Diffuse Alveolar Hemorrhage Secondary to Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: Predictors of Respiratory Failure and Clinical Outcomes

Abstract

Objective. To identify predictors of respiratory failure and to evaluate the therapeutic efficacy of plasma exchange (PE) and of rituximab versus cyclophosphamide in a cohort of patients with diffuse alveolar hemorrhage (DAH) secondary to antineutrophil cytoplasmic antibody-associated vasculitis (AAV) with or without respiratory failure.Methods. We performed a single-center historical cohort study of all consecutive patients with AAVassociated DAH who were evaluated over a 16-year period. Logistic regression models were developed to examine the predictive role of the baseline clinical characteristics for the development of respiratory failure, and for the effect of PE and remission induction therapy on the main outcome (complete remission at 6 months).Results. Seventy-three patients with DAH were identified, and 34 of them experienced respiratory failure. The degree of hypoxemia upon initial presentation, a higher percentage of neutrophils in the bronchoalveolar lavage fluid cell count, and higher C-reactive protein levels were independently associated with the development of respiratory failure. PE was not associated with achieving complete remission at 6 months, with an odds ratio (OR) of 0.49 (95% confidence interval [95% CI] 0.12-1.95) (P 5 0.32). Rituximab treatment was independently associated with achieving complete remission at 6 months (OR 6.45 [95% CI 1.78-29], P 5 0.003).Conclusion. Our findings indicate that the most important predictor of respiratory failure in patients with DAH secondary to AAV is the degree of hypoxemia upon presentation. No clear benefit of the addition of PE to standard remission induction therapy was demonstrated. Complete remission by 6 months was achieved at a higher rate with rituximab than with cyclophosphamide in patients with DAH secondary to AAV, including those needing mechanical ventilation.

Legionella pneumonia associated with severe acute respiratory distress syndrome and diffuse alveolar hemorrhage - A rare association

“…Intravenous methylprednisolone up to 500 mg every 6 hours for 4–5 days is recommended by most experts, although lower doses seem to have similar efficacy, followed by a gradual taper to maintenance doses of oral steroids [24]. Based on the severity of the disease, underlying etiology and organ involvement, plasma exchange or other immunosuppressive drugs such as cyclophosphamide, azathioprine, methotrexate, mycophenolate mofetil, etanercept may be indicated [25], [26], [27].…”

Chronic and Asymptomatic Diffuse Alveolar Haemorrhage with Microscopic Polyangiitis: A Case Report and Review of the Literature

“…Fibreoptic bronchoscopy, particularly with BALF evaluation, is the best method to specifically diagnose DAH and for excluding other diseases [10]. Some studies of DAH patients with AAV showed that almost all patients have respiratory symptoms, such as dyspnoea and haemoptysis [3, 11, 12]. Additionally, DAH progresses rapidly and causes acute respiratory failure, such that the patients sometimes require admission to the Intensive Care Unit (ICU).…”

“…Additionally, DAH progresses rapidly and causes acute respiratory failure, such that the patients sometimes require admission to the Intensive Care Unit (ICU). Cartin-Ceba et al reported that 34 of 73 patients with DAH secondary to AAV (47%) underwent mechanical ventilation (MV), and 41 patients (56%) were admitted to the ICU [11]. Moreover, DAH was reported to be an independent prognostic factor of MPA and the mortality rate of MPA patients with DAH is 8.65 times greater than that of MPA patients without DAH [5].…”

“…Regarding the relationship between the disease activity of AAV and biomarkers, Kronbichler et al reported that CRP titre was one of the important factors of ANCA-associated disease activity [15]. Moreover, DAH patients with MPA without acute respiratory failure often demonstrate significantly lower CRP levels than patients with acute respiratory failure [11]. These data suggest that there may be rare phenotypes of DAH with MPA, which manifest with less inflammation and lower disease activity.…”

S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

“…Neuere Daten zeigen jedoch keinen eindeutigen Nutzen der Plasmaseparation bei alveolärer Hämorrhagie [28]. Bei schwerer alveolärer Hämorrhagie kann zusätzlich eine Plasmaseparation erwogen werden.…”