Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature.
Occurrence of pulmonary arterial hypertension (PAH) in idiopathic inflammatory myopathies (IIMs) without extensive interstitial lung disease (ILD) has rarely been described in the medical literature. 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 the presence of PAH, and to describe treatment modalities of these patients.All cases of IIM-PAH were retrieved from the French PH Registry, which gathers PH patients prospectively enrolled by 27 referral hospital centers across France. Patients were excluded if they had an extensive ILD or overlap syndrome. Characteristics of IIM-PAH patients were compared with a control group of IIM patients without PH.Among the 5223 PH patients in the Registry, 34 had a diagnosis of IIM. Among them, 3 IIM-PAH patients (2 females and 1 male) had no evidence of extensive ILD or overlap syndrome, and were included in this study. In these 3 patients, dermatomyositis (DM) was the only identified IIM. One patient had autoantibodies classically associated with IIM (anti-Ku). PAH had always developed after IIM onset, was severe in all cases, and led to a marked functional impairment.By pooling our cases with 6 patients previously reported in the literature, and comparing them with 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 did not seem to respond to IIM treatment alone.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.
aUniversity of Lille, INSERM U995, LIRIC, Lille Inflammation Research International Center bCHU Lille, Département de Médecine Interne et Immunologie Clinique cCentre National de Référence Maladies Systémiques et Auto-immunes Rares (Sclérodermie Systémique), Lille dUniversity Paris-Sud, Faculté de Médecine, Université Paris-Saclay eAP-HP, Service de Pneumologie, DHU Thorax Innovation, Hôpital Bicêtre, Le Kremlin-Bicêtre fINSERM UMR_S999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson gDépartement 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 hClinique Universitaire de Pneumologie, Centre Hospitalier Universitaire, Grenoble, France iUniversité Joseph Fourier, Grenoble jService de médecine interne et rhumatologie 3C/5D, Centre Hospitalier Universitaire Pierre Zobda-Quitman kDépartement de Cardiologie, Centre Hospitalier Universitaire Pierre Zobda-Quitman, Fort-de-France, Martinique lHospices Civils de Lyon, Service de Pneumologie, Centre de Compétence de l'Hypertension Pulmonaire, Centre de Référence des Maladies Pulmonaires Rares, Lyon mService de Médecine Interne, Centre de Référence des Vascularites Nécrosantes et de la Sclérodermie Systémique, Université Paris Descartes, Hôpital Cochin, Paris, France.
Sébastien
S
Sanges
Cécile M
CM
Yelnik
Olivier
O
Sitbon
Olivier
O
Benveniste
Kuberaka
K
Mariampillai
Mathilde
M
Phillips-Houlbracq
Christophe
C
Pison
Christophe
C
Deligny
Jocelyn
J
Inamo
Vincent
V
Cottin
Luc
L
Mouthon
David
D
Launay
Marc
M
Lambert
Pierre-Yves
PY
Hatron
Laurence
L
Rottat
Marc
M
Humbert
Eric
E
Hachulla
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000150:complications* / Q000188:drug therapy; D011446:Prospective Studies; D012042:Registries; D012189:Retrospective Studies
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents
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