Background: Systemic sclerosis (SSC) is a disorder characterised by fibrosis of skin and visceral organs. The disease in males is different from that in female patients, with early organ involvement and an aggressive course. Very few studies are exclusively on male SSC. Objective: To study the clinical, immunological and treatment profile of male SSC patients from Northern India. Materials and methods: 31 male scleroderma patients who met the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria were included in the study. Their demographic, clinical and autoantibody profiles and treatment protocols were recorded retrospectively from patient files. Results: Median age of study population was 38(15) years with a mean duration of disease 60(108) months. Diffuse cutaneous systemic sclerosis (dcSSc) was observed in 19(61%) patients, 10(32%) patients had limited cutaneous systemic sclerosis (lcSSc) and two (6%) patients had sine SSC. Vasculopathy is the predominant manifestation in 28(90%) of the patients. Other systems involved were musculoskeletal in 19(61%), pulmonary 19(61%) and gastrointestinal manifestations in 18(58%) patients. Two patients had scleroderma renal crisis (SRC). Three patients had overlap with other rheumatic diseases. 25(80%) patients were antinuclear antibody (ANA) positive with Scl70 being the common autoantibody. Glucocorticoids were used in 14(52%) of patients. cs DMARDS were used in 27(87.1%) of patients and more than 50% of patients required b DMARDS. One patient had an aggressive course and died in the early phase of illness. Conclusion: SSC in males is associated severe organ involvement, ANA negativity and requires aggressive early aggressive immunosuppression to prevent morbidity and mortality.
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