[Antisynthetase syndrome with pulmonary hypertension: 4 original observations]

R Lecomte; F Perrin; L Journeau; O Espitia; N Piriou; D Horeau-Langlard; A Néel; A Masseau; M Hamidou; C Agard

doi:10.1016/j.revmed.2015.03.013

[Antisynthetase syndrome with pulmonary hypertension: 4 original observations]

Rev Med Interne. 2015 Dec;36(12):794-9. doi: 10.1016/j.revmed.2015.03.013. Epub 2015 Apr 18.

[Article in French]

Authors

R Lecomte¹, F Perrin¹, L Journeau¹, O Espitia¹, N Piriou², D Horeau-Langlard³, A Néel¹, A Masseau¹, M Hamidou¹, C Agard⁴

Affiliations

¹ Service de médecine interne, pôle hospitalo-universitaire 3, centre de compétences maladies systémiques et auto-immunes rares, hôpital Hôtel-Dieu, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.
² Clinique cardiologique et des maladies vasculaires, pôle hospitalo-universitaire 2, hôpital Nord Laënnec, boulevard Jacques-Monod, 44093 Nantes cedex 1, France.
³ Service de pneumologie, pôle hospitalo-universitaire 2, hôpital Nord Laënnec, boulevard Jacques-Monod, 44093 Nantes cedex 1, France.
⁴ Service de médecine interne, pôle hospitalo-universitaire 3, centre de compétences maladies systémiques et auto-immunes rares, hôpital Hôtel-Dieu, place Alexis-Ricordeau, 44093 Nantes cedex 1, France; Inserm UMR1087, CNRS UMR6291, unité de recherche de l'institut du thorax, équipe 2 « Signalisation et hypertension artérielle », IRS université de Nantes, 8, quai Moncousu, BP70721, 44007 Nantes cedex 1, France. Electronic address: [email protected].

PMID: 25895991
DOI: 10.1016/j.revmed.2015.03.013

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.

Conclusion: 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.

Keywords: Anti-tRNA-synthetase antibody; Anticorps antiARNt synthétase; Antisynthetase syndrome; Hypertension artérielle pulmonaire; Hypertension pulmonaire; Pulmonary arterial hypertension; Pulmonary hypertension; Syndrome des antisynthétases.

Publication types

Case Reports
English Abstract

MeSH terms

Adult
Female
Humans
Hypertension, Pulmonary / etiology*
Male
Middle Aged
Myositis / complications*

Supplementary concepts

Antisynthetase syndrome