Background/Purpose:  Pulmonary arterial hypertension (PAH) is detected in 0.5-17.5% of patients with systemic lupus erythematosus (SLE). Systemic inflammation may play a dominant role in its development, particularly in the early phases of PAH. Aim of this study was to describe the characteristics of PAH in a defined lupus cohort.

Methods:  We identified patients who had been diagnosed with PAH, defined as right ventricular systolic pressure (RVSP)>40mmHg on at least two separate transthoracic echocardiograms (TTE) using our database of a long term longitudinal prospective cohort study of SLE patients. Patients’ charts were hand-searched based on a pre-established protocol to derive PAH cause and symptoms at presentation. Variables including demographics, associated clinical and immunological features, PAH-targeted and systemic treatment as well as survival were retrieved from the database.

Results:   Fifty-one patients (47 females) were diagnosed with PAH since clinic inception. SLE was the sole cause (SLE-PAH) in 42 (82.4%) whereas other causes were identified in 9 (17.6%). Mean age of the SLE-PAH patients was 31.2±11.2 years at lupus onset and 38.7±12.3 years at PAH diagnosis. PAH was diagnosed within the first year of SLE in 13 (31%) cases. Main indications for TTE were unexplained dyspnea in 33 patients (78.6%), chest pain in 15 (35.7%), dry cough in 17 (40.5%) and syncope in 3 (7.1%). Mean initial RVSP was 59.2±17mmHg. Right heart catheterization (RHC) was performed in 15 patients [mean systolic pulmonary artery pressure (PAP)=62.7±24.7mmHg, diastolic PAP=25.4±9.4mmHg, mean PAP=39.4±14.8mmHg, pulmonary capillary wedge pressure=9.1±4.7mmHg, pulmonary vascular resistance=901±508 dynes-sec-cm^-5]. Upon PAH diagnosis, there was no other lupus clinical manifestation in 11 patients (26.2%, 8/11 had active serology which was concordant with PAH over time). Thirty-one (73.8%) had active involvement of other organs (4 central nervous system, 8 kidneys, 4 heart, 3 lung, 7 vasculitis, 8 musculoskeletal, 17 mucocutaneous and 1 catastrophic antiphospholipid syndrome). Active serology (low complement and/or increased anti-dsDNA titers) was detected in 28 (66.7%) patients, while 29 (69%) had positive anti-RNP, 10 (23.8%) lupus anticoagulant and 13 (31%) anticardiolipin antibodies. Immunosuppressive therapy was administered in 28 patients and was succesful in 24 (85.7%) with a mean RVSP reduction of 17.7±7.3mmHg (from 63.7±19.1 to 44.1±14mmHg) in 6.1±3.1 months. Induction treatment consisted of prednisone (mean initial dose 41.6±16mg/d) in all patients, methylprednisolone pulses in 8, cyclophosphamide pulses in 4 and other immunosuppressants in 12 (8 mycophenolate, 4 azathioprine). PAH-targeted therapy was administered to 5/28 patients (11 in total, 6 phosphodiesterase-5 inhibitors, 7 bosentan, 1 epoprostenol) and was discontinued in two due to PAP normalization. Two-year and 5-year survival was 95.2% and 90.5% respectively.

Conclusion:   SLE-PAH was usually diagnosed early in disease course and accompanied by other clinical and serological lupus manifestations. Aggressive immunosuppressive therapy was succesful in the majority of cases.

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/pulmonary-arterial-hypertension-in-systemic-lupus-erythematosus-a-single-centre-experience/