Mycophenolate mofetil as a treatment of steroid dependent Cogan's syndrome in childhood

Hautefort, Charlotte; Loundon, Natalie; Montchilova, Marta; Marlin, Sandrine; Garabédian, E. N.; Ulinski, Tim

doi:10.1016/j.ijporl.2009.06.025

Cited by 31 publications

(7 citation statements)

References 17 publications

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“…However, the extended follow-up of patients treated with cyclophosphamide for other benign conditions has been associated with a high incidence of serious drug-related toxicities prompting the search for other immunosuppressive options. The evidence supporting the use of ciclosporin A [57], MMF [37,58] and AZA [37,60] in AIED is based on case reports and small case series and not conclusive. We will discuss agents for which stronger evidence is available.…”

Section: Steroidsmentioning

confidence: 99%

Autoimmune sensorineural hearing loss: the otology-rheumatology interface

2013

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Autoimmune sensorineural hearing loss (SNHL) is a rare clinical entity characterized by a progressive fluctuating bilateral asymmetric SNHL that develops over several weeks to months. Vestibular symptoms, tinnitus and aural fullness are present in up to 50% of patients. Due to the lack of specific diagnostic tests, both clinical suspicion and responsiveness to corticosteroids are the pillars for the diagnosis of autoimmune SNHL. The evaluation of patients in whom this condition is suspected should include a detailed history and physical examination, an audiogram, an MRI and a limited laboratory workup to exclude secondary causes of hearing loss. The low frequency of this condition, the heterogeneity in the designs of the available studies and the absence of randomized trials comparing treatment responses and assessing long-term outcomes are some of the factors accounting for the limited evidence to guide the clinician in the approach to the diagnosis and treatment of autoimmune SNHL.

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Section: Steroidsmentioning

confidence: 99%

Autoimmune sensorineural hearing loss: the otology-rheumatology interface

2013

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“…Precise mechanisms of the concurrence of these two diseases remains still unclear, however autoimmune origin has been consolidated by the recent discoveries of Lunardi et al as a result of the dysregulation of the response of B and T lymphocytes, as is also demonstrated in ANCA-associated glomerulonephritis, in children with sensorineural hearing loss including Cogan syndrome [15]. In addition, immunosuppressive agents such as mycophenolate mofetil or cyclophosphamide are partly effective for quieting disease activity in both diseases, indicating involvement of cellular and humoral immune disorders in the pathogenesis of these two diseases [1,16]. Since chlamydia pneumoniae infection as the antecedent infection is demonstrated in some patients with two respective diseases [12,17], such pathogen might be involved in our patient, however precise pathogenesis remains uncertain.…”

Section: Discussionmentioning

confidence: 99%

Childhood Cogan syndrome with aortitis and anti-neutrophil cytoplasmic antibody-associated glomerulonephritis

et al. 2014

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Cogan syndrome is a systemic disease manifesting interstitial keratitis, sensorineural hearing loss, tinnitus, and rotatory vertigo. Renal complications of this syndrome are very rare. We encountered an adolescent with Cogan syndrome complicated by aortitis and anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. At the age of 14, the patient showed proteinuria in a screening urinalysis at school and was found to lack a right radial pulse. Magnetic resonance angiography disclosed right subclavian artery stenosis. Examination of a renal biopsy specimen showed ANCA-positive crescentic glomerulonephritis. Steroid and immunosuppressant treatment improved renal function and histopathology, but repeated recurrences followed. At 18, the patient developed rotatory vertigo, a sense of ear fullness, and sensorineural hearing loss. The patient was diagnosed with Cogan syndrome. We know of no previous description of ANCA-positive crescentic glomerulonephritis in children with Cogan syndrome. Accordingly, evaluation of aortitis in childhood should include not only otolaryngologic and ophthalmologic examinations, but also periodic urine examination and renal function tests.

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“…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 [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

Rituximab ameliorated severe hearing loss in Cogan's syndrome: a case report

Orsoni

Laganà

Rubino

et al. 2010

Orphanet J Rare Dis

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BackgroundRituximab 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).Case Presentationa 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.Discussionin 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.

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Mycophenolate mofetil as a treatment of steroid dependent Cogan's syndrome in childhood

Cited by 31 publications

References 17 publications

Autoimmune sensorineural hearing loss: the otology-rheumatology interface

Autoimmune sensorineural hearing loss: the otology-rheumatology interface

Childhood Cogan syndrome with aortitis and anti-neutrophil cytoplasmic antibody-associated glomerulonephritis

Rituximab ameliorated severe hearing loss in Cogan's syndrome: a case report

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