Antiglomerular basement membrane disease associated with Castleman disease

We described a 33-year-old man with gross hematuria and acute renal failure, who had suffered from fever for 6 months, multiple cervical lymph node swelling, splenomegaly, left-sided pleural effusion. He also suffered from anemia, thrombocytopenia, hypergammaglobulinemia, and his serum interleukin-6 levels were markedly elevated. Antiglomerular basement membrane antibodies were positive in the patient's serum. Lymph node biopsy results were compatible with Castleman disease of "plasma cell" variant. Renal biopsy revealed cellular crescents in most of the glomeruli. Immunofluorescence studies showed strong deposition of IgG in a linear pattern along the glomerular basement membrane. Pathologic features were compatible with crescentic glomerulonephritis because of antiglomerular basement membrane disease. With intensive plasmapheresis and monthly chemotherapy (cyclophosphamide, oncovin, prednisone regimen), the patient experienced clinical and biochemical remission. Although autoimmune phenomenon had been described frequently in Castleman disease, to the best of our knowledge, this was the first report that the patient with rapid progressive glomerulonephritis mediated by antiglomerular basement membrane antibodies, which might be associated with Castleman disease.SCI(E)PubMed0ARTICLE3206-933