title: [Antisynthetase syndrome with pulmonary hypertension: 4 original observations].
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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.
authors.forename: R
authors.initials: R
authors.lastname: Lecomte
authors.affiliation: 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.
authors.forename: F
authors.initials: F
authors.lastname: Perrin
authors.affiliation: 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.
authors.forename: L
authors.initials: L
authors.lastname: Journeau
authors.affiliation: 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.
authors.forename: O
authors.initials: O
authors.lastname: Espitia
authors.affiliation: Clinique cardiologique et des maladies vasculaires, pôle hospitalo-universitaire 2, hôpital Nord Laënnec, boulevard Jacques-Monod, 44093 Nantes cedex 1, France.
authors.forename: N
authors.initials: N
authors.lastname: Piriou
authors.affiliation: Service de pneumologie, pôle hospitalo-universitaire 2, hôpital Nord Laënnec, boulevard Jacques-Monod, 44093 Nantes cedex 1, France.
authors.forename: D
authors.initials: D
authors.lastname: Horeau-Langlard
authors.affiliation: 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.
authors.forename: A
authors.initials: A
authors.lastname: Néel
authors.affiliation: 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.
authors.forename: A
authors.initials: A
authors.lastname: Masseau
authors.affiliation: 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.
authors.forename: M
authors.initials: M
authors.lastname: Hamidou
authors.affiliation: 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: christian.agard@chu-nantes.fr.
authors.forename: C
authors.initials: C
authors.lastname: Agard
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000150:complications*
keywords: Anti-tRNA-synthetase antibody; Anticorps antiARNt synthétase; Antisynthetase syndrome; Hypertension artérielle pulmonaire; Hypertension pulmonaire; Pulmonary arterial hypertension; Pulmonary hypertension; Syndrome des antisynthétases