| Literature DB >> 25895991 |
R Lecomte1, F Perrin1, L Journeau1, O Espitia1, N Piriou2, D Horeau-Langlard3, A Néel1, A Masseau1, M Hamidou1, C Agard4.
Abstract
INTRODUCTION: 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. CASES: 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.Entities:
Keywords: Anti-tRNA-synthetase antibody; Anticorps antiARNt synthétase; Antisynthetase syndrome; Hypertension artérielle pulmonaire; Hypertension pulmonaire; Pulmonary arterial hypertension; Pulmonary hypertension; Syndrome des antisynthétases
Mesh：
Year: 2015 PMID： 25895991 DOI： 10.1016/j.revmed.2015.03.013
Source DB: PubMed Journal: Rev Med Interne ISSN： 0248-8663 Impact factor: 0.728