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.
References
- Heather A. Van Mater, C. Egla Rabinovich. Nelson Textbook of Pediatrics:First South Asia Edition, Robert M. Kleigman, 2016,Elsevier India, Volume II, Ch 160:1186-87.
- Akter T, Silver RM, Bogatkevich GS (2014) Recent advances in understanding the pathogenesis of scleroderma-interstitial lung disease.Curr Rheumatol Rep 16: 411.
- Russo RA, Katsicas MM (2007) Clinical characteristics of children with Juvenile Systemic Sclerosis: follow-up of 23 patients in a single tertiarycenter. Pediatric rheumatology online journal 5: 6.
- Lo CY, Shyur SD, Chu SH, Huang LH, Kao YH, et al. (2010) Juvenile scleroderma: experience in one institution. Asian Pac J Allergy Immunol 28: 279-286.
- Recasens MA, Puig C, Ortiz-Santamaria V (2012) Nutrition in systemic sclerosis. Reumatol Clin 8: 135-140.
- Nagaraja V, McMahan ZH, Getzug T, Khanna D3 (2015) Management of gastrointestinal involvement in scleroderma. Curr Treatm Opt Rheumatol1: 82-105.
- Shanmugam VK, Steen VD (2012) Renal disease in scleroderma: an update on evaluation, risk stratification, pathogenesis and management. Curr Opin Rheumatol 24: 669-676.
- Zulian F, Woo P, Athreya BH, et al. The Pediatric Rheumatology European Society/ American College of Rheumatology/ European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis. Arthritis Rheum. 2007;57(2):203-12. (This publication describes in detail the new classification).