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Glucocorticoid-dependent Tubulointerstitial Nephritis with IgM-positive Plasma Cells Presenting with Intracellular Crystalline Inclusions within the Rough Endoplasmic Reticulum.

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.
AuthorsMasanori Minato, Taichi Murakami, Naoki Takahashi, Hiroyuki Ono, Kenji Nishimura, Masanori Tamaki, Kojiro Nagai, Hideharu Abe, Masayuki Iwano, Kensuke Joh, Toshio Doi
JournalInternal medicine (Tokyo, Japan) (Intern Med) Vol. 60 Issue 19 Pg. 3129-3136 (Oct 01 2021) ISSN: 1349-7235 [Electronic] Japan
PMID33840699 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Glucocorticoids
  • Immunoglobulin M
Topics
  • Endoplasmic Reticulum, Rough
  • Glucocorticoids
  • Humans
  • Immunoglobulin M
  • Male
  • Middle Aged
  • Nephritis, Interstitial
  • Plasma Cells

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