Background: Systemic autoimmune rheumatic disease-associated pulmonary arterial hypertension (SARD-PAH) is a major contributor to mortality. Few studies have explored the clinical profile and outcomes of this condition in India.

Objectives: To describe the profile of SARD-PAH of the patients attending the rheumatology clinic at our centre, and to assess the response to therapy at 6 months from the diagnosis of PAH.

Methods: We analysed the 4-year records of SARD-PAH patients who had at least 6 months of follow-up echocardiographic data since the diagnosis of PAH. Patients were diagnosed with SARD-PAH if they had definite evidence of a systemic autoimmune rheumatic disease, and echocardiographic PAH as defined by a right ventricular systolic pressure (RVSP) ≥30 mmHg and no pulmonary valve pathology. We used echocardiographic data as right heart catheterisation is not available at our centre, and is available only in a few centres across the country. Demographic data, diagnoses, clinical manifestations, details of autoantibodies, echocardiographic data at baseline and at 6 months, as well as details of therapy, were retrieved. Patients were considered to be responders if they had a decline in RVSP by ≥15mmHg or normalisation of pressures as measured by echocardiogram at 6 months.

Results: Twenty-one patients were included, 86% were females with a median age of 42 years. The most common diagnosis was an overlap syndrome (43%), followed by mixed connective tissue disorder and systemic sclerosis (each 19%). Interstitial lung disease was present in 48% of patients. The median baseline RVSP was 47mm Hg, and at 6 months it was 36mm Hg (for n= 15 patients), while 6 patients had a qualitative report of normal RVSP. No patient had LV dysfunction either at baseline or at follow-up. No patient had chronic thromboembolic pulmonary hypertension.

Fifteen (71%) patients responded to therapy. Almost all patients (95%) had received anti-PAH therapy, and almost all of them (95%) received immunosuppression for any indication.

Conclusion: We describe one of the first SARD-PAH cohorts from India with follow-up data. Our study shows that most patients (71%) respond to appropriate anti-PAH measures and immunosuppression. Long-term, prospective follow-up studies are required to better understand this condition in our population and to design appropriate therapy.

REFERENCES: NIL.

Acknowledgements: NIL.

Disclosure of Interests: None declared.