Diffuse Lung Disease: Student/Resident Case Report Poster - Diffuse Lung Disease |

Advances in Management of Diffuse Alveolar Hemorrhage in ANCA-Associated Vasculitis FREE TO VIEW

Gwen Thompson, MD; Rodrigo Cartin-Ceba, MD; Ulrich Specks, MD
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Mayo Clinic, Rochester, MN

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):536A. doi:10.1016/j.chest.2016.08.550
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SESSION TITLE: Student/Resident Case Report Poster - Diffuse Lung Disease

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a potential complication of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). The clinical course of these patients varies making triage difficult. Herein, we describe an AAV case that presented with DAH and progressed to respiratory failure. Recent literature will be applied to outline clues to predict respiratory failure in this patient and treatment recommendations will be discussed.

CASE PRESENTATION: A 21 year old male with granulomatosis with polyangiitis presented with a cough and dyspnea. Chest x-ray demonstrated bilateral pulmonary infiltrates. He was saturating 93% on room air and labs demonstrated a hemoglobin of 11.0 g/dL. He was admitted to the general floor. Bronchoscopy was completed and demonstrated progressively bloodier return confirming diffuse alveolar hemorrhage. BAL showed 66% neutrophils. He progressed to respiratory failure and required intubation. He was treated with high-dose glucocorticoids, plasma exchange and rituximab and responded to therapy with no recurrences of DAH or flares of his disease.

DISCUSSION: DAH is potentially life-threatening. In one study of patients with AAV and DAH 47% required mechanical ventilation and there was an 11% mortality rate.1 Factors that predicted respiratory failure in DAH included Sp02/Fi02 <450 at presentation, >30% neutrophils on BAL and CRP >25 mg/dl with the Sp02/Fi02 ratio having the greatest predictive value. This patient had signs of impending respiratory failure with a Sp02/Fi02 ratio of 442 on admission and 66% neutrophils present on BAL. With this new evidence, consideration may have been given to triage this patient to a higher level of care on admission and promptly initiate therapy. There is little evidence regarding choice of induction agent in patients with AAV and DAH who require mechanical ventilation. One recent study suggests similar mortality rates and increased remission rates when rituximab compared to cyclophosphamide is used for induction in this patient population.1 The evidence for plasma exchange for DAH is limited with only a few retrospective studies with conflicting results published to date. The ongoing PEXIVAS trial will add insight into its utility.

CONCLUSIONS: DAH is a severe disease manifestation of AAV that requires prompt definitive treatment. The Sp02/Fi02 ratio is the strongest predictor for progression to respiratory failure and should be used to triage patients. Rituximab is at least as effective as cyclophosphamide for remission induction in patients with DAH, even those in respiratory failure. The role of PLEX for DAH remains unknown. This is currently being explored by the ongoing PEXIVAS trial.

Reference #1: Cartin-Ceba R, Diaz-Caballero L, Alqadi MO, Tryfon S, Fervenza FC, Ytterberg SR, Specks U. Diffuse alveolar hemorrhage secondary to ANCA-associated vasculitis: Predictors of respiratory failure and clinical outcomes. Arthritis Rheumatol. 2015;29.

DISCLOSURE: Ulrich Specks: Grant monies (from industry related sources): Genentech <$10,000 in 2016 The following authors have nothing to disclose: Gwen Thompson, Rodrigo Cartin-Ceba

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