Background Pulmonary arterial hypertension (PAH) is a rare but severe manifestation of systemic lupus erythematosus (SLE). It is important to suspect the development of PAH in time and prescribe treatment.

Methods The study included 6 patients with PAH associated with SLE, all 6 were women. All patients met the SLICC 2012 criteria for SLE. PAH was verified by right heart catheterization (RHC). Excluded were other forms of PH. Functional class (FC) of PAH was determined by the NYHA scale. Prior to the development of PAH, all patients (pts) were taking corticosteroids and immunosuppressants.

Results The mean age at diagnosis of PAH was 30,8±3,8 years. The mean duration of SLE up to PAH 9.3 years. In 5 patients, no other lesion of internal organs was detected, except for PAH, 1 patient had moderately severe glomerulonephritis. The ds-DNA antibodies were detected in 3 pts, Sm in 5, RNP-70 in 3, Ro-52 in 5, La in 2, nucleosomes in 2, ACL in 1 pts. The distance in the 6-minute walk test was 449±112 m, dyspnea on the Borg scale was 3.3±1.4. FC of PAH averaged 2.5 ± 0.5, 50% of patients each had FC I and FC II. All patients had chronic heart failure NYHA I or II. During RHC the mean PAP was 49.5±8.9 mm Hg, mean PAWP 5.5±1.2 mm Hg, PVR 9.79±2.4 units Wood. After the diagnosis of PAH, each patient was prescribed one specific drug (sildenafil, bosentan, macitentan or riociguat) with a positive result.

Conclusions PAH in SLE proceeds relatively favorably. Reception of immunosuppressants and glucocorticoids does not stop the development of PAH. Timely detection of this condition and the appointment of specific therapy lead to an extension of patient survival and an improvement in the quality of life.