Swati Khapekar/Majarikar, Revat Meshram, Dinesh Hinge, Amar Taksande, Mangesh Deshmukh, Abhijit Kumar Agrawal
DMIHER University. Fiona Stanley Hospital.
India and Australia
British Medical Journal Case Reports
BMJ Case Rep 2025; 18:
DOI: 10.1136/bcr-2025-264899
Abstract
Juvenile systemic sclerosis (JSSc) is a rare, progressive autoimmune disorder that can affect the skin, musculoskeletal system, gastrointestinal tract, lungs, heart, kidney and endocrine glands. Clinical presentation varies depending on organs involved and may include oesophageal dysmotility, interstitial lung disease, pulmonary hypertension, constrictive pericarditis, patchy myocardial fibrosis and glomerulonephritis with considerable morbidity and mortality.We are reporting a rare case of JSSc with multisystem involvement in an adolescent male. He presented with a 5-month history of polyarticular arthritis, skin thickening and hypopigmented skin lesions. After 2 months of treatment with methotrexate, he reported minimal improvement in joint pain, swelling and skin thickening, followed by worsening after self-cessation of treatment due to varicella zoster infection. His condition progressed with worsening skin thickening, polyarticular arthritis, joint contractures, restricted mobility, interstitial lung disease and dysphagia.
Category
Class I. Pulmonary Hypertension Associated with Connective Tissue Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
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