title: Rituximab Treatment for PR3-ANCA-Positive Membranoproliferative Glomerulonephritis Associated with Adult-Onset Periodic Fever Syndrome.
abstract: We report the case of a 36-year-old Japanese woman with nephrotic syndrome due to membranoproliferative glomerulonephritis (MPGN) Type I diagnosed after a 5-year history of periodic fever syndrome (PFS). Hypocomplementemia and elevation of anti-proteinase 3 anti-neutrophil cytoplasmic autoantibody (PR3-ANCA) were observed. HIV, and hepatitis B and C serology were negative. Nephrotic syndrome and periodic fever did not respond to oral steroid and intravenous steroid pulse therapies combined with cyclosporine, dipyridamole, warfarin and losartan. We tried immunotherapy using rituximab, a human-mouse chimeric monoclonal antibody directed against the CD20 antigen on mature B cells. This therapeutic approach led to improvement of renal function and remission of nephrotic syndrome and hypocomplementemia. However, it did not have a beneficial effect on periodic fever. Suspecting adult-onset hereditary PFS, we analyzed her genetic alteration of MEFV and TNFRSF1A genes. A rare genotype in intron 6 of TNFRSF1A was revealed. The etiological relationship between periodic fever and MPGN is discussed. Rituximab is a hopeful choice of induction therapy for refractory MPGN.
authors.affiliation: Division of Nephrology, Department of Internal Medicine, Jichi Medical University Hospital, Shimotsuke, Tochigi, Tochigi, Japan ; Department of Nephrology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo, Tochigi, Japan.
authors.forename: Yoshitomo
authors.initials: Y
authors.lastname: Hamano
authors.forename: Hiromichi
authors.initials: H
authors.lastname: Yoshizawa
authors.forename: Taro
authors.initials: T
authors.lastname: Sugase
authors.forename: Takuya
authors.initials: T
authors.lastname: Miki
authors.forename: Naoko
authors.initials: N
authors.lastname: Ohtani
authors.forename: Shiho
authors.initials: S
authors.lastname: Hanawa
authors.forename: Eri
authors.initials: E
authors.lastname: Takeshima
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Morishita
authors.forename: Osamu
authors.initials: O
authors.lastname: Saito
authors.forename: Fumi
authors.initials: F
authors.lastname: Takemoto
authors.forename: Shigeaki
authors.initials: S
authors.lastname: Muto
authors.forename: Wako
authors.initials: W
authors.lastname: Yumura
authors.forename: Eiji
authors.initials: E
authors.lastname: Kusano