title: Long-lasting uveitis remission and hearing loss recovery after rituximab in Vogt-Koyanagi-Harada disease.
abstract: Vogt-Koyanagi-Harada disease (VKHD) is a multisystemic disorder characterized by granulomatous panuveitis variably combined with T cell-mediated neurologic and cutaneous manifestations. Early and aggressive treatment with systemic corticosteroids is the mainstay of treatment for VKHD. Additional use of immunosuppressants, intravenous immunoglobulins, and tumor necrosis factor-alpha inhibitors can help the most severely affected patients and work as corticosteroid-sparing agents. We report the case of a young woman with relapsing and multiresistant VKHD who demonstrated a stable remission of both uveitis and high-frequency hearing loss following rituximab intravenous administration (1 g. twice, 2 weeks apart, and 6 months later). A complete clinical response was observed 1 month since the first infusion, and no ocular relapses were recorded during the following year; in addition, audiometry showed a high-frequency hearing recovery in the right ear. Further observational studies are required to define the role of CD20 inhibition in the management of VKHD.
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behçet's disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.|Rheumatology Unit, Department of Medicine DIMED, University of Padova, Padova, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Caso
authors.affiliation: Istitute of Pediatrics, Policlinico "A. Gemelli", Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Donato
authors.initials: D
authors.lastname: Rigante
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behçet's disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Vitale
authors.affiliation: Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, Naples, Italy.
authors.forename: Luisa
authors.initials: L
authors.lastname: Costa
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behçet's disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
authors.forename: Vittoria
authors.initials: V
authors.lastname: Bascherini
authors.affiliation: Ophthalmology and Neurosurgery Department, University of Siena, Siena, Italy.
authors.forename: Eugenia
authors.initials: E
authors.lastname: Latronico
authors.affiliation: Ophthalmology and Neurosurgery Department, University of Siena, Siena, Italy.
authors.forename: Rossella
authors.initials: R
authors.lastname: Franceschini
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behçet's disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy. cantariniluca@hotmail.com.|Rheumatology Unit, Policlinico "Le Scotte", University of Siena, Viale Bracci 1, 53100, Siena, Italy. cantariniluca@hotmail.com.
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000737:chemistry / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D018951:Antigens, CD20 / Q000378:metabolism; D001299:Audiometry; D016572:Cyclosporine / Q000009:adverse effects; D003907:Dexamethasone / Q000008:administration & dosage; D005260:Female; D034381:Hearing Loss / Q000150:complications / Q000628:therapy*; D006319:Hearing Loss, Sensorineural / Q000473:pathology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000737:chemistry; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008775:Methylprednisolone / Q000008:administration & dosage; D015864:Panuveitis / Q000473:pathology; D011239:Prednisolone / Q000008:administration & dosage; D011241:Prednisone / Q000008:administration & dosage; D011379:Prognosis; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab / Q000627:therapeutic use*; D014605:Uveitis / Q000150:complications / Q000473:pathology / Q000628:therapy*; D014607:Uveomeningoencephalitic Syndrome / Q000150:complications / Q000188:drug therapy*
chemical_list: D000305:Adrenal Cortex Hormones; D000894:Anti-Inflammatory Agents, Non-Steroidal; D018951:Antigens, CD20; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003907:Dexamethasone; D016572:Cyclosporine; D011239:Prednisolone; D011241:Prednisone; D008775:Methylprednisolone
keywords: Corticosteroid; Rituximab; Sensorineural deafness; Uveitis; Vogt-Koyanagi-Harada disease