Pulmonary Arterial Hypertension In A Female Patient With Systemic Lupus Erythematosus

Abstract

Abstract Introduction. Systemic lupus erythematosus (SLE) is a chronic autoimmune disease of unknown origin, characterized by multisystemic involvement and a potentially severe evolution. Pulmonary arterial hypertension (PAH) is a rare complication of SLE, with low 5-year survival. Case presentation. We are presenting the case of a female patient, aged 56 years old, diagnosed in 1992 with SLE with cutaneous manifestations (butterfly-shaped erythematous rash), joint manifestations (polyarthritis), serositis manifestations (massive pleuropericarditis), and immunological manifestations (positive anti-dsDNA antibodies, decreased C3), ignored therapeutically for a long time. In 2010 she complained of dyspnea on medium exertion and leg edemas, with marked increase of PAPs by echocardiography. She was diagnosed with severe PAH (confirmed by right heart catheterization) and in the “Marius Nasta” National Institute of Pneumology she started a treatment with an endothelin receptor antagonist (Bosentan) in combination with a prostacyclin receptor agonist (Selexipag). Since 2013 the patient is on oral anticoagulant treatment for permanent atrial fibrillation. In 2015 she was referred back to out clinic as she complained of recurrent episodes of massive ascites with evacuatory paracenteses in amounts of about 6-9L per paracentesis. After excluding other causes, ascites was considered to be secondary to the SLE, and a treatment was initiated with Hydroxychloroquine (HCQ) and pulse therapy with Methylprednisolone, on which the remission of the ascites was achieved during the following months. Currently, the SLE is well controlled without recurrence of ascites on treatment with HCQ and gradual decrease until stopping of cortisone doses, and the PAH is stable. Conclusion. PAH is a rare complication of the SLE, with a complex pathophysiological immune mechanism, for which - together with the specific vasodilator treatment - the increase of immune suppression is recommended.