• Asmita Neupane, Prabesh Luintel, and Subodh Sagar Dhakal.
• Kathmandu Medical College and Teaching Hospital, Sinamangal, Kathmandu, Nepal.
• JNMA J Nepal Med Assoc. 2019 Nov 1; 57 (220): 457-459.

AbstractSystemic sclerosis is a connective tissue disease characterized by wide-spread vascular lesions and fibrosis of the skin and internal organs. It is an immune mediated rheumatic disease with the presence of an immunological dysfunction of T lymphocytes, especially Th1 and Th17 subtypes. It affects gastrointestinal, pulmonary, vascular, musculoskeletal, cardiac and various other systems. This disease is rare but has high morbidity and mortality with less known effective management. We report a case of 70-year-old female with systemic sclerosis presented with pain along with swelling over multiple joints since 18 months which exacerbated since last 6 months and wound over finger tips since last 2 weeks. We present here other various signs, investigations and management of this uncommon disease systemic sclerosis, also known as scleroderma. Various systems are evident to be involved including cardiac (left ventricular diastolic dysfunction) and peripheral vascular system (Raynaud's phenomenon). Keywords: connective tissue disease; immune-mediated; rheumatic disease; scleroderma; systemic sclerosis.

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