Membranous nephropathy with repeated flares in IgG4-related disease

Hiroko Kanda; Junji Koya; Hiroshi Uozaki; Shoko Tateishi; Kojiro Sato; Noboru Hagino; Tetsuji Sawada; Kazuhiko Yamamoto

doi:10.1093/ckj/sfs180

Membranous nephropathy with repeated flares in IgG4-related disease

Clin Kidney J. 2013 Apr;6(2):204-207. doi: 10.1093/ckj/sfs180. Epub 2013 Feb 19.

Authors

Hiroko Kanda¹, Junji Koya², Hiroshi Uozaki³, Shoko Tateishi¹, Kojiro Sato⁴, Noboru Hagino⁵, Tetsuji Sawada⁶, Kazuhiko Yamamoto¹

Affiliations

¹ Department of Allergy and Rheumatology , University of Tokyo , Tokyo , Japan.
² Department of Hematology and Oncology , Graduate School of Medicine , University of Tokyo , Tokyo , Japan.
³ Department of Pathology , University of Tokyo , Tokyo , Japan.
⁴ Department of Rheumatology and Applied Immunology, Faculty of Medicine , Saitama Medical University , Saitama , Japan.
⁵ Department of Hematology and Rheumatology , Teikyo University Chiba Medical Center , Ichihara , Japan.
⁶ Department of Rheumatology , Tokyo Medical University Hospital , Tokyo , Japan.

Abstract

IgG4-related disease (IgG4-RD) is associated with the infiltration of IgG4-positive plasma cells into various organs. Nephropathy of IgG4-RD is generally interstitial nephritis and glomerulonephritis is rare. We describe a case of membranous nephropathy (MN) without interstitial nephritis associated with IgG4-RD symptoms including lymphadenopathy and pulmonary and pleural lesions. Treatment with steroids improved these clinical symptoms, but withdrawal of steroids induced the repeated relapse of MN. Finally, flaring of MN was prevented by the combination of steroids and cyclosporine. This is the first report of the successful treatment of MN associated with IgG4-RD by this combination therapy.

Keywords: IgG4-related disease; cyclophosphamide; cyclosporine A; flares; membranous nephropathy.