Carido-Pulmonary profile in Systemic Sclerosis.

Tamilselvam, T N (2007) Carido-Pulmonary profile in Systemic Sclerosis. Masters thesis, Madras Medical College, Chennai.


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INTRODUCTION : Systemic sclerosis is a generalised disorder of connective tissue characterized clinically by thickening and fibrosis of skin and by distinctive form of involvement of internal organs, notably the heart, lungs, kidneys and gastrointestinal tract. Morbidity and mortality are substantial and are directly related to the extent and severity of visceral involvement. Scleroderma or systemic sclerosis is characterized by fibrosis and micro vascular occlusion. These two processes are seen in all involved organs of patients with systemic sclerosis. Pulmonary arterial vascular disease with associated pulmonary hypertension is one of the difficult clinical situations in systemic sclerosis. The pulmonary vascular process can be indolent and remain clinically undetectable until severe irreversible pulmonary hypertension and right sided cardiac failure develop. The natural course of the lung disease is variable. The majority will have an early but modest decline in function and then follow a stable course or improve. One third will have a more severe progressive decline in lung function that continues for 4-5 years and then stabilize. OBJECTIVES : The objectives of the study are: 1. To study the cardiac manifestations in limited and diffuse cutaneous types of systemic sclerosis. 2. To study the pulmonary manifestations including pulmonary hypertension in limited and diffuse cutaneous types of systemic sclerosis. MATERIALS AND METHODS : This study was conducted in the department of Rheumatology, Madras Medical College and Govt. General Hospital Chennai during the period between August 2004 and December 2006.All the 63 consecutive patients with systemic sclerosis attending the Department of rheumatology, Govt. General Hospital, Chennai were included in this study. The patients fulfilling the American College of Rheumatology preliminary criteria for the classification of systemic sclerosis were included. Inclusion Criteria: I. Major criteria: Sclerodermatous skin change in any location proximal to the metacarpophalangeal joints. II. Minor Criteria: 1. Sclerodactaly, 2. Digital pitting scars on finger tips or loss of digital finger pad substance, 3. Bibasilar pulmonary fibrosis. Patients having the major and at least two or more of the minor criteria were included in this study. The selected cases must be of 16 and above years of age Exclusion Criteria: 1. Patients with overlap syndromes were excluded from the study. 2. Patients with features of MCTD and UCTD were excluded. 3. Patients with previous history of tuberculosis and occupational lung disease were excluded. RESULTS : Sixty three patients who satisfied the American college of rheumatology criteria for the classification of systemic sclerosis were subjected to various clinical and laboratory investigations including PFT, echocardiography, HRCT, and bronchoalveolar lavage study. The 63 patients were divided in to 2 groups as to whether they belong to diffuse cutaneous or limited cutaneous systemic sclerosis. CONCLUSION : 1. Systemic sclerosis is common in females and the female: male ratio is 15:1, with an age range of 17 to 60 years with a mean age of 37 in males and 35 in females. 2. Limited cutaneous type of presentation is more common. 3. Raynaud’s phenomenon is seen in both limited and diffuse cutaneous types, but the occurrence is more common in limited cutaneous type. 4. Secondary pulmonary hypertension due to interstitial lung is more common than the primary type of pulmonary hypertension. 5. Resting ECG revealed abnormalities in 42% of patients. ST-T wave changes followed by intra ventricular conduction disturbances constitute the most common abnormalities. 6. Mild pulmonary hypertension was noted in 6.3%, modereate pulmonary hypertension in 15.9%, and severe PHT in 3.2% of patients. Reversible cardiomyopathy was seen in 1 patient. 7. Mitral valve prolapse with or without mitral regurgitation was observed in 4.8% of patients. 8. Significant difference was noted in the right and left ventricular chamber dimensions between the patients and normal controls. The difference in the estimated pulmonary pressure was considerable in patients with and without ventricular diastolic dysfunction. 9. Chest X ray showed features of interstitial lung disease in 31.7% while HRCT revealed features of interstitial lung disease in 44.4% of individuals. 10. Pulmonary function tests showed mild restrictive type of ventilatory defect in 3.2%, moderate restriction in 19% and severe restriction in 15.9%. Normal PFT was seen in 61.9% of patients. 11. Interstitial lung disease was more common in diffuse cutaneous systemic sclerosis.

Item Type: Thesis (Masters)
Uncontrolled Keywords: Caridopulmonary profile ; Systemic Sclerosis.
Subjects: MEDICAL > Rheumatology
Depositing User: Subramani R
Date Deposited: 16 Aug 2017 00:48
Last Modified: 16 Aug 2017 07:11

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