Juvenile Systemic Scleroderma: A comprehensive Review and Clinical Case Report from a Tertiary Care Hospital in India

Abstract

Juvenile systemic sclerosis (JSSc) is a highly uncommon, chronic connective tissue disorder that affects multiple systems, marked by progressive deposition of collagen under the dermis, and various deep organs. Delay in diagnosis can cause irreversible vital organ damage, leading to severe morbidities and seriously affecting quality of life. We report a clinical case of a 9-year-old boy with extensive and diffuse skin manifestations with vascular, pulmonary, and gastrointestinal involvement. The sero-immunological test revealed positive ANA results and high titers of anti-topoisomerase (Scl-70) antibodies, indicative of severe manifestation of the disease. He was managed with systemic immunosuppression and other pharmacological and rehabilitative measures to prevent ongoing organ damage and alleviate the patient’s symptoms.

Keywords

• connective tissue disorder
• immunosuppression
• juvenile systemic sclerosis
• pediatric