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Abstract: PO1792

Clinicopathological Analysis of Renal Dysfunction due to Idiopathic Multicentric Castleman Disease in Japan

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Mizuno, Hiroki, Toranomon Hospital, Minato Ward, Tokyo, Japan
  • Hoshino, Junichi, Toranomon Hospital, Minato Ward, Tokyo, Japan
  • Ubara, Yoshifumi, Toranomon Hospital, Minato Ward, Tokyo, Japan
Background

Renal dysfunction is a fatal complication of idiopathic multicentric Castleman disease. Although AA amyloidosis and glomerular endotheliopathy with severe proteinuria were reported due to this disease, human immunodeficiency virus (HIV) were positive in these previously reported cases. On the other hand, HIV-negative cases are common in Japan, and the precise renal involvement of Japanese cases has not been studied yet.

Methods

Case-series was designed for analyzing the clinicopathological features of renal dysfunction accompanied with Castleman disease. Inclusion criteria of the object is renal biopsy performed between 1990 and 2019 and the patients who were diagnosed as Castleman disease. Clinical and pathological data was collected from the electrical medical record.

Results

Eight patients were eligible to the study. Seven out of eight cases were plasma cell type, while anti-HIV antigen/antibody was negative in all cases. Laboratory data at the time of renal biopsy showed; median serum creatinine was 0.75(0.6-5.0) mg/dL, urine protein was 1.28(0.04-8.9) mg/dL. In glomerular lesion, following involvement were found: two cases of AA amyloidosis and membranous nephropathy respectively, while IgA deposition and nephrosclerosis in one case each. Nephrotic range proteinuria was found in two cases of AA amyloidosis as well as one case of membranous nephropathy. Among two cases diagnosed as membranous nephropathy, immunofluorescent analysis about IgG subclass showed that IgG1 was dominant in one case and IgG2 was in the other. IgG4 was negative in both cases. In interstitial lesion, chronic tubulointerstitial nephritis was diagnosed in one case.

Conclusion

Heterogenous glomerular lesions were found in our cohort. Previous cohort study showed that membranous nephropathy was rare, but in this study, two out of eight cases turned out to be membranous nephropathy. In addition, analysis of IgG subclass suggests that either IgG1 or IgG2 was dominant in secondary membranous nephropathy due to Castleman disease and that deposition of immunoglobulin complex could be associated to the onset of proteinuria of Castleman disease.