We performed a cross-sectional study of the demography, clinical and laboratory features of patients with polymyositis and dermatomyositis followed up in our centre from 2006 to 2009. There were 12 cases, with the majority of them (58.3%) being woman. They have a mean age of 57.8 years and mean disease duration of 11.83 (SD 9.92) months. Our patients comprised of multi-ethnic groups with predominantly Chinese (83.3%), Sarawak natives (8.3%) and Malays (8.3%). They have a mean lag time to diagnosis of 3.67 (SD 4.27) months. Nine (75%) patients had dermatomyositis and 3(25%) had polymyositis. The common clinical manifestations found in our patients were proximal myopathy (100%), neck weakness (33.3%), dysphagia (33.3%) and interstitial lung disease (33.3%). For the nine patients with dermatomyositis, the most common dermatological manifestations were shawl sign (88.9%) and V sign (88.9%). Muscle enzymes were raised in 91.7% of patients. Electromyographies were carried out in four patients, and only one of our patients had muscle biopsy. Only 41.7% of our patients have positive ANA. The majority received prednisolone (100%) and hydroxychloroquine (58.3%). Malignancy occurred in five (three nasopharyngeal carcinomas, one sigmoid colon cancer and one lung cancer) out of the nine dermatomyositis patients but none in the polymyositis group. The mortality rate in our group was 4(33.3%) over the 4-year period. This study demonstrated the rarity of PM/DM in our centre with considerable lag time to diagnosis in our patients. Despite lack of muscle biopsy in our centre, our centre achieved appropriate diagnosis and management of PM/DM.
Objectives: To examine the descriptive epidemiology of the patient population referred to paediatric rheumatology centres (PRCs) in Southeast Asia (SEA) and to compare the frequency of conditions encountered with other PRC populations.
Methods: A web-based Registry for Childhood Onset Paediatric Rheumatic Diseases was established in 2009 and seven PRCs in four SEA countries, where paediatric rheumatologists are available, participated in a prospective 24 month data collection (43 months for Singapore).
Results: The number of patients analysed was 4038 (788 from Malaysia, 711 from the Philippines, 1943 from Singapore and 596 from Thailand). Over 70% of patients evaluated in PRCs in Malaysia, the Philippines and Thailand had rheumatic diseases (RDs), as compared with one-half of the proportion seen in Singaporean PRCs, which was similar to the Western PRC experience. Among RDs diagnosed (n = 2602), JIA was the most common disease encountered in Malaysia (41%) and Thailand (61%) as compared with systemic vasculitides in the Philippines (37%) and Singapore (35%) among which Henoch-Schönlein purpura was the most prevalent. SLE and related diseases were more common, but idiopathic pain syndrome and abnormal immunological laboratory tests were rarer than those seen in the West. JIA subtype distributions were different among countries. Among non-RDs (n = 1436), orthopaedic and related conditions predominated (21.7-59.4%).
Conclusion: The frequencies of RDs seen by SEA PRCs were different from those in the West. Systemic vasculitides and SLE were common in addition to JIA. Paediatric rheumatologist availability and healthcare accessibility partially explain these observed discrepancies.
Study site: multination + Selayang Hospital, Malaysia