Diffuse Lung Disease: Fellow Case Report Poster - Diffuse Lung Disease I |

An Unusual Cause of Respiratory Failure in a 29-Year-Old Male FREE TO VIEW

Michael Wert, MD; Maria Lucarelli, MD
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Ohio State University Medical Center, Columbus, OH

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

Chest. 2016;150(4_S):486A. doi:10.1016/j.chest.2016.08.500
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SESSION TITLE: Fellow Case Report Poster - Diffuse Lung Disease I

SESSION TYPE: Affiliate Case Report Poster

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

INTRODUCTION: Goodpasture's disease is classically thought to be a syndrome of both pulmonary hemorrhage and glomerulonephritis. In rare instances, however, pulmonary involvement is the predominant or sole manifestation of the disease. In patients with diffuse alveolar hemorrhage (DAH), a high index of suspicion for Goodpasture's disease must be maintained, regardless of the presence of glomerulonephritis, as untreated disease can have catastrophic consequences.

CASE PRESENTATION: A 29 year old male presented with chest pain, hemoptysis, and shortness of breath. On presentation, he was markedly hypoxic. Initial labs revealed a marked anemia and normal renal function. Chest radiograph revealed bilateral infiltrates and chest CT showed diffuse groundglass opacities. He was started on empiric antibiotics. Urgent bronchoscopy was performed with bronchoalveolar lavage (BAL) findings consistent with DAH. Infectious work-up from the BAL did demonstrate an Aspergillus antigen of 3.2 (negative < 0.5). He worsened clinically and ultimately required mechanical ventilation. On day 5, his anti-glomerular basement membrane (anti-GBM) antibody came back markedly positive at 2.2 (negative < 1). He underwent emergent plasmapheresis and was started on methylprednisolone. Cyclophosphamide was held due to concerns for aspergillus infection. He was quickly weaned from the ventilator and discharged home with no oxygen requirement. His kidney function remained normal throughout hospitalization. He is currently being maintained on a prolonged prednisone taper. He remains asymptomatic with no renal involvement.

DISCUSSION: Anti-GBM disease is relatively rare (incidence of 0.5-1.8 cases/1 mil/yr), with <10% cases consisting of pulmonary involvement alone. This case demonstrates the need to maintain a high index of suspicion for anti-GBM disease with alveolar hemorrhage even in the setting of normal renal function. Rapid initiation of plasmapheresis and immunusuppressive therapy can prevent adverse outcomes and may need to be started prior to confirmation of the presence of anti-GBM antibodies. Anti-GBM testing has a high specificity and is important to confirm the diagnosis. Howevery, therapy should be instituted in life-threatening disease while work-up and anti-GBM testing are underway.

CONCLUSIONS: Regardless of presence of renal involvement, Goodpasture's disease must remain on the differential in patients presenting with DAH.

Reference #1: Sinico,RA. Anti-GBM antibodies in the diagnosis of Goodpasture syndrome. Nephrol Dial Transplant. 2006;21(2):397

DISCLOSURE: The following authors have nothing to disclose: Michael Wert, Maria Lucarelli

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