RESUMEN
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 (40âmg daily), which was changed to oral prednisone 50âmg/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.