• XiaoYing Ma, HaiPing Xu, Jing Yi Sun, Yuresha Surangani Siyabalagaba Gedara, and FuYun Sun.
• Nephrology Department, Cangzhou Central Hospital, Yunhe Qu, Cangzhou, Hebei, China.
• Medicine (Baltimore). 2020 Oct 16; 99 (42): e22817e22817.

RationaleImmunoglobulin (Ig) G4-related disease (IgG4-RD) is a newly recognized, systemic disease. Membranous nephropathy is the most common glomerular lesion in IgG4- related kidney disease. However, the lack of relationship with IgG4-related kidney disease and monoclonal gammopathy of undetermined significance (MGUS) warrants investigation of the potential mechanisms.Patient ConcernsA 62-year-old patient was diagnosed with IgG4-RD, tubulointerstitial nephritis, retroperitoneal fibrosis. After 2 years, she was presented with proteinuria, hypoproteinemia, facial, and bilateral lower limb edema. Furthermore, this patient exhibited deposits of IgG k of monoclonal hyperplasia, and bone marrow plasma cell count was 2.5%.DiagnosisThe patient was diagnosed with nephrotic syndrome, acute kidney injury, and MGUS. The pathological diagnosis was IgG4-related tubulointerstitial nephritis, IgG4-related membranous nephropathy.InterventionsThe patient was treated with intravenous methylprednisolone (40 mg daily), which was changed to oral prednisone 50 mg/d after 2 months.OutcomesAfter 1 month, the patient exhibited a rapid response only with corticosteroid, and experienced partial remission of serum albumin and proteinuria.LessonsThis case may suggest a possible relationship between IgG4-RD and MGUS, provide some guidance for investigating the mechanism between them.

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