Title: Juvenile Systemic Sclerosis (JSS): A Rare Case from Berhampur, Odisha, India
Authors: Dr Sunil Kumar Agarwalla, Dr Kali Prasad Mishra, Dr Nithya B.G, Dr Dibya Ranjan Panda
DOI: https://dx.doi.org/10.18535/jmscr/v6i11.33
Abstract
Juvenile systemic sclerosis (JSS) is a multisystem connective tissue disease characterized by skin fibrosis and internal organ involvement. It has a low prevalence (1/1,00,000) even in a tertiary facility setting. Reynaud phenomenon, Proximal sclerosis, sclerodactyly are the predominant skin manifestation. Respiratory involvement occurred in two thirds of patients, and it manifested as dyspnea as well as abnormal imaging and/or pulmonary function tests; Pulmonary hypertension is a poor prognostic sign. Dysphagia was the commonest gastrointestinal symptom. The most frequent musculoskeletal symptom was arthralgia. Reynaud phenomenon heralds the beginning of the disease. Capilaroscopy is a major adjuvant in the diagnosis, since autoantibody determination may not offer sensitive and specific markers. Skin and vascular manifestations are the most common clinical features, while internal organ involvement is more rare. Cardiopulmonary disease is the most frequent visceral involvement, leading to significant morbidity. Herewith we are going to present a 11 year old female child presented with JSS.
Keywords: Juvenile systemic sclerosis (JSS), Connective tissue disorder, Capilaroscopy.
