Antisynthetase syndrome and pulmonary hypertension: report of two cases and review of the literature.

Modern Rheumatology Case Reports
Antía García-FernándezCarlos de la Puente-Bujidos

Abstract

Antisynthetase Syndrome (ASS) is a subset of idiopathic inflammatory myopathies characterised by specific clinical features such as interstitial lung disease (ILD), fever, myositis, Raynaud's phenomenon, cutaneous involvement and arthritis related to the presence of anti-aminoacyl-tRNA-synthetase (anti-ARS) autoantibodies. Moreover, Pulmonary arterial hypertension (PAH) is a life-threatening complication associated with connective tissue diseases mainly systemic sclerosis (SSc-PAH). It has been suggested that PAH can complicate ASS patients but little is known about the prevalence and risk factors to develop this complication. Here we report on two patients with ASS and PH. The first one represents a complete picture of ASS anti-Jo-1 positive, the second an amyophatic ASS anti-PL-12 positive. In one of our ASS-PAH patients, specific treatment lead to improvement of PAH. There are no specific recommendations on current guidelines regarding either PAH screening or treatment in ASS, but performing echocardiogram, ECG, pulmonary function test and prompt initiation of specific therapies seems to improve right heart catheterisation (RHC) parameters and survival.

References

Jan 30, 2010·Scandinavian Journal of Rheumatology·Yoshinori TaniguchiY Terada
Apr 15, 2010·Arthritis Care & Research·Soumya Chatterjee, Carol Farver
Feb 12, 2013·The European Respiratory Journal·Baptiste HervierEric Hachulla
May 21, 2013·Annals of the Rheumatic Diseases·J Gerry CoghlanUNKNOWN DETECT study group
Jul 24, 2013·Tüberküloz ve toraks·Özlem Erçen DikenÖzlem Özdemir Kumbasar
Oct 3, 2013·The Journal of Rheumatology·Vasile ForisHorst Olschewski
Sep 7, 2016·Clinical Pulmonary Medicine·Leah J WittMary E Strek
Dec 13, 2016·Annals of the Rheumatic Diseases·Otylia Kowal-BieleckaUNKNOWN EUSTAR Coauthors
Feb 17, 2017·International Journal of Rheumatic Diseases·Ramya AithalaDebashish Danda

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