Study on Pulmonary Hypertension and Diastolic Dysfunction in Scleroderma

Balamurugan, S (2006) Study on Pulmonary Hypertension and Diastolic Dysfunction in Scleroderma. Masters thesis, Madras Medical College, Chennai.


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INTRODUCTION: Systemic sclerosis (scleroderma) is a generalized disorder of connective tissue characterized clinically by thickening & fibrosis of skin & by distinctive forms of involvement of internal organs notably heart, lungs, kidneys, gastrointestinal tract. The etiology & pathogenesis are unknown. It is characterized by fibrotic arteriosclerosis of peripheral & sytemic vasculature • variable degrees of extracellular matrix accumulation (mainly collagen) occur in both skin & viscera. • associated with specific antibodies most notably anticentromere & anti-scl-70 (anti topoisomerase). Various subsets with specific clinical features & variable involvement of various organs. The incidence of PulmHT varies between 6-60% of pts with scleroderma. In diffuse form upto 33% have PulmHT both isolated & in association with interstitial lung disease. In patients with limited scleroderma formerly referred to as CREST (calcinosis cutis, raynauds phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) upto 60% have pulmonary hypertension. Pulmonary hypertension is defined as a mean pulmonary artery pressure exceeding 25mmHg at rest or 30 mm Hg in exercise. When it occurs as a manifestation of SSc it is particularly severe & one year survival is approximately 55%. Studies suggest that both right and left ventricular dysfunction is common in scleroderma and that diastolic ventricular dysfunction may occur independent of systolic dysfunction. Diastolic dysfunction may be primary (myocardial fibrosis) or secondary to pulmonary or systemic hypertension. AIM OF STUDY: • To assess the prevalence of pulmonary hypertension in scleroderma patients with limited & diffuse forms. • To differentiate between those pulmonary hypertension due to interstitial lung disease (secondary PulmHT) from those without ILD (isolated PulmHT). • To assess the prevalence of ventricular diastolic dysfunction in scleroderma patients. MATERIALS AND METHODS: All patients were prospectively identified from rheumatology department of our college. There were 40 patients of SSc defined according to previously mentioned ACR criteria were studied. Of them 36 were females and 4 were males. Of them thirty patients satisfied the criteria for limited disease & ten had diffuse disease. Mean age of forty patients is 38.5 years. The average duration of illness is 3.5 years. All patients except three had raynauds phenomenon. Twenty patients were affected with digital pitting scars & ulcers, & two had digital amputation. For comparison twenty normal persons were selected (seventeen females, three male) of the same age group. All patients & controls gave consent for the study. None of the subjects included in the study had evidence of cardiac disease, hypertension, diabetes mellitus, chronic obstructive pulmonary disease, pulmonary tuberculosis or pulmonary thromboembolism as assessed by history, physical examination. STATISTICAL ANALYSIS: Continuous data were described as mean and standard deviation (mean +/- SD), and categorical variables as numbers. Comparisons between 2 categories were made using Student t test (2 tailed) for continuous variables. To analyze categorical data we performed the chi square test. Pearson correlation was used to correlate the continuous variables like disease duration and pulmonary artery pressure and parameters of diastolic dysfunction. CONCLUSIONS: Scleroderma is higher in females than in males. Pulmonary hypertension occurs in a significant proportion of patients with scleroderma. More often it is asymptomatic. Symptoms consistent with PAH have no Discriminant power to differentiate those with or without elevated PASP. Its more commonly associated with ILD in diffuse disease, but can occur without lung or heart abnormalities in limited form (isolated PulmHT). The ratio of patients with limited to diffuse is 3:1. Mild or early intermittent pulmonary hypertension are not likely to be identified by these non-invasive studies & hence mild PulmHT cannot be excluded in other patients. There is no statistically significant difference between patients with or without PulmHT when compared by type of SSc , age, sex, duration of disease or CRP When comparing Echo and Doppler differences between Patients With SSc and controls there was a significant difference between the two groups regarding the right ventricular cardiac chamber dimensions. There was a significant difference in the estimated pulmonary artery systolic pressure between patients with and without ventricular diastolic dysfunction. Raynaud phenomenon within the lung is a possibility. Diastolic dysfunction of RV, LV or both can occur in SSc, which can occur with or without the presence of PulmHT or systemic hypertension. More often it is asymptomatic. The extent of RV diastolic dysfunction was not related to the duration of the disease or to the SSc skin score. The hallmarks of SSc heart disease are myocardial fibrosis and ischemia. Intermittent coronary vasospasm, similar to Raynaud phenomenon, is prevalent in patients with SSc. The pattern of RV &LV diastolic disturbance seen in the present study could therefore be related to myocardial fibrosis and/or ischemia. Doppler echocardiography is a sensitive and non-invasive method of detecting cardiac abnormalities and systolic and/or diastolic function and for detecting pulmonary hypertension. Since so many therapeutic options are in the offing for management of PulmHT early detection is useful in altering the natural course of disease & in decreasing morbidity & mortality & to monitor the progression of disease. Detection of abnormalities in diastolic function might provide a means of identifying patients at risk for progressive heart failure.

Item Type: Thesis (Masters)
Uncontrolled Keywords: Pulmonary Hypertension ; Diastolic Dysfunction ; Scleroderma.
Subjects: MEDICAL > General Medicine
Depositing User: Subramani R
Date Deposited: 24 Mar 2018 17:29
Last Modified: 25 Mar 2018 02:47

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