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Updated: Apr 14, 2026

The Left Pneumonectomy Combined with Monocrotaline or Sugen as a Model of Pulmonary Hypertension in Rats
Published on: March 8, 2019
[Antisynthetase syndrome with pulmonary hypertension: 4 original observations]
R Lecomte1, F Perrin1, L Journeau1
1Service 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.
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.
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