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Combined membranous nephropathy and tubulointerstitial nephritis as a rare renal manifestation of IgG4-related disease: a case-based literature review

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Abstract

IgG4-related disease (IgG4-RD) is a newly recognized immune-mediated multisystemic disease characterized by a fibro-inflammatory condition with tissue infiltration of IgG4-positive plasma cells and often associated with elevated serum IgG4 levels. Typical renal involvement of IgG4-RD presents as tubulointerstitial nephritis (TIN), membranous or membranoproliferative nephropathy. We are presenting a case with combined IgG4 membranous nephropathy and TIN, as well as a literature review on pathophysiology, diagnosis and treatment of IgG4-RD. A 62-year-old man presented with weight loss and fatigue. Labs showed significant proteinuria and hematuria with elevated serum creatinine (2.5 mg/dL). CT/PET scan found scattered lymphadenopathy without increased FDG uptake. Kidney biopsy showed glomerular lesions as well as severe interstitial fibrosis and tubular atrophy. Immunohistochemistry study was negative for anti-phospholipase A2 receptor antibodies and showed interstitial lymphocytic infiltration with IgG4 positive plasma cells. Patient also had elevated serum IgG4 level and IgG4 to total IgG ratio. Prednisone treatment was initiated soon after the diagnosis was made, patient responded well with proteinuria and hematuria both resolved. IgG4-related disease (IgG4-RD) is a newly increasingly recognized immune-mediated multisystemic disease; IgG4-related membranous nephropathy should be included in the differential diagnosis for patients with proteinuria.

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Authors and Affiliations

  1. Internal Medicine Residency Program, Brookwood Baptist Health, 817 Princeton Ave. SW, Birmingham, AL, 35211, USA

    Wei Zhang, Jeffrey H. Glaze & David Wynne

  2. Nephrology Associates, P.C., 817 Princeton Avenue S.W., Birmingham, AL, 35211, USA

    Jeffrey H. Glaze

  3. Summit Internal Medicine, 3570 Grandview Pkwy #100a, Birmingham, AL, 35243, USA

    David Wynne

Authors
  1. Wei Zhang
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  2. Jeffrey H. Glaze
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  3. David Wynne
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Correspondence to Wei Zhang or David Wynne.

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The authors have no conflicts of interests to disclose. Consent, for the publication of this case report and any additional related information, was taken from the patient involved in the study. This article does not contain any studies with human participants or animals performed by any of the authors.

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Zhang, W., Glaze, J.H. & Wynne, D. Combined membranous nephropathy and tubulointerstitial nephritis as a rare renal manifestation of IgG4-related disease: a case-based literature review. CEN Case Rep 7, 137–142 (2018). https://doi.org/10.1007/s13730-018-0311-8

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  • DOI: https://doi.org/10.1007/s13730-018-0311-8

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