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FRI0299 Pulmonary Arterial Hypertension in Idiopathic Inflammatory Myopathies: Data from The French Pulmonary Hypertension Registry and Review of The Literature
  1. S. Sanges1,2,3,4,5,
  2. C. Yelnik1,2,3,4,5,
  3. O. Sitbon6,7,8,
  4. O. Benveniste9,
  5. K. Mariampillai9,
  6. M. Phillips-Houlbracq10,11,
  7. C. Pison10,11,
  8. C. Deligny12,
  9. J. Inamo13,
  10. V. Cottin14,
  11. L. Mouthon15,
  12. D. Launay1,2,3,4,5,
  13. M. Lambert1,2,3,4,5,
  14. P.-Y. Hatron1,3,4,5,
  15. L. Rottat6,7,8,
  16. M. Humbert6,7,8,
  17. E. Hachulla1,2,3,4,5
  1. 1Univ. Lille, U995, LIRIC - Lille Inflammation Research International Center
  2. 2INSERM, U995
  3. 3CHU Lille, Département de Médecine Interne et Immunologie Clinique
  4. 4Centre National de Référence Maladies Systémiques et Auto-immunes Rares (Sclérodermie Systémique)
  5. 5FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies, F-59000 Lille
  6. 6Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay
  7. 7AP-HP, Service de Pneumologie, DHU Thorax Innovation, Hôpital Bicêtre, le Kremlin-Bicêtre
  8. 8INSERM UMR_S999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, le Plessis-Robinson
  9. 9Département de Médecine Interne et Immunologie Clinique, Centre National de Référence Maladies Neuromusculaires, Hôpital la Pitié-Salpêtrière, AP-HP, INSERM U974, Université Paris VI Pierre et Marie Curie, Paris
  10. 10Clinique Universitaire de Pneumologie, Centre Hospitalier Universitaire
  11. 11Université Joseph Fourier, Grenoble, France
  12. 12Service de médecine interne et rhumatologie 3C/5D
  13. 13Département de Cardiologie, Centre Hospitalier Universitaire Pierre Zobda-Quitman, Fort de France, Martinique
  14. 14Hospices Civils de Lyon, Service de Pneumologie, Centre de Compétence de l'Hypertension Pulmonaire, Centre de Référence des Maladies Pulmonaires Rares, Lyon
  15. 15Service de Médecine Interne, Centre de Référence des Vascularites Nécrosantes et de la Sclérodermie Systémique, Université Paris Descartes, Hôpital Cochin, Paris, France


Background Occurrence of pulmonary arterial hypertension (PAH) in idiopathic inflammatory myopathies (IIM) without extensive interstitial lung disease (ILD) has been rarely described.

Objectives This study aimed to report all cases with association of PAH and IIM in the French Pulmonary Hypertension (PH) Registry, to identify IIM features associated with PAH, and to describe treatment modalities of these patients.

Methods All cases of IIM-PAH were retrieved from the French PH Registry, which gathers PAH patients prospectively enrolled by 21 referral centers across France. Patients were excluded if they had an extensive ILD or overlap syndrome. Characteristics of IIM-PAH patients were compared to a control group of IIM patients without PH.

Results Among the 5223 PAH patients in the Registry, 34 had a diagnosis of IIM. Among them, 3 IIM-PAH patients had no sign of extensive ILD or overlap syndrome, and were included in this study. In these patients, dermatomyositis (DM) was the only identified IIM. Two of them had a myopathic DM, confirmed by electromyography and biopsy. The last patient had an amyopathic DM, associated with anti-Ku antibodies. Pre-capillary PH was hemodynamically proven in all patients. PAH always developed after IIM onset, was severe in all cases, and led to a marked functional impairment.

By pooling our cases with 6 previously reported patients, and comparing them to a control cohort of 35 IIM patients without PH, we identify several IIM characteristics possibly associated with PAH occurrence, including DM subtype (78% vs. 46%, p=0.02), skin involvement (p=0.04), anti-SSA antibodies (p=0.05) and peripheral microangiopathy (p=0.06).

Overall, IIM-PAH patients were managed by corticosteroids and/or immunosuppressants, either alone or combined with PAH therapy. Patients that did not receive PAH therapy did not seem to respond to IIM treatment alone.

Conclusions Our study reports for the first time the rare but possible association of PAH and IIM in a large prospective PH Registry. In that setting, PAH seems associated with DM, skin involvement, peripheral microangiopathy and anti-SSA positivity. The best therapeutic strategy for IIM-PAH remains to be defined.

Disclosure of Interest None declared

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