Clinical Features and Outcome of Cogan Syndrome

Pagnini, Ilaria; Zannin, Maria Elisabetta; Vittadello, Fabio; Sari, Marianna; Simonini, Gabriele; Cimaz, Rolando; Zulian, Francesco

doi:10.1016/j.jpeds.2011.07.051

Cited by 41 publications

(11 citation statements)

References 29 publications

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“…Evidence of vasculitis was present in two skin biopsies. Among paediatric CS, Pagnini et al 15 noted 3 of 23 patients with cutaneous manifestations.…”

Section: Discussionmentioning

confidence: 99%

Cogan’s syndrome with pyoderma gangrenosum: management of two uncommon disorders with aggressive presentation in a patient

Chua¹,

Mallett

Dahiya

2018

BMJ Case Reports

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Pyoderma gangrenosum (PG) coexisting with Cogan's syndrome (CS) is uncommon, although cutaneous manifestations are known to develop in CS. A middle-aged white female patient had chronic relapsing PG requiring ciclosporin and prednisolone. Despite receiving optimal doses of ciclosporin and prednisolone, she developed acute vestibulo-auditory symptoms as a result of CS. Ciclosporin was switched to methotrexate and prednisolone was increased. However, she continued to develop acute scleritis, requiring methylprednisolone pulses, and still had further flares of PG. Her methotrexate was held off when she developed severe pneumonia and she then received a trial of intravenous immunoglobulins (IVIG) for her recurrent leg ulcers. Unfortunately, she failed to respond to IVIG. Her ulcers eventually responded to six doses of monthly intravenous cyclophosphamide induction. Although CS is not an antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, we used pulse cyclophosphamide, based on the experience of cyclophosphamide efficacy in severe ANCA-associated vasculitis (AAV). Following induction, both diseases currently remain under control with azathioprine as maintenance treatment.

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“…Evidence of vasculitis was present in two skin biopsies. Among paediatric CS, Pagnini et al 15 noted 3 of 23 patients with cutaneous manifestations.…”

Section: Discussionmentioning

confidence: 99%

Cogan’s syndrome with pyoderma gangrenosum: management of two uncommon disorders with aggressive presentation in a patient

Chua¹,

Mallett

Dahiya

2018

BMJ Case Reports

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“…Our population included 11 children, nine of whom (81.8%) were found positive for at least two of four anti-inner ear antibodies. Adult populations diagnosed with autoimmune sensorineural hearing loss have been well described, but current pediatric data is limited to few case reports and small case series [11][12][13][14][15][16][17][18][19][20].…”

Section: Discussionmentioning

confidence: 99%

Identification of autoantibodies against inner ear antigens in a cohort of children with idiopathic sensorineural hearing loss

et al. 2013

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Immune-mediated pathogenesis has been suggested for idiopathic sensorineural hearing loss. Recent studies have investigated the relationship between idiopathic sensorineural hearing loss and autoantibodies against inner ear antigens. We conducted a prospective, observational study in a series of pediatric patients affected by idiopathic sensorineural hearing loss. Autoantibodies against inner ear (anti-Cogan peptide, anti-connexin 26, anti-DEP1/CD148 and anti-reovirus), previously described in the serum of patients with Cogan's syndrome, were detected in our population. The characteristics of children whose results were positive were also evaluated to verify if clinical data, disease progression and response to treatment could confirm an immune-mediated pathogenesis. Eleven patients were enrolled and 9 of them were positive for inner ear antibodies. Non-organ specific autoantibodies were present in 5 children out of 9. An immune-mediated condition was diagnosed in 2 cases and minor immune manifestations were found in 2 additional patients. In 5 cases hearing loss remained stable without therapy, while 4 children developed progression. Two subjects were treated with corticosteroids and methotrexate, achieving hearing improvement. Another subject showed stabilization on methotrexate. Inner ear autoantibodies can be positive in children with autoimmune sensorineural hearing loss, and in conjunction with clinical data may assist the clinician in identifying a subset amenable for immune modulation therapy. Large prospective studies are needed to investigate usefulness, diagnostic and prognostic role of these autoantibodies.

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“…Поражение периферических сосудов различного калибра и локализации встречается примерно у каждого десятого пациента [27][28][29] и может развиваться как в дебюте СК, так и через несколько лет после начала за-болевания. Сердечно-сосудистые осложнения, обуслов-ленные аортитом, вовлечением коронарных артерий, недостаточностью аортального клапана с последующим прогрессированием левожелудочковой недостаточно-сти, являются основной причиной смерти 10% больных СК [13].…”

Section: Cogan's Syndrome Is a New Nosological Entity In The Current unclassified

Cogan's syndrome is a new nosological entity in the current classification of systemic vasculitides: A clinical case and a review of literature

Бекетова¹

2016

rsp

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Clinical Features and Outcome of Cogan Syndrome

Cited by 41 publications

References 29 publications

Cogan’s syndrome with pyoderma gangrenosum: management of two uncommon disorders with aggressive presentation in a patient

Cogan’s syndrome with pyoderma gangrenosum: management of two uncommon disorders with aggressive presentation in a patient

Identification of autoantibodies against inner ear antigens in a cohort of children with idiopathic sensorineural hearing loss

Cogan's syndrome is a new nosological entity in the current classification of systemic vasculitides: A clinical case and a review of literature

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