Diagnosis and Management of Juvenile Scleroderma in a Resource-limited Setting: A Case Report from Zambia.

Abstract

A 15-year-old female adolescent with juvenile systemic sclerosis (JSSc), an uncommon variant of scleroderma marked by organ fibrosis and inflammation, is described. The patient had skin, musculoskeletal, lung, and gastrointestinal symptoms. The skin on the fingers and other parts of the body was hard and the joints had a limited range of motion. Juvenile systemic sclerosis was diagnosed using the 2013 American College of Rheumatology and European League Against Rheumatism (ACR-EULAR) classification criteria for Systemic sclerosis, supported by the diagnostic examination, which included histopathology. With a multidisciplinary approach, the patient was treated with drugs that reduced inflammation, eased symptoms, and slowed disease progression. The patient's management faced difficulties due to delayed presentation, travel distances for scheduled reviews, and budgetary limitations. In rare disorders like JSSc, early detection, interdisciplinary management, and removing obstacles to care are critical for better outcomes and are illustrated here.