ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 738

Pulmonary Fibrosis In ANCA-Associated Vasculitis

Cloé Comarmond1, Bruno Crestani2, Abdellatif Tazi3, Baptiste Hervier4, Sylvain Adam-Marchand5, Hilario Nunes6, Fleur Cohen-Aubart7, Marie Wislez8, Jacques Cadranel8, Bruno Housset9, Célia Lloret-Linares10, Pascal Sève11, Christian Pagnoux12, Sébastien Abad13, Juliette Camuset14, Boris Bienvenu15, Michael Duruisseaux16, Eric Hachulla17, Jean-Benoît Arlet18, Mohamed Hamidou19, Alfred Mahr20, Anne-Laure Brun21, Philippe Grenier21, Patrice Cacoub22 and David Saadoun23, 1Internal Medicine and Clinical Imunology, Referal Center for Autoimmune diseases, Internal Medicine and Clinical Imunology, Hôpital Pitié Salpétrière, Paris, France, 2Pneumology A, Hôpital Bichat, Paris, France, 3Hôpital Saint-Louis, Paris, France, 4Internal Medicine & Clinical Immunology Dpt, Pitié-Salpêtrière Hospital, APHP, Paris, France, 5Pneumology, Centre Hospitalier Universitaire de Tours, Tours, France, 6Department of Pneumology, Avicenne Hospital (AP-HP), Bobigny, France, 7Internal Medicine Dpt 2, Pitié-Salpêtrière Hospital, APHP, Paris, France, 8Pneumology, Hôpital Tenon, Paris, France, 9Pneumology, Centre Hospitalier Intercommunal de Créteil, Créteil, France, 10Médecine Interne A, Hôpital Lariboisière, Paris, France, 11Internal medicine, CHU Lyon, Lyon, France, 12Rheumatology, Mount Sinai Hospital, Toronto, Canada, Toronto, ON, Canada, 13Internal Medicine, Avicenne Hospital, Bobigny, France, 14Pneumology, Centre Hospitalier Victor Dupouy, Argenteuil, France, 15Division of Internal Medicine, Centre Hospitalier Régional Universitaire de Caen, Côte de Nacre, Caen, France, Caen, France, 16Pneumology, CHU de Grenoble, Grenoble, France, 17Internal Medicine, Lille CEDEX, France, 18Internal Medicine, HEGP, Paris, France, 19Internal Medicine Department, Nantes University Hospital, Nantes, France, 20Department of Internal Medicine, Hospital Saint-Louis, Paris, France, 21Radiology, Hôpital Pitié-Salpêtrière, Paris, France, 22Médecine Interne 2, Hopital Pitié-Salpétrière, Paris, France, 23Groupe Hospitalier Pitié Salpétrière, Service de Médecine Interne, DHU i2B, Paris, France

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: , ,

Session Information

Title: Vasculitis I

Session Type: Abstract Submissions (ACR)

Background/Purpose:

The association of pulmonary fibrosis (PF) with anti neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), especially microscopic polyangiitis (MPA), is rare but related to poor prognosis. The possible link between PF, occult alveolar hemorrhage, ANCA positivity and specificity, and vasculitis remains unclear. The aim of the current study was to describe the characteristic features and long-term outcome in a large cohort of patients with PF associated to AAV.

Methods:

We performed a retrospective study of the characteristics and outcome of 49 patients with PF associated to AAV, diagnosed between 1996 and 2013, and according to the American College of Rheumatology criteria and/or Chapel Hill definitions.

Results:

Data were obtained from 30 (61%) men and 19 (39%) women. The median age at diagnosis of AAV was 66.5 [IQR: 57−74] years. Thirty nine (80%) patients had MPA and 10 (20%) had granulomatosis with polyangiitis. Besides constitutional symptoms, main extra-pulmonary manifestations included kidney involvement (57%), peripheral neuropathy (52%), and myalgia (37%). All patients had pulmonary symptoms, including crackles (77%), dyspnea (76%), chronic cough (27%) and/or hemoptysis (10%). The diagnosis of PF preceded the development of vasculitis in 21 (43%) patients, was concomitant in 21 (43%) and occurred subsequently in 7 (14%). At AAV diagnosis, restrictive syndrome was present in 67% of patients. Alveolar hemorrhage was present in 43% of patients. Thoracic computed tomography showed usual interstitial pattern in 39 (80%) patients, nonspecific interstitial pneumonia in 5 (10%) patients, and combined pulmonary fibrosis and emphysema in 5 (10%) patients. ANCA had mostly anti-myeloperoxydase specificity (84%). Hypereosinophilia was frequently observed at diagnosis of AAV (n=15,31%). All patients were treated with steroids as induction therapy, combined with cyclophosphamide (CYC) (n=37, 76%) or rituximab (RTX) (n=1, 2%). The 1-year survival in patients treated with steroids alone or combined with CYC or RTX as induction therapy was of 60% and 92%, respectively (p=0.016). After a median follow-up of 48 [14−88] months, vasculitis relapses occurred in 18 (37%) patients. Eighteen (37%) patients died, related to respiratory failure in 11 (61%) of them.

Conclusion:

PF is a rare manifestation of AAV with a very poor prognosis. Induction therapy with CYC might improve the outcome.

Disclosure:

C. Comarmond,
None;

B. Crestani,
None;

A. Tazi,
None;

B. Hervier,
None;

S. Adam-Marchand,
None;

H. Nunes,
None;

F. Cohen-Aubart,
None;

M. Wislez,
None;

J. Cadranel,
None;

B. Housset,
None;

C. Lloret-Linares,
None;

P. Sève,
None;

C. Pagnoux,
None;

S. Abad,
None;

J. Camuset,
None;

B. Bienvenu,
None;

M. Duruisseaux,
None;

E. Hachulla,
None;

J. B. Arlet,
None;

M. Hamidou,
None;

A. Mahr,
None;

A. L. Brun,
None;

P. Grenier,
None;

P. Cacoub,
None;

D. Saadoun,
None.

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