the clinical spectrum of inflammatory myopathies at a referral hospital in South India. METHODS: Patients were assessed for the pattern of muscle involvement, for the presence of arthritis, Raynaud's phenomenon, interstitial lung disease (ILD) and cardiac involvement. Muscle enzymes, electromyogram (EMG) and muscle biopsies were done. RESULTS: Eighty seven patients with inflammatory myopathies were encountered over 10 years. These included 24 with adult polymyositis, 26 with adult dermatomyositis, one with amyopathic dermatomyositis, five with juvenile myositis, one with dermatomysitis following carcinoma breast and 30 with overlap with other connective tissue diseases. There was a female preponderance (M:F = 1:2.35) except in juvenile myosits group (M:F = 1.5:1). The mean age of onset in years was 33.26 in adult polymyositis, 35.03 in adult dermatomyositis, 7.4 in juvenile dermatomyositis, 42 in malignancy-associated dermatomyositis and 25.51 in the overlap group. Proximal muscle weakness was seen in 98.8% patients, dysphagia in 33.3%, distal muscle weakness in 12.5%, respiratory muscle weakness in 9.2% and dysphonia in 4.6%. Other features included arthritis 35.63%, interstitial lung disease (ILD) 9.2%, Raynaud's 5.7%, myocarditis 4.6% and conduction disturbances 1.15%. Eleveated muscle enzymes were seen in 85.1% patients. Eletromyogram was positive in 66.6%. Muscle biopsy was positive in 85.29%. Anti-nuclear antibody was positive in 67.24%. All received steroids, non-responders needed methotrexate (13 patients) or azathioprine (11 patients). Death occurred in 10 (seven with dermatomyositis predominantly due to respiratory involvement and three with overlap). CONCLUSION: There was female preponderance except in juvenile myositis group. Proximal muscle weakness was the commonest feature. ILD was the commonest respiratory problem, while myocarditis was the commonest cardiac problem seen. Response to therapy and prognosis in polymyositis were good with no mortality during the study period. Death in the dermatomyositis group was mainly due to respiratory involvement.

A follow-up study of juvenile rheumatoid arthritis into adulthood.

AIM OF THE STUDY: To study the clinical profile of various subtypes of juvenile rheumatoid arthritis (JRA) in adulthood and analyse the outcome of the disease in terms of functional status, educational achievement, growth abnormalities, radiological progression and activity of the disease. METHODS: From a group of 150 JRA cases, 26 adult patients were included in the study. All of them were under follow up since the onset of disease in childhood. Clinical data at the onset were obtained from old medical documents. Detailed clinical and laboratory assessment of all cases were done. RESULTS: There were 10 oligoarticular, 13 polyarticular and three systemic onset cases. Mean age of onset of disease was 11.7 +/- 3.39 years (range 2-15). Mean duration of follow up was 11.4 +/- 4.46 years range (6-22). Twenty-one patients had active disease. Ninety percent of oligoarthritis group were in class 1 status whereas none of the systemic onset JRA cases were in class I. Micrognathia, short stiff neck and short stature were noticed among polyarticular and systemic onset JRA. Seventy percent of oligoarthritis group developed inflammatory low back ache. Bony ankylosis of tarsal and carpal bones were seen in eight cases. CONCLUSION: In our study there is a male predominance in JRA. Pauciarticular (oligoarthritis) JRA occurring in older boys had the best functional outcome. Growth abnormalities and radiological changes were more common in polyarticular and systemic onset JRA.