title: Cryoglobulinemic vasculitis with primary Sjögren's syndrome: A case report.
abstract: A 63-year-old Japanese woman with Sjögren's syndrome and peripheral neuropathy was admitted for evaluation of purpura on her lower extremities. Skin biopsy revealed leukocytoclastic vasculitis with the deposition of IgM, and serum cryoglobulin was positive. Accordingly, cryoglobulinemic vasculitis was diagnosed. There was no response to high-dose steroid therapy and plasmapheresis, but intravenous cyclophosphamide pulse therapy was effective for 4 years. Thereafter, proteinuria and hematuria developed, with cryoglobulinemic glomerulopathy being diagnosed by renal biopsy. Because the total dose of cyclophosphamide had reached 8000 mg, treatment with rituximab was selected. While rituximab was initially effective for her skin lesions and nephropathy, relapse occurred within 2 years and additional administration of this agent was required. The long-term efficacy of treatment for cryoglobulinemic vasculitis remains uncertain in patients with Sjögren's syndrome.
authors.affiliation: a Nephrology Center and Department of Rheumatology , Toranomon Hospital , Tokyo , Japan.|b Department of Nephrology , Ohkubo Hospital , Tokyo , Japan.
authors.forename: Jumpei
authors.initials: J
authors.lastname: Hasegawa
authors.affiliation: a Nephrology Center and Department of Rheumatology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Hayami
authors.affiliation: a Nephrology Center and Department of Rheumatology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Junichi
authors.initials: J
authors.lastname: Hoshino
authors.affiliation: a Nephrology Center and Department of Rheumatology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Suwabe
authors.affiliation: a Nephrology Center and Department of Rheumatology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Keiichi
authors.initials: K
authors.lastname: Sumida
authors.affiliation: a Nephrology Center and Department of Rheumatology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Koki
authors.initials: K
authors.lastname: Mise
authors.affiliation: a Nephrology Center and Department of Rheumatology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Toshiharu
authors.initials: T
authors.lastname: Ueno
authors.affiliation: a Nephrology Center and Department of Rheumatology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Yamanouchi
authors.affiliation: a Nephrology Center and Department of Rheumatology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Sawa
authors.affiliation: c Department of Pathology , Toranomon Hospital , Tokyo , Japan , and.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ohashi
authors.affiliation: c Department of Pathology , Toranomon Hospital , Tokyo , Japan , and.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Fujii
authors.affiliation: a Nephrology Center and Department of Rheumatology , Toranomon Hospital , Tokyo , Japan.|d Okinaka Memorial Institute for Medical Research , Tokyo , Japan.
authors.forename: Kenmei
authors.initials: K
authors.lastname: Takaichi
authors.affiliation: a Nephrology Center and Department of Rheumatology , Toranomon Hospital , Tokyo , Japan.|d Okinaka Memorial Institute for Medical Research , Tokyo , Japan.
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Ubara
mesh_terms: D003449:Cryoglobulinemia / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008875:Middle Aged; D000069283:Rituximab / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D012859:Sjogren's Syndrome / Q000150:complications* / Q000473:pathology; D014657:Vasculitis / Q000150:complications / Q000188:drug therapy* / Q000473:pathology
chemical_list: D000069283:Rituximab; D003520:Cyclophosphamide
keywords: Cryoglobulin; Cryoglobulinemic vasculitis; Intravenous cyclophosphamide pulse therapy; Rituximab; Sjögren’s syndrome