Glucocorticoid-dependent Tubulointerstitial Nephritis with IgM-positive Plasma Cells Presenting with Intracellular Crystalline Inclusions within the Rough Endoplasmic Reticulum
Open Access
- 1 October 2021
- journal article
- research article
- Published by Japanese Society of Internal Medicine in Internal Medicine
- Vol. 60 (19), 3129-3136
- https://doi.org/10.2169/internalmedicine.7118-21
Abstract
Tubulointerstitial nephritis (TIN) with IgM-positive plasma cells (IgMPC-TIN) is an autoimmune kidney disease characterized by IgM/CD138-double-positive plasma cell infiltration in the tubulointerstitium. A 50-year-old man developed IgMPC-TIN and presented with crystalline inclusions in the rough endoplasmic reticulum. Intracellular crystal formation is a rare finding in paraprotein-related kidney diseases, but this case showed no pathogenic monoclonal immunoglobulin. Prednisolone (PSL, 30 mg) improved the TIN, but PSL tapering resulted in the recurrence of TIN. Combination therapy with 15 mg PSL and 150 mg mizoribine ultimately stabilized TIN. This case offers original evidence concerning the pathophysiology and treatment strategy of IgMPC-TIN.Keywords
This publication has 41 references indexed in Scilit:
- Aggregates, Crystals, Gels, and Amyloids: Intracellular and Extracellular Phenotypes at the Crossroads of Immunoglobulin Physicochemical Property and Cell PhysiologyInternational Journal of Cell Biology, 2013
- In Vivo Crystallization of Human IgG in the Endoplasmic Reticulum of Engineered Chinese Hamster Ovary (CHO) CellsOnline Journal of Public Health Informatics, 2011
- Immunostaining of Plasma Cells in Primary Biliary CirrhosisAmerican Journal of Clinical Pathology, 2009
- Dysproteinemia-related nephropathy associated with crystal-storing histiocytosisKidney International, 2006
- Multiple myeloma, nephrotic syndrome and crystalloid inclusions in podocytesKidney International, 2006
- Peculiar Cytoplasmic Inclusions in Oncocytic Adrenal Cortical Tumors: An Electron Microscopic ObservationUltrastructural Pathology, 2002
- Adult Faconi Syndrome Secondary to Light Chain Gammopathy: Clinicopathologic Heterogeneity and Unnsual Features in 11 PatientsMedicine, 2000
- Protease resistance and binding of Ig light chains in myeloma-associated tubulopathiesKidney International, 1995
- Malakoplakia and Malakoplakialike Lesions of the Upper Gastrointestinal TractUltrastructural Pathology, 1984