Recent reports have indicated that a significant number of
immune complex glomerulonephritis (GN) cases are associated with antineutrophilic cytoplasmic antibody (
ANCA). However, most of the reported cases were associated with underlying primary glomerular diseases. When primary glomerular diseases were not found, immune deposits tended to be non-specific and the level of
ANCA is usually borderline. We report here upon a case of life-threatening
pulmonary-renal syndrome manifested simultaneously with
immune complex GN and
myeloperoxidase (MPO)-
ANCA seropositivity. A 29- year-old man was admitted with pulmonary
hemorrhage and rapidly progressing renal dysfunction. On admission,
ANCA revealed perinuclear staining with a titer of 1:160. The MPO-
ANCA level was 59 IU by ELISA. Other serologic markers including ANA, anti-
DS-DNA and
anti-GBM Ab were negative. Renal biopsy showed cellular crescents in eight of 18 glomeruli. Immunofluorescence staining showed strong granular deposits of C3, C1q,
IgG and
IgM in the capillary loop and the mesangium. Electron microscopy showed multifocal electron dense deposits scattered in the mesangium, paramesangium, and the subendothelial and subepithelial areas. The patient initially responded to
steroid and
cyclophosphamide. MPO-
ANCA decreased to less than 10 IU. Twenty three days after hospital discharge, the patient was re-admitted urgently with
fever, generalized papulonodular skin lesions, and a recurrence of massive pulmonary
hemorrhage and renal dysfunction. He died from uncontrolled pulmonary
hemorrhage and
respiratory insufficiency.
P-ANCA titer and MPO-
ANCA level at the second admission were 1:320 and 82 U/ml respectively. Interestingly, relapse was shown to be triggered by
varicella zoster infection.