Abstract IgA nephropathy is characterized by the deposition of IgA antibodies within the renal mesangium, leading to progressive glomerular injury. Pulmonary involvement is uncommon in this disease. In contrast, pulmonary-renal syndromes are most often associated with ANCA-associated vasculitides. Diffuse alveolar hemorrhage (DAH) is a life-threatening manifestation of pulmonary-renal syndromes, and prompt recognition and treatment are key determinants of outcomes. We present a rare case of DAH associated with IgA nephropathy. A 27-year-old man with a history of IgA nephropathy, end-stage renal disease on hemodialysis (Monday/Wednesday/Friday), hypertension, and prior transient ischemic attack presented with new-onset hypoxia noted on home pulse oximetry. He reported progressive dyspnea and a productive cough without hemoptysis, fever, or chills. Chest CT revealed bilateral ground-glass opacities, raising concern for community-acquired pneumonia versus DAH. Bronchoscopy demonstrated progressively bloodier aliquots on bronchoalveolar lavage, confirming the diagnosis of DAH. The patient was started on pulse-dose corticosteroids. Despite initial therapy, his respiratory status worsened, progressing to acute hypoxic respiratory failure requiring mechanical ventilation. Given his known IgA nephropathy and the new onset of DAH, an IgA-mediated pulmonary-renal vasculitis was suspected. He subsequently underwent five sessions of plasma exchange (PLEX) in addition to corticosteroids. His respiratory status improved, allowing successful extubated, and discharged on a prednisone taper. DAH secondary to IgA nephropathy is exceedingly rare and may be underrecognized. The clinical presentation can mimic pneumonia, pulmonary edema, or volume overload—especially in patients with end-stage renal disease—leading to diagnostic delays. A high index of suspicion is essential in patients with known IgA nephropathy who develop unexplained hypoxia or diffuse infiltrates. Early recognition and aggressive immunosuppressive therapy, including corticosteroids and plasma exchange, can be life-saving and significantly improve outcomes. This abstract is funded by: None
Vuchula et al. (Fri,) studied this question.