Pulmonary arterial hypertension in patients with systemic lupus erythematosus is a rare manifestation of a rare disease

The aim of the study was to determine the frequency, assess survival, and characterize the clinical features of systemic lupus erythematosus (SLE) in association with pulmonary arterial hypertension (PAH). Material and methods . According to the results of echocardiographic screening, 8 patients with PAH-SLE were identified after verification of the diagnosis on a right heart catheterization. Two cohorts of patients with SLE observed at the V.A. Nasonova Research Institute of Rheumatology were taken as a comparison group: the first is historical, with a long follow-up period (n=154), the second is modern, with a short duration of the disease (n=400). The analyzed patient groups were examined according to a single scheme, differences in signs and survival were assessed. The intrahospital frequency of PAH in SLE was estimated based on statistical data for the period from 2008 to 2017. Results. Despite the small number of patients in the main group, signs associated with the presence of PAH were identified: predominantly chronic course of SLE, with a long period (6–11 years) the course of the disease before the first clinical signs of PAH, the presence of Raynaud’s syndrome without capillaroscopic changes, Roand Smithautoantibodies. The survival rate of patients with PAH-SLE and the long-term observed SLE cohort did not differ. The in-hospital prevalence of SLE with echocardiographic screening was 0.29%. Conclusion . PAH is a rare manifestation of SLE associated with the features of the course and clinical picture, which does not significantly affect survival when using PAH-specific therapy.