SURVIVAL AND OUTCOMES OF SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS WITH PULMONARY HYPERTENSION - A PROSPECTIVE OBSERVATIONAL COHORT STUDY

PV148 / #463 Poster Topic: AS17 - Miscellaneous Background/Purpose Pulmonary hypertension (PH) is a severe manifestation in patients with systemic Lupus erythematous (SLE). Many treatment approaches have been suggested for SLE-PH. These treatments include vasodilatory drugs, steroids, immunosuppressive agents. There are many observational studies, case series, case reports & 1 RCT supporting the beneficial effect of cyclophosphamide in PH. However, response to other immunosuppressive agents has not been studied. This prospective observational study was planned to record survival at 24 months and treatment response in SLE-PH patients diagnosed by echocardiography to different immunosuppressive agents and vasodilators at 12 months. Methods Patients aged >18yrs with a diagnosis of SLE based on 2019 ACR/EULAR criteria & with clinical suspicion of PH are screened for PH by resting Doppler echocardiography. 22 patients with SLE-PH based on Systolic pulmonary artery pressure (SPAP) of >30 mmHg were recruited from among inpatient and outpatients of Clinical Immunology and Rheumatology department of Christian Medical College, Vellore from January 2021 till 2023 August. Patients with overlap connective tissue disease and pregnant females are excluded from the study. The clinical profile, antinuclear antibody, antiphospholipid antibodies (APLA), anti ribonucleoprotein (RNP)antibody, anti ds DNA antibody levels and complement levels are documented at baseline. Follow-up echo at 6,12 & 24 months were noted. They were given either IV or oral steroids followed by second line immunosuppression with either cyclophosphamide, or mycophenolate or rituximab or azathioprine and also vasodilatory therapy for PH based on clinician’s decision. Patients were assessed at 12 months to monitor treatment response in the form of improvement in clinical features, lab parameters & echocardiographic parameters. Patient’s response to treatment was documented in the following manner: Clinical improvement in - Shortness of breath – NYHA class, improvement in Resting doppler echocardiography parameters - decrease of SPAP by > 15 mmhg or > 20 % improvement from baseline or TRVmax < 2.8m/sec. The survival at 12 and 24 months is documented. Results The study included 22 patients of SLE-PH. 95.4 % (21/22) were women. At diagnosis of PH 13.64 % (3/22), did not report suggestive symptoms. SLE & PH was simultaneously diagnosed in 18.18% (4/22) patients. The mean age at diagnosis of pulmonary hypertension was 34.7 years (range 19-57 years). PH based on PASP values was severe in 45.4%, moderate in 40.9% and mild in 13.63%. 45.4% cases were in NYHA functional class III/1V & 54.5% in NYHA function class I/II. 18 patients had follow-up PASP values at 12 months, 61.1% (n=11) were treatment responders, 38.9% (n=7, 1 died) non responders. The mean decrease in PASP value in responder group was 22.84mmHg and non responder group was 0.78mmhg. No significant difference was noted in baseline characteristics, treatment modalities between the groups. One year survival was 95.4% (n=21) and 2 year survival was 80% (n=12/15). Conclusions SLE patients with PH have a high 1 and 2 year mortality in our cohort of patients. Anti U1RNP antibodies are in a high percentage of our SLE patients with PH. A higher percentage of patients had their PH responding to immunosuppression and vasodilatory therapy.

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