STUDY OF SKIN CHANGES, HAND ABNORMALITIES AND PULMONARY MANIFESTATIONS IN DIFFUSE SCLERODERMA IN A TERTIARY CARE HOSPITAL OF EASTERN INDIA

Abstract

Introduction: Scleroderma is a connective tissue disease characterized by brosis affecting the skin and internal organs.Joint involvement has been reported to occur in 46%- 97% of SSc patients. Bone and soft tissues may also be targeted; calcinosis and acro-osteolysis are thought to be the main characteristic ndings in SSc. Pulmonary disease in SSc falls into two major categories: interstitial lung disease (ILD) and pulmonary hypertension. Using PFTs, signicant pulmonary involvement is detectable in 25% of the patients with SSc within 3 years of initial diagnosis. The gold standard for determination of PAH in patients with SSc is right heart catheterization. The HRCT features of lung disease are ground-glass opacication predominant or admixed with reticular abnormalities in the majority of cases and little or no honeycombing. To study the demographic prole, Clini Aims and Objectives: cal Manifestations, Radiological abnormalities, Lung involvement and PAH. 35 newly diagnosed Sclederma patients Materials and Methods: attending Medicine and Rheumatology OPD of Medical College Kolkata were studied in this Cross-sectional observational study. Clinical Manifestations are noted and Radiological Investigations were recorded in structured Proforma. Data analysed using standard statistical methods. In Results and Analysis: our study, the study population had an F: M ratio of 10:1. majority of population (38%) having age group(30-40) yrs followed by less than 30 yrs & 41yrs or more (31% each). In our study clinically hand abnormality was evident in 69% patients & radiologically hand abnormality was evident in 74% patients. In our study population 29% patients have had acroosteolysis, 26% patients had erosion & 19% patients had exion contracture. In our study we found all the patient with documented ILD had abnormal PFT. In our study group restrictive pattern in PFT was evident in 63% patients & mixed pattern was evident in 23% patients. Twelve patients (35% of the total cohort) had pulmonary hypertension dened as PAs of 30 mm Hg or greater. In our study we found Out of 30 patients with documented ILD in HRCT 20 patients had PAH & 10 patients had no PAH. Females are more affected than males. Majority Conclusion: of the patients affected are in the age group of 30-40 years.69% patients had clinically evident hand abnormality and 74% patients had radiologically evident hand abnormality.CXR showed ILD in 63% patients whereas86% patients had documented ILD in HRCT.Restrictive pattern of PFT abnormality is more than mixed pattern.57% patients had documented PAH in echocardiography.HRCT is more sensitive than chest radiography for detection of ILD.