Abstract: PUB552
Diffuse Alveolar Hemorrhage: A Rare Manifestation of IgA Nephropathy
Session Information
Category: Trainee Case Report
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Hu, Jiun-Ruey, Vanderbilt University Medical Center, Nashville, Tennessee, United States
- Kovach, Cassie, Vanderbilt University Medical Center, Nashville, Tennessee, United States
- Burgner, Anna Marie, Vanderbilt University Medical Center, Nashville, Tennessee, United States
Introduction
Pulmonary Renal Syndrome (PRS) describes combined renal and respiratory failure, often in the setting of glomerulonephritis (GN) with diffuse alveolar hemorrhage (DAH). IgA Nephropathy (IgAN) is the most common GN worldwide, but rarely causes DAH, and can be overlooked as a cause of PRS. We describe a case of PRS from IgAN successfully treated with plasmapheresis and glucocorticoids.
Case Description
A 71-year-old woman with hypertension and diabetes developed proteinuria and hematuria and her creatinine rose from 1.0 to 2.0 mg/dL over 9 months. Renal biopsy showed IgA nephropathy with 40-50% tubulointerstitial fibrosis and 15/33 glomeruli globally sclerosed. MEST-C score was M1, E1, S1, T1, C0. She was treated with maximum dose lisinopril. She had no findings to suggest an IgA vasculitis at the time of biopsy.
Three months later she presented with dyspnea, cough, and fevers. Chest x-ray showed multifocal infiltrates concerning for pneumonia. Despite antibiotics and diuresis, she developed worsening hypoxic respiratory failure and acute kidney injury. Renal replacement therapy was started for volume removal with no improvement in respiratory failure. A bronchoscopy was diagnostic for DAH. Other vasculitic workup was negative, leaving IgAN as the etiology. With high dose steroids and 7 sessions of plasmapheresis, renal function improved, dialysis was stopped, proteinuria resolved, lung infiltrates cleared, and breathing returned to normal.
Discussion
Proposed mechanisms for DAH in IgAN include nonspecific mucosal hemorrhage, immune complex-mediated damage of the GBM (Type III hypersensitivity), or IgA-mediated capillaritis against GBM antigens (Type II hypersensitivity). Type II hypersensitivity is most likely given elevated IgA levels, deposition of IgA in lung tissue, and case reports of IgA deposits on the skin and circulating IgA1 immune complexes.
The prognosis of PRS due to IgAN is variable. It is fatal in a quarter of patients and leads to end-stage kidney disease in half of patients. Recurrence of disease has also been reported.
Given its rarity, there are no treatment guidelines. Patients are usually given glucocorticoids and sometimes methotrexate, cyclophosphamide, or azathioprine. Plasmapheresis has also been used in treatment.