Cogan's syndrome as an extra-articular manifestation of rheumatoid arthritis

Dekker, Joost; Dinant, Huibert J.; Soesbergen, R. M. Van

doi:10.1007/bf02230360

Cited by 8 publications

(5 citation statements)

References 16 publications

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“…This peptide showed similarity with autoantigen SSA/Ro and with Reovirus III major core protein lambda and shared sequence homology with the cell density-enhanced protein tyrosine phosphatase-1 (DEP1/CD148), which is expressed on the sensory epithelia of the inner ear and on endothelial cells [ 69 ]. Traditional antibodies such as antinuclear antibodies and rheumatoid factor are not consistently found, but in 15–30% of cases, especially in the atypical form [ 70 ]. Recently, Jung et al observed a lymphocytic, neutrophilic, and histiocytic infiltrate in both basal turns of the cochlea, vestibular system, and surrounding tissue, with thrombosis and necrosis of the small vessel wall.…”

Section: Audiovestibular Pathology Associated With Systemic Autoimmentioning

confidence: 99%

Update on Vertigo in Autoimmune Disorders, from Diagnosis to Treatment

Girasoli

Cazzador

Padoan

et al. 2018

Journal of Immunology Research

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The prevalence of autoimmune diseases has been increasing over the last 20 years. The clinical presentation of this large and heterogeneous group of disorders depends on whether the involvement is organ-specific or non-organ-specific. Dizziness, vertigo, and disequilibrium are common symptoms reported by patients with vestibulocochlear involvement. The association of vertigo and autoimmune diseases has been largely documented, suggesting that autoimmune disorders could be overrepresented in patients with vertigo in comparison to the general population. The aim of this review is to present the recent literature findings in the field of autoimmune-mediated diseases with cochleovestibular involvement, focusing on the clinical presentation, diagnosis, and treatment of immune-mediated inner ear diseases including autoimmune inner ear disease (AIED), Meniere's disease, and bilateral vestibulopathy, as well as of systemic autoimmune diseases with audiovestibular disorders, namely, Behçet's disease, Cogan's syndrome, sarcoidosis, autoimmune thyroid disease, Vogt-Koyanagi-Harada syndrome, relapsing polychondritis, systemic lupus erythematosus, antiphospholipid syndrome, IgG4-related disease, and ANCA-associated vasculitides.

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Section: Audiovestibular Pathology Associated With Systemic Autoimmentioning

confidence: 99%

Update on Vertigo in Autoimmune Disorders, from Diagnosis to Treatment

Girasoli

Cazzador

Padoan

et al. 2018

Journal of Immunology Research

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“…18,19 Cogan's syndrome has been described as presenting in 2 forms: first, a typical or classical form consisting of interstitial keratitis and vestibulo-auditory symptoms and second, an atypical form with interstitial keratitis or other ocular inflammation and vestibulo-auditory symptoms in association with a systemic inflammatory disorder such as rheumatoid arthritis. 5,14 The case described here is more suggestive of the atypical form of Cogan's syndrome with a systemic inflammatory state induced by rheumatoid arthritis, which had been present for many months. The criteria for inclusion in the systemic inflammatory response syndrome (SIRS) are not satisfied by the clinical presentation of the case described herein.…”

Section: Discussionmentioning

confidence: 75%

“…Occasionally, Cogan's syndrome is associated with other inflammatory disorders, including aortitis causing aortic insufficiency 3 in 12% of patients and more rarely, a systemic vasculitis. 4,5 Although Cogan's syndrome is commonly considered to be caused by an immune mediated mechanism with vasculitis, evidence for vasculitis has not been described in either the eye or the ear to date. 6,7 The histopathology of the inner ear in Cogan's syndrome has been the subject of 6 case reports.…”

Section: Introductionmentioning

confidence: 99%

Histopathology of the Inner Ear in a Case With Recent Onset of Cogan’s Syndrome

Jung

Nadol

Folkerth

et al. 2015

Ann Otol Rhinol Laryngol

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The association of sensorineural hearing loss and vertigo with inflammatory eye disease, usually interstitial keratitis, has been called Cogan's syndrome. The pathogenesis of Cogan's syndrome is unknown, but it has been assumed to be an immune mediated disorder with vasculitis. The histopathology of the inner ear in Cogan's syndrome has been described in 6 case reports. Although common pathologic findings in these reports include degeneration of the auditory and vestibular neuroepithelium, endolymphatic hydrops, fibrosis, and new bone formation, direct pathologic evidence of a vasculitis has not been published. A possible reason for this failure to identify vasculitis was a substantial delay (range, 4-40 years) between the onset of symptoms and examination of the otopathology. In the current case report, the patient had both auditory and vestibular symptoms and interstitial keratitis with a time delay of only 2 to 4 weeks between symptoms and death. Evidence of a vasculitis as a possible underlying etiology included H&E histopathology and anti-CD45 immunostaining of vessels both in the auditory and vestibular systems, supporting the hypothesis of a vasculitis as a mechanism in this disorder.

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“…The most common cardiovascular manifestation, described in approximately 10% of the cases, is aortitis with aortic insufficiency [15][16][17] . It is difficult to differentiate aortitis in Cogan's syndrome from Takayasu's arteritis 18 . Large blood vessels are particularly affected.…”

Section: Discussionmentioning

confidence: 99%

Cogan’s syndrome: A case series

et al. 2018

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Introduction. Cogan's syndrome is a rare variable vessel vasculitis. It can be typical and atypical. Basis of the treatment comprises glucocorticoids, and in patients with systemic manifestations, immunosuppressive drugs. Case report. We wanted to present the experience of the Clinic for Rheumatology and Clinical Immunology of the Military Medical Academy, Belgrade, in diagnosing and treating patients suffering from Cogan's syndrome. The analysis included 7 patients. Patients' demographic characteristics, disease manifestations, course of the disease, applied treatment and treatment outcome were analysed. Five of the patients were women and 2 were men, with the average age of 39 ± 13 (25-65) years. The typical form of the disease manifested in 1 patient. In 6 patients, the first manifestation was the audiovestibular dysfunction. In 1 patient, systemic manifestations were the first to appear. In the cases where the disease manifested atypically, 3 patients developed conjunctivitis, 2 episcleritis, and 1 uveitis. They all had systemic manifestations. One female patient was diagnosed with aortitis and aortic insufficiency. They all tested positive for inflamma-tory biohumoral syndrome. Four patients had positive antinuclear antibodies, 3 anticytoplasmic antibodies, and 1 positive rheumatoid factor. They were all treated with glucocorticoid and immunosuppressive drugs. Methotrexate was administered to all the patients in doses up to 20 mg per week. Pulses of cyclophosphamide were administered to 2 female patients. All patients went successfully into remission. The female patient with the typical form of the disease experienced permanent bilateral hearing loss. Conclusion. Patients with a rapidly developed audiovestibular dysfunction should be viewed as suffering from Cogan's syndrome from the viewpoint of differential diagnosis. A timely treatment with glucocorticoids can prevent hearing loss and the development of systemic manifestations of the disease. Precedence should be given to methotrexate when selecting an immunosuppressive drug.

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Cogan's syndrome as an extra-articular manifestation of rheumatoid arthritis

Cited by 8 publications

References 16 publications

Update on Vertigo in Autoimmune Disorders, from Diagnosis to Treatment

Update on Vertigo in Autoimmune Disorders, from Diagnosis to Treatment

Histopathology of the Inner Ear in a Case With Recent Onset of Cogan’s Syndrome

Cogan’s syndrome: A case series

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