Rituximab ameliorated severe hearing loss in Cogan's syndrome: a case report
© Orsoni et al; licensee BioMed Central Ltd. 2010
Received: 11 February 2010
Accepted: 16 June 2010
Published: 16 June 2010
Rituximab is a monoclonal antibody inducing depletion of B lymphocytes and presently approved for the treatment of non-Hodgkin's lymphoma and rheumatoid arthritis. Here is the first report of the use of this drug in a case of Cogan's syndrome (CS).
a 25-year-old Italian woman was referred with conjunctival hyperaemia, interstitial keratitis, moderate bilateral sensorineural hearing loss accompanied by tinnitus, dizziness, nausea and vertigo, poorly responsive to oral and topical steroidal therapy. Diagnosis of typical CS was made. The administration of a combined immunosuppressive treatment resolved ocular inflammation, dizziness, nausea, and vertigo but gave little results in controlling progressive hearing loss. A noticeable improvement in hearing function was documented by pure tone audiometry after infusion of Rituximab.
in CS, hearing function is often the most difficult parameter to control with therapy. A positive effect of Rituximab on was observed in our case. The drug also allowed to significantly reduce the number of adjuvant immunosuppressive medications.
Rituximab is a chimeric human-mouse monoclonal antibody against lymphocyte CD20 surface antigen, and treatment induces depletion of B lymphocytes by various mechanisms. These antibodies are thought to act in vivo mostly through activation of antibody-dependent cell-mediated cytotoxicity and complement-dependent cytotoxicity, although direct growth inhibition and/or induction of apoptosis may also take place . Rituximab is presently approved for the treatment of non-Hodgkin's lymphoma and rheumatoid arthritis. Here is the first report of the use of this drug in a case of severe Cogan's syndrome (CS).
Cogan's Syndrome is a rare autoimmune vasculitis characterized by bilateral interstitial keratitis associated with vertigo, tinnitus and typically profound sensorineural deafness. This clinical presentation is called "typical" CS. "Atypical" CS is the name given to those forms with similar vestibuloauditory symptoms but ocular involvement different from interstitial keratitis (i.e. scleritis, retinal vasculitis, papillitis, etc), and/or other severe systemic vasculitic manifestation[4, 5]. Considering both forms, CS is a rare clinical entity which usually occurs in early adulthood but with possible onset in children or in elderly patients [6, 7]. Anti hsp-70 antibody has been indicated as a marker of the autoimmune origin of hearing loss .
While different immunomodulatory drugs, including mycophenolate and anti-TNF molecules, have been proposed in CS with satisfactory results, refractory cases are still reported, most concerning hearing function [7–10]. The use of Rituximab in systemic vasculitis and autoimmune diseases with antibody-mediated aetiology has a strong rationale and is increasingly reported in the literature [11, 12]. In CS, hearing function seems to be the most difficult parameter to control with therapy. The effect of Rituximab on B cells may therefore be of interest to avoid deafness and the demand of cochlear implant in severe cases. It can also allow to significantly reduce the number of medications necessary to control the multiple manifestations of the syndrome. We recommend the four-week division of the overall cycle dose of the drug as it appears to be particularly safe, even though we do not recommend the use of this drug as a first line therapy.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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