Background Pulmonary arterial hypertension (PAH) is a rare disease characterized by the progressive increase in pulmonary artery pressures, leading to right heart failure. Systemic lupus erythematosus (SLE) is the second connectivity after Scleroderma can be complicated by PAH. This study aims to estimate the prevalence of PAH in SLE, detected by echocardiography.
Methods This is a retrospective study of ten years about 80 patients with SLE fulfilling the criteria of the ACR (American College of Rheumatology), followed in our department of internal medicine. Echocardiography with measurement of pulmonary artery pressure was performed in 72 patients. Above 30 mmHg mean arterial pressure was used to define PAH.
Results Ten patients (7% of cases) had pulmonary arterial pressure greater than 30mmHg. Two of them had lung disease (the first with emphysematous lung and the other a lupus interstitial lung disease). Three patients had mitral insufficiency. A syndrome of anti-phospholipid antibodies (APS) was noted in four patients (40% of cases). Five other lupus patients had isolated PAH (50% of cases). We noted a positive anti-Sm antibody in three patients (40% of cases).
Conclusions PAH is a rare and severe complication of SLE and is a bad prognostic factor. Its detection is based on echocardiography. PAH lupus may be the expression of APS or secondary to pulmonary fibrosis or left valvulopathy. The treatment is based on corticosteroids and immunosuppressive association. Anticoagulants are indicated in cases of APS.
Due to the severity of PAH in SLE, a screening echocardiography should be systematically offered to patients with SLE to treat early this serious complication and to enhance prognosis.
Disclosure of Interest None declared