Cardio-pulmonary involvement in systemic sclerosis: A study at a tertiary care center

Geetakiran Arakkal, Sudha Rani Chintagunta, Vasavilatha Chandika, Sudha Vani Damarla, Srinivas Manchala, B Udaya Kumar

Indian Journal of Dermatology, Venereology, and Leprology 2017 83(6):677-682

Background: Systemic sclerosis is a multisystem disorder characterized by microangiopathy, dysregulation of the immune system and massive deposition of collagen in the connective tissue of the skin, blood vessels and various internal organs. Although the mortality from renal crises has dropped significantly due to the use of angiotensin-converting enzyme inhibitors, cardiac and pulmonary involvement accounts for significant morbidity and mortality. We studied 28 patients with systemic sclerosis at Gandhi Medical College and Hospital, Hyderabad, over a period of two years for cardiopulmonary involvement. Aim: The aim of this study was to analyze the cardiopulmonary involvement in systemic sclerosis. Methods: All patients with systemic sclerosis attending the dermatology outpatient department were included in the study. The diagnosis of systemic sclerosis was made based on the American Rheumatology Association criteria, and was further confirmed by skin biopsy and serological investigations. X-ray chest, electrocardiogram, two-dimensional ECHO, high-resolution computed tomography chest, pulmonary function tests and bronchoalveolar lavage were done to evaluate cardiopulmonary involvement. Observations: Out of 28 patients, 17 had diffuse systemic sclerosis and 11 had limited systemic sclerosis. Mean duration of symptoms was 2.9 years. Abnormalities in chest X-ray were found in 16 patients. Pulmonary function tests showed a restrictive pattern in 23 patients. High-resolution computed tomography of the chest showed evidence of interstitial lung disease in 21 patients, while five patients each had pleural effusion and cardiomegaly. Bronchoalveolar lavage showed different cellular patterns such as neutrophilia, eosinophilia and lymphocytosis. Pulmonary arterial hypertension was observed in seven patients and isolated pulmonary arterial hypertension in two patients. Electrocardiogram abnormalities were found in twenty patients. Two-dimensional ECHO was abnormal in 17 patients with valvular abnormalities being the most common finding. Overall, pulmonary involvement was observed in 27 patients and cardiac involvement in 17 patients. Limitations: A small sample size was a limitation of this study. Diffusing capacity of lung for carbon monoxide, and right cardiac catheterization were not done, as these were not available at our centre. Conclusions: In our patients, pulmonary involvement was more common than cardiac involvement. Interstitial lung disease and cardiac involvement were more commonly seen in diffuse systemic sclerosis whereas pulmonary hypertension was more frequent in limited systemic sclerosis. Hence, it is important to screen the patients for cardiopulmonary involvement for early diagnosis and treatment and a better prognostic outcome.