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FRI0460 Pulmonary Fibrosis in Anca-Associated Vasculitis: Clinical Characteristics and Long-Term Followup of 49 Patients
  1. C. Comarmond1,
  2. B. Crestani2,
  3. A. Tazi3,
  4. B. Hervier1,
  5. S. Adam Marchand4,
  6. H. Nunes5,
  7. F. Cohen-Aubart6,
  8. M. Wislez7,
  9. J. Cadranel7,
  10. B. Housset8,
  11. C. Lloret-Linares9,
  12. P. Sève10,
  13. C. Pagnoux11,
  14. S. Abad12,
  15. J. Camuset13,
  16. B. Bienvenu14,
  17. M. Duruisseaux15,
  18. E. Hachulla16,
  19. J.-B. Arlet17,
  20. M. Hamidou18,
  21. A. Mahr19,
  22. M. Resche-Rigon20,
  23. A.-L. Brun21,
  24. P. Grenier21,
  25. P. Cacoub1,
  26. D. Saadoun1
  1. 1Internal Medicine, Groupe Hospitalier Pitié-Salpêtrière
  2. 2Pneumologie, Hôpital Bichat
  3. 3Pneumologie, Hôpital Saint-Louis, Paris
  4. 4Pneumologie, CHRU Tour, Tours
  5. 5Pneumologie, CHU d'Avicenne, Bobigny
  6. 6Internal Medicine, Hôpital Pitié-Salpêtrière
  7. 7Pneumologie, Hôpital Tenon, Paris
  8. 8Pneumologie, CHIC, Créteil
  9. 9Internal Medicine, Hôpital Lariboisière, Paris
  10. 10Internal Medicine, Hôtel-Dieu, Lyon, France
  11. 11Rheumatology, Mont Sinaï, Toronto, Canada
  12. 12Internal Medicine, CHU d'Avicenne, Paris
  13. 13Pneumologie, Hôpital d'Argenteil, Argenteil
  14. 14Internal Medicine, CHU de Caen, Caen
  15. 15Pneumologie, CHU de Grenoble, Grenoble
  16. 16Internal Medicine, CHU de Lille, Lilles
  17. 17Internal Medicine, HEGP, Paris
  18. 18Internal Medicine, CHU de Nantes, Nantes
  19. 19Internal Medicine
  20. 20Biostatistiques, Hôpital Saint-Louis
  21. 21Radiology, Hôpital Pitié-Salpêtrière, Paris, France


Background Pulmonary fibrosis (PF) is a rare manifestation observed in patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA).

Objectives To report long-term outcome of a cohort of patients with the uncommon association of PF and AAV.

Methods We performed a retrospective multicentric study of characteristics and outcome of patients with PF associated to AAV, fulfilling the ACR and/or Chapel Hill definitions.

Results Forty nine patients [30 (61%) men, median age at diagnosis of AAV of 68 [58–73] years] with PF associated to AAV were identified. Forty (81.6%) patients had microscopic polyangiitis and 9 (18.4%) had granulomatosis with polyangiitis. The diagnosis of PF preceded the onset of vasculitis in 22 (45%) patients. Usual interstitial pneumonia was the main radiological pattern (n=18, 43%). ANCA were mostly of anti-myeloperoxydase specificity (88%). All patients were treated with steroids as induction therapy, combined with cyclophosphamide (CYC) (n=36, 73.5%) or rituximab (RTX) (n=1, 2%). Factors associated with mortality included occurrence of chronic respiratory insufficiency (HR 7.44, 95% CI (1.6 to 34.5), P=0.003), older age at PF diagnosis (HR 1.08, (1.02-1.13), P=0.005), and a higher eosinophil count at AAV diagnosis (HR 1.32, (1.07-1.63), P=0.0084). The 3-year survival rate in patients treated with steroids alone or combined with immunosuppressant (CYC or RTX) as induction therapy was of 64% (95% CI, 41-99) and 94% (95% CI, 86-100), respectively (P=0.03, Fig. 1). After a median follow-up of 48 [14–88] months, eighteen (37%) patients died, including 11 related to respiratory insufficiency.

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

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.4159

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