Recurrent diffuse alveolar hemorrhage in Immunoglobulin A vasculitis

Immunoglobulin A vasculitis (IgAV) is a small-vessel immune complex-mediated vasculitis that predominantly affects children, with significantly fewer cases reported in adults. When IgAV does occur in adults, it often presents with more severe systemic involvement and worse renal outcomes compared to pediatric populations. A rare pulmonary complication of IgAV is diffuse alveolar hemorrhage (DAH). We present a case of recurrent hypoxic respiratory failure with DAH in a patient found to have biopsy proven IgAV.

A man in his 60s was admitted with recurrent hypoxia following recent hospitalization for presumed pneumonia. Shortly after admission, he experienced cardiac arrest. He was intubated and had a return of spontaneous circulation. He was transferred to the ICU and underwent bronchoscopy with bronchoalveolar lavage (BAL). This revealed bloody aliquots and eosinophilia. Infectious workup was negative. Autoimmune serologies were negative. Serum inflammatory markers were elevated. Worsening kidney function raised concern for a pulmonary-renal syndrome and high-dose intravenous methylprednisolone was initiated empirically with improvement in clinical status. Renal biopsy was performed which confirmed IgA vasculitis. After seven days, the patient returned to the hospital with recurrent hypoxia and a new cavitary lung lesion. Repeat bronchoscopy with BAL was performed which was concerning for DAH. The patient was initiated on high dose IV steroids and IV cyclophosphamide. Shortly after, the patient elected for comfort care and was discharged to in-patient hospice.

This case underscores the importance of timely immunosuppressive therapy and multidisciplinary care. However, prognosis remains poor in patients with severe systemic involvement.