Open AccessIdiopathic membranous nephropathy in a patient diagnosed with IgG4-related disease
Author(s) -
Xiaoying Ma,
Haiping Xu,
Jing Sun,
Yuresha Surangani Siyabalagaba Gedara,
Fuyun Sun
Publication year - 2020
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000022817
Subject(s) - medicine , igg4 related disease , membranous nephropathy , proteinuria , nephrotic syndrome , monoclonal gammopathy of undetermined significance , retroperitoneal fibrosis , pathology , gastroenterology , kidney , fibrosis , immunology , monoclonal , antibody , monoclonal antibody
Rationale: Immunoglobulin (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 concerns: A 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%. Diagnosis: The patient was diagnosed with nephrotic syndrome, acute kidney injury, and MGUS. The pathological diagnosis was IgG4-related tubulointerstitial nephritis, IgG4-related membranous nephropathy. Interventions: The patient was treated with intravenous methylprednisolone (40 mg daily), which was changed to oral prednisone 50 mg/d after 2 months. Outcomes: After 1 month, the patient exhibited a rapid response only with corticosteroid, and experienced partial remission of serum albumin and proteinuria. Lessons: This case may suggest a possible relationship between IgG4-RD and MGUS, provide some guidance for investigating the mechanism between them.