Adult onset polymyositis/dermatomyositis: Clinical and laboratory features and treatment response in 75 patients

Abstract

Objectives - To determine possible similarities and differences in clinical and laboratory features and treatment response between patients in Singapore with polymyositis (PM) and dermatomyositis (DM) and reported series. Methods - Case records of adult patients (16 years old and above) referred to the 3 main electromyographic (EMG) laboratories in Singapore between 1 June 1986 and 31 May 1991 were reviewed if the referring diagnosis was myositis or myopathy for investigation. A computer search for adult patients with a diagnosis of PM/DM (ICD codes 710.3, 710.4, 517.8) who attended the main rheumatology and neurology centre during this period was also carried Out. The criteria for PM/DM proposed by Bohan and Peter was adopted. Results - The incidence of PM/DM was 7.7 cases per million population per year. There were 35 PM and 40 DM cases with a median age at diagnosis of 50.7 years (SD: 16.7) and significantly more females in the PM group (p < 0.05). At presentation, 86.7% had proximal myopathy, 34.7% had arthralgia/arthritis and 18.7% had cutaneous vasculitis. The creatine kinase level was elevated in 89.3% of patients and positive EMG and muscle biopsy in 79.4% and 76.4% respectively. Systemic lupus erythematosus was the commonest associated connective tissue disease. The percentage of patients with malignancy was higher in DM compared with PM (p < 0.01) and they were significantly older (mean age 61.8 years) (p < 0.001). Patients who achieved remission were significantly younger (mean age 46.4 years, p < 0.05). The overall mortality rate was 26.7% with infection and malignancy as the main causes of death. Conclusion - The results of the study suggest ethnicity does not influence the expression of PM/DM in view of the cnsiderable similarities in frequency and clinical expression of disease in the population studied compared with series from other countries.