Antisynthetase syndrome with pulmonary hypertension: 4 original observations

• Published in: La revue de medecine interne Vol. 36; no. 12; pp. 794 - 799
• Main Authors: Lecomte, R, Perrin, F, Journeau, L, Espitia, O, Piriou, N, Horeau-Langlard, D, Néel, A, Masseau, A, Hamidou, M, Agard, C
• Format: Journal Article
• Language: French
• Published: France 01.12.2015
• Subjects: Adult; Female; Humans; Hypertension, Pulmonary - etiology; Male; Middle Aged; Myositis - complications; Antisynthetase syndrome; Hypertension pulmonaire; Pulmonary arterial hypertension; Anti-tRNA-synthetase antibody; Syndrome des antisynthétases; Pulmonary hypertension; Anticorps antiARNt synthétase; Hypertension artérielle pulmonaire
• ISSN: 1768-3122
• DOI: 10.1016/j.revmed.2015.03.013

Pulmonary hypertension (PH) may occur in patients with antisynthetase syndrome (ASS) but this association is poorly studied. In this article, we report 4 new cases of PH associated with ASS, and we discuss PH mechanisms in this specific disease. Four patients (3 females, 1 male) with confirmed ASS associated with anti-Jo1 (n=3), anti-PL7 (n=1), and anti-Ro52 (n=3) antibodies were analyzed. They presented with subacute dyspnea in average ten years after they were first diagnosed as ASS. Diagnosis of pre-capillary PH was made (mean of mPAP: 34mmHg): PAH (n=1), group 3 PH (n=2) and PH associated to hyperthyroidism (n=1). Among three patients who received specific PAH therapy, two had significant improvement in both clinical and hemodynamic parameters. During ASS, PH may occur in 5 to 10 % of cases, caused by various mechanisms. Unexplained dyspnea may be due to PH among ASS patients.