Pulmonary hypertension in systemic sclerosis and related disorders

Abstract EN

Twenty-five patients with systemic sclerosis and related disorders were selected from the Rheumatology, Chest, Cardiology and Clinical Pathology Departments, Zagazig University Hospitals.

They were divided into proximal and distal scleroderma groups with or without overlaping features of SLE and/or polymyositis.

They underwent multiple cardiopulmonary tests including chest radiology, pulmonary function testing, forced vital capacity (FVC%), diffusing lung capacity for carbon monoxide (DLCO%) and echocardiography to screen for pulmonary fibrosis and pulmonary hypertension (PH).

Pulmonary hypertension (systolic pressure  40 mm Hg) was diagnosed in 16 patients out of 20 patients subjected to doppler echocardiography.

Pulmonary hypertension was significantly higher in patients with proximal scleroderma (p < 0.05) and in those with an SLE / polymyositis overlap (p < 0.01).

The FVC% & DLCO% were significantly reduced in the proximal scleroderma group (p < 0.001) but not in the other.

Pulmonary hypertension was attributable to pulmonary fibrosis in 10 patients (8 in proximal and 2 in distal groups) and was attributable to pulmonary arteriopathy in 6 patients (one in proximal and 5 in distal).

The duration of the disease was significantly higher among patients with PH than those without PH (p < 0.05).