Clinical Profile of Systemic Sclerosis

INTRODUCTION: Systemic sclerosis is a chronic multisystem disorder of unknown etiology characterized clinically by thickening of the skin caused by accumulation of collagen and by structural and functional abnormalities of visceral organs including the gastro intestinal tract, lungs, heart and kidneys. Systemic sclerosis is often of tragic consequence to patients. Survival is determined by the severity of visceral disease, especially involving the lungs, heart and/or kidneys. Earlier reports indicate that SSc rare in Asian Indian, Chinese and Malaysian. Contrary to this, later reports from India definitely have shown SSc is not uncommon in India, with a distinctly different clinical picture compared to that observed in the Western Countries. AIMS OF THE STUDY: 1. To study the clinical spectrum of patients presenting with Systemic Sclerosis (SSc). 2. To evaluate the internal organ involvement. MATERIALS AND METHODS: Patients who attended the Rheumatology department from October 2005 to September 2006 were taken up for the study. Systemic sclerosis was diagnosed in 25 patients as per the American College of Rheumatology (formerly American Rheumatism Association Scleroderma criteria cooperative study)42 criteria. Major Criteria: Sclerodermatous skin change in any location proximal to the Metacarpophalangeal joints. Minor criteria: • Sclerodactyly. • Digital pitting scars of finger tips (or) • Loss of digital finger pad substance and • Bibasillar pulmonary fibrosis. RESULTS: There were 2 males and 23 females giving a sex ratio of (1 : 11.5). • The age ranged from 20 – 60 years with a mean age of 40 years. • The highest incidence seen in 3rd decade (32%) 8 patients and 4th decade (38%) 9 patients. • The initial presentation was skin manifestation (52%) Arthralgia (40%), Raynaud’s (8%) Viseral symptom (4%). • Cumulative manifestations include skin (Cutaneous) 100%, Diffuse skin. • Involvement seen in (4%), Arthralgia (80%). • No one presented with fully developed CREST syndrome. • Calinosis – Nil. • Reynaud’s 28%. • Esophageal dysmotility 48%. • Sclerodactyly 40%. • Pigmentary lesions in the form of Hyperpigmentation and pepper salt. • Pigmentation in (68%). • Joint involvement in the form of arthralgia was seen in 80%. • Gastro Intestinal system was involved in 72%. • Respiratory system was involved in 16%. • Cardiovascular system was involved in 16%. • Renal system was not involved even in one case. CONCLUSION: From this study of 25 patients of systemic sclerosis following conclusions were derived. 1. Female preponderance (Female : Male 11.5 : 1) 2. The highest incidence seen in 3rd and 4th decade. 3. Limited cutaneous type was commoner than the diffuse SSc. 4. Skin and joint manifestations were predominant 5. Sclerodactyly and pigmentary changes are more common. 6. Raynaud’s phenomenon is very much less probably due to climatic causes. 7. Fully developed CREST was not present in this study. 8. Resorptions of terminal phalanges were less common. 9. Gastrointestinal involvement is more than other studies. 10. Respiratory involvement was low compared to other studies. 11. Cardiac involvement is less like other studies. 12. Renal involvement is NIL in this study