Aimee Willett, D.O.
Resident Physician
OhioHealth Riverside Methodist Hospital, United States
Disclosure information not submitted.
Title: Goodpasture's Disease Presenting with Alveolar Hemorrhage in the Absence of Renal Manifestations
Introduction: Goodpasture's syndrome is a rare small vessel vasculitis in which circulating antibodies target type 4 collagen of glomerular and alveolar basement membranes. 90% of anti-glomerular basement membrane (GBM) disease presents as rapidly progressive glomerulonephritis. Concurrent alveolar hemorrhage occurs around 45% of the time. Only 5-10% of cases demonstrate pulmonary manifestations alone. This case details one such patient with serum positive anti-GBM disease presenting with pulmonary hemorrhage in the absence of renal pathology.
Description: A 37-year-old male with asthma, obstructive sleep apnea, and tobacco abuse presented with a two-day history of dyspnea, cough without hemoptysis, and subjective fevers. Chest computed tomography showed severe diffuse bilateral alveolar infiltrates. Creatinine and blood urea nitrogen (BUN) were at baseline, 1.02 mg/dL and 11 mg/dL respectively. Urinalysis was without the presence of blood, red blood cells (RBC), or protein. Bronchoscopy revealed blood pooling with persistent hemorrhage on serial bronchoalveolar lavage. Serology was anti-GBM antibody positive at 3.3 U. Repeat urinalysis revealed few RBC, hyaline casts, and white blood cell clumps. Creatinine remained stable at 1.04 mg/dL with an increase in BUN to 44 mg/dL. Treatment included plasma exchange, intravenous immune globulin, pulse steroids, cyclophosphamide, and rituximab. Course was complicated by progressive pulmonary hemorrhage requiring intubation, hypoxia induced cardiac arrest causing cardiogenic shock, and multifactorial acute kidney injury requiring renal replacement therapy with recovery back to baseline on discharge.
Discussion: This case demonstrates the importance of early consideration for anti-GBM disease in all patients presenting with alveolar hemorrhage regardless of initial renal function. Early diagnosis and rapid intervention are key determinants of prognosis in anti-GBM disease, especially in anti-GBM cases with creatinine levels < 5.7 mg/dL. Environmental exposures such as smoking should increase suspicion for this disease process. Overall prognosis for patients presenting with pulmonary manifestations alone is improved compared to patients with renal involvement when identified early.