Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Alveolar Hemorrhage (AH) in ANCA-Associated Vasculitis: Characteristics and Prognostic Factors in 65 Patients.

Kostianovsky2,  Alex M., Hauser2,  Thomas, Pagnoux2,  Christian, Cohen2,  Pascal, Daugas3,  Eric, Mouthon2,  Luc, Cordier4,  Jean-Francois

Hopital Cochin-Paris Univ, Paris, France
Internal Medicine, Hopital Cochin, APHP, Universite Paris Descartes, Paris, France
Nephrology, Assistance Publique de Paris, Paris, France
Pulmonology, Hopsice Civils de Lyon, Lyon, France

AH can be a mild or a life-threatening sign of ANCA-associated vasculitis, but its prognostic impact and specific characteristics have not been determined. To do so, we retrospectively analyzed AH episodes that occurred, between 1991 and 2010, in patients with ANCA-associated vasculitis entered in the FVSG database.

Patients and Methods:

We selected confirmed AH cases, as assessed by the presence of hemoptysis and ground-glass images on X-rays or CT scans and/or bloody bronchoalveolar lavage fluid. Renal insufficiency was defined as creatininemia >150 mmol/l. Severity criteria were: hypoxia, Hb decline >1 g/dl over 48 h after AH occurred and requiring mechanical ventilation.


For the 65 cases (35 men and 30 women) analyzed, their mean age at the 1st AH episode was 53 years. ANCA-associated vasculitis diagnoses were: 55% Wegener's granulomatosis (WG), 34% microscopic polyangiitis (MPA) and 9% Churg–Strauss syndrome (CSS). Pre-AH hemoptysis occurred in 41 (63%) patients: 39% the week preceding, 29.3% 2–4 weeks before and 19.5% >1 month prior to AH diagnosis, which led to ANCA-associated vasculitis diagnosis for 45 (69.2%) patients. Among 10 (15.4%) patients (4 WG, 4 MPA, 2 CSS) requiring mechanical ventilation, 4 had hemoptysis previously. Twenty-three (35%) patients were hypoxic and 23 (35%) had major Hb decreases; 36 (55%) had concomitant renal insufficiency (pulmo-renal syndrome): 18 WG (51% of all WG) and 18 MPA (81% of all MPA). Paucimmune glomerulonephritis was seen in 31/33 kidney biopsies. Intriguingly, no CSS patient had renal impairment when AH occurred.

Treatments included a combination of corticosteroids and intravenous cyclophosphamide (CYC) for 55 (85%) patients; oral CYC was used in 5, methotrexate in 4 and plasma exchange in 7. Rituximab and remicade were prescribed to 1 patient each. Mean time between AH and treatment onset was 5.87 (range 0–60) days. Mean follow-up was 8.3 years (range 1 month–19 years). Follow-up information was not available for 8 patients. A total of 38 (58%) patients relapsed: 7 with AH and other organ involvement, 21 with non-AH manifestation(s) and 10 with AH alone. Three patients underwent kidney transplantation.

The 13 (20%) deaths (6 WG and 7 MPA) were attributed to: septic shock for 4, lung cancer for 2, and 1 each of the following: bladder cancer, AH, sudden death, hemorrhagic stroke, ARDS due to misplaced nasogastric tube and probably drug-related (methotrexate). Although 9/13 patients with pulmo-renal syndrome died, no death resulted directly from the initial AH.


These findings highlight the importance of recognizing premonitory hemoptysis as an early sign of AH in ANCA-associated vasculitides, since 63% of the cohort patients had bloody sputum before AH was diagnosed. Also, patients with AH and renal insufficiency have poorer survival prognoses. As demonstrated by the five-factor score, AH alone is not predictive poor prognosis; conversely, kidney involvement dictates a poor outcome.

To cite this abstract, please use the following information:
Kostianovsky, Alex M., Hauser, Thomas, Pagnoux, Christian, Cohen, Pascal, Daugas, Eric, Mouthon, Luc, et al; Alveolar Hemorrhage (AH) in ANCA-Associated Vasculitis: Characteristics and Prognostic Factors in 65 Patients. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :2009
DOI: 10.1002/art.29774

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