Intraventricular hemorrhage as a complication of microscopic polyangiitis

A 50-year-old woman underwent partial lobectomy of left upper lobe of her lung for massive hemoptysis 1 year ago. Although bronchiectasis was initially suspected, common risk factors such as smoking or pulmonary infections were not identified. One month prior to this admission, she presented to our emergency department with fever, dry cough, hematuria, non-nephrotic proteinuria, and a rapid increase in serum creatinine (Cr) to 5.2 mg/dl. She was referred for nephrologic evaluation, and renal biopsy was advised. The patient’s condition on admission deteriorated, with multi-system manifestations of dyspnea and hemoptysis, migratory polyarthralgia, vertigo, tinnitus, and sensorineural hearing loss. The chest radiograph showed a pulmonary infiltrate of the left lower lobe, and active pulmonary hemorrhage was suspected. Laboratory data revealed serum Cr 8.8 mg/dl, hemoglobin 7.1 g/dl, cytoplasmic antineutrophil cytoplasmic antibody 0.1 IU/ml (normal), and perinuclear antineutrophil cytoplasmic antibody >177 IU/ml. Renal pathology reported necrotizing and crescentic glomerulonephritis, supporting the clinical diagnosis of microscopic polyangiitis (MPA). The patient experienced severe headaches and altered consciousness change 1 week after admission. Her Glasgow coma score dropped to E2M4V2 within hours. Brain imaging studies showed fourth intraventricular hemorrhage (IVH) and obstructive hydrocephalus resulted from a blood clot in the foramen magnum (Figures 1 and 2). Cerebral angiography disclosed no evidence of abnormalities in the large vessels leading to the diagnosis of small vessel vasculitis-associated hemorrhage (Supplementary material: Figures 1 and 2). After emergent surgery and steroid therapy, the patient was successfully weaned from temporary hemodialysis without neurologic deficits. In patients with MPA presenting neurological abnormalities, cerebral vasculitis–associated primary IVH should be considered, which may require emergent surgical intervention.