The spectrum of idiopathic inflammatory myopathies in South Africa
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ORIGINAL ARTICLE
The spectrum of idiopathic inflammatory myopathies in South Africa Keith J Chinniah 1
&
Girish M Mody 1
Received: 27 January 2020 / Revised: 4 March 2020 / Accepted: 16 March 2020 # International League of Associations for Rheumatology (ILAR) 2020
Abstract Introduction There are many reports on idiopathic inflammatory myopathies (IIM) but little information from sub-Saharan Africa. We conducted a retrospective study of IIM in a multi-ethnic cohort seen at a single centre in Durban, South Africa. Method The study included patients who fulfilled the Bohan and Peter or European League Against Rheumatism/American College of Rheumatology criteria for IIM. The information recorded included demographic data, clinical findings, results of investigations, treatment and outcome. Patients with overlap myositis (OM) had myositis and criteria for another connective tissue disease. Results There were 104 patients with IIM; 82.7% female and 70.2% African blacks. They included 41 (39.4%) with OM, 26 (25%) polymyositis (PM), 26 (25%) dermatomyositis (DM), six (5.8%) juvenile dermatomyositis and five (4.8%) cancerassociated myositis. Our patients had a younger mean age at diagnosis (36.8 ± 14.7 years) compared with 45–55 years in most other studies. Scleroderma-myositis overlap accounted for 26 (63.4%) of the patients with OM. Patients with OM were significantly younger than PM (p = 0.004) and DM (p = 0.044) and had lower, but not statistically significant, creatine kinase levels at diagnosis compared with PM (p = 0.052) and DM (p = 0.073). Interstitial lung disease was more common in OM (p = 0.001) and PM (p = 0.024) than DM. Oropharyngeal weakness was more common in DM than OM (p = 0.001) and PM (p = 0.032). African blacks were younger (p = 0.028) at diagnosis and had more cardiac abnormalities (p = 0.034) than Indians. Conclusion The spectrum of IIM in our cohort of mainly African blacks is similar to other studies, with OM being the most frequent subtype. Key Points • As there is limited information on idiopathic inflammatory myopathies (IIM) in sub-Saharan Africa, this study reports the spectrum of IIM in a South African cohort of predominantly African blacks. • Our patients were younger at diagnosis, and overlap myositis was the most common phenotype. • Comparisons with other studies show similarities in the manifestations of IIM.
Keywords African blacks . Dermatomyositis . Myositis . Overlap myositis . Polymyositis . Scleroderma
Introduction The idiopathic inflammatory myopathies (IIM) are a group of rare and heterogeneous disorders characterised by muscle inflammation and involvement of organ systems such as the skin, lungs, heart, joints and gastrointestinal tract [1]. They are associated with significant morbidity and an increase in mortality. The identification of myositis-specific antibodies (MSA) led to the recognition of subgroups of patients, and * Girish M Mody [email protected] 1
Department of Rheumatology, Nelson R Mandela School of Medicine, University of KwaZulu-Natal, Durban, South Africa
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