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A 42 year-old man with lumbago and fever had hydronephrosis due to a mass surrounding the abdominal aorta, associated with pachymeningitis and a suprasellar tumor. He was given a diagnosis of multifocal fibrosclerosis, involving both retroperitoneal and intracranial fibrosis. He responded to treatment with corticosteroid and cyclophosphamide. The intracranial lesions seemed to be rare, but important manifestations of the systemic disease, and were clearly visualized by magnetic resonance imaging. The phenotype of the infiltrated lymphocytes was CD4+, supporting the hypothesis that autoimmunity was involved pathogenetically. We propose a comprehensive category termed "inflammatory fibrotic disease" including both systemic and localized diseases.(Internal Medicine 42: 1006-1010, 2003)

We describe herein a case of nephrotic syndrome (NS) following allogeneic bone marrow transplantation (allo-BMT) for natural killer cell leukemia/lymphoma. Histologic studies defined the diagnosis as crescentic glomerulonephritis with massive immunoglobulin A (IgA) deposition, which has never been reported in NS cases following allo-BMT. Most of the massive infiltrated cells in the interstice were CD3+CD4−CD8+ T cells derived from the donor. We observed mesangial deposition of Haemophilus parainfluenza outer membrane (OMHP) antigen and decreased glycosylation of the IgA1 hinge in the recipient's samples is consistent with the recently reported pathogenesis of IgA nephropathy. Further, the titer of IgA antibody against the donor serum was as high as other IgA nephropathy cases. These findings suggest that NS and crescentic glomerulonephritis in this case occurred as one of the forms of chronic graft-versus-host disease (GVHD), and that IgA deposition was associated with H parainfluenza and decreased glycosylation of the IgA1 hinge.