title: Visceral disseminated varicella zoster virus infection after rituximab treatment for granulomatosis with polyangiitis.
abstract: We report on a 30-year-old Japanese woman with granulomatosis with polyangiitis (GPA) complicated by pituitary diabetes insipidus and multiple lung granulomas. The granulomas disappeared with prednisolone (50 mg/day) and rituximab, although continuous nasal desmopressin was needed to control diabetes insipidus after immunosuppressive therapies. At the time of presentation, the patient had abdominal pain and disseminated intravascular coagulation but no rash. She died of continuous hemorrhage from her skin of neck, mucosa of her pharynx, and small intestine. At autopsy, varicella zoster virus (VZV)-DNA detected in serum and VZV antigens detected in tissues of her pharynx, esophagus, and liver led to a diagnosis of visceral disseminated VZV infection (VD-VZV). She also complicated cytomegalovirus infection in her stomach and ovaries. Her posterior pituitary gland had been replaced by foamy macrophages. In 38 reported cases of VD-VZV, rash appeared following the onset of abdominal pain (mean interval, 6.5 days) but was lacking in 11% of cases. The mortality rate associated with VD-VZV was as high as 29% and survived cases were treated with antivirals earlier than mortal cases. A quick diagnosis with detection of VZV-DNA or VZV antigens in sera or tissues using PCR or immunohistochemistry examination and early empirical treatment with antivirals are important.
authors.affiliation: a Department of Rheumatology and Clinical Immunology , Graduate School of Medicine, Kyoto University , Kyoto , Japan.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Tsuji
authors.affiliation: a Department of Rheumatology and Clinical Immunology , Graduate School of Medicine, Kyoto University , Kyoto , Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Yoshifuji
authors.affiliation: a Department of Rheumatology and Clinical Immunology , Graduate School of Medicine, Kyoto University , Kyoto , Japan.
authors.forename: Takao
authors.initials: T
authors.lastname: Fujii
authors.affiliation: a Department of Rheumatology and Clinical Immunology , Graduate School of Medicine, Kyoto University , Kyoto , Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Matsuo
authors.affiliation: a Department of Rheumatology and Clinical Immunology , Graduate School of Medicine, Kyoto University , Kyoto , Japan.
authors.forename: Ran
authors.initials: R
authors.lastname: Nakashima
authors.affiliation: a Department of Rheumatology and Clinical Immunology , Graduate School of Medicine, Kyoto University , Kyoto , Japan.
authors.forename: Yoshitaka
authors.initials: Y
authors.lastname: Imura
authors.affiliation: a Department of Rheumatology and Clinical Immunology , Graduate School of Medicine, Kyoto University , Kyoto , Japan.
authors.forename: Naoichiro
authors.initials: N
authors.lastname: Yukawa
authors.affiliation: a Department of Rheumatology and Clinical Immunology , Graduate School of Medicine, Kyoto University , Kyoto , Japan.
authors.forename: Koichiro
authors.initials: K
authors.lastname: Ohmura
authors.affiliation: b Department of Diagnostic Pathology , Graduate School of Medicine, Kyoto University , Kyoto , Japan.
authors.forename: Shinji
authors.initials: S
authors.lastname: Sumiyoshi
authors.affiliation: a Department of Rheumatology and Clinical Immunology , Graduate School of Medicine, Kyoto University , Kyoto , Japan.
authors.forename: Tsuneyo
authors.initials: T
authors.lastname: Mimori
mesh_terms: D000328:Adult; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy*; D006562:Herpes Zoster / Q000209:etiology*; D014645:Herpesvirus 3, Human / Q000302:isolation & purification; D006801:Humans; D007155:Immunologic Factors / Q000009:adverse effects* / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D000069283:Rituximab / Q000009:adverse effects* / Q000627:therapeutic use
chemical_list: D007155:Immunologic Factors; D000069283:Rituximab; D011239:Prednisolone
keywords: Diabetes insipidus; Granulomatosis with polyangiitis; Rituximab; Varicella zoster virus