Cogan's syndrome: State of the art of systemic immunosuppressive treatment in adult and pediatric patients

Abstract Cogan’s syndrome (CS) is a rare autoimmune vasculitis of unknown aetiology characterised by non-syphilitic interstitial keratitis, audio-vestibular symptoms, sometimes systemic symptoms and multi-organ involvement. Atypical CS has other ocular features such as scleritis, episcelritis, retinitis and optic neuritis. Diagnosis of CS is purely clinical without a confirmatory test. Hereby, we report a case of atypical CS presenting with features of encephalitis who was treated successfully with intravenous pulse methylprednisolone with cyclophosphamide. It is important to consider Cogan’s syndrome in the differential diagnosis of encephalitis with ocular and vestibular symptoms in young patients, as high morbidity and mortality rates are effectively lowered by early immunosuppressive treatment.

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Cogan's syndrome – A rare aortitis, difficult to diagnose but with therapeutic potential

Revista da Associação Médica Brasileira ◽

10.1590/1806-9282.63.12.1028 ◽

2017 ◽

Vol 63 (12) ◽

pp. 1028-1031 ◽

Cited By ~ 3

Author(s):

Raiza Colodetti ◽

Guilherme Spina ◽

Tatiana Leal ◽

Mucio Oliveira Jr ◽

Alexandre Soeiro

Keyword(s):

Disease Control ◽

Rare Case ◽

Aortic Wall ◽

Therapeutic Potential ◽

Immunosuppressive Treatment ◽

Rare Condition ◽

Cogan’S Syndrome ◽

Ocular Manifestations ◽

Cogan's Syndrome

Summary The inflammation of aortic wall, named aortitis, is a rare condition that can be caused by a number of pathologies, mainly inflammatory or infectious in nature. In this context, the occurrence of combined audiovestibular and/or ocular manifestations eventually led to the diagnosis of Cogan's syndrome, making it the rare case, but susceptible to adequate immunosuppressive treatment and satisfactory disease control.

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Case Report: Effective and Safe Treatment With Certolizumab Pegol in Pregnant Patients With Cogan’s Syndrome: A Report of Three Pregnancies in Two Patients

Frontiers in Immunology ◽

10.3389/fimmu.2020.616992 ◽

2021 ◽

Vol 11 ◽

Author(s):

Nils Venhoff ◽

Jens Thiel ◽

Markus A. Schramm ◽

Ilona Jandova ◽

Reinhard E. Voll ◽

...

Keyword(s):

Inflammatory Diseases ◽

Treatment Options ◽

Certolizumab Pegol ◽

Immunosuppressive Agents ◽

Immunosuppressive Treatment ◽

Cogan’S Syndrome ◽

Safe Treatment ◽

Tnf Α ◽

Cogan's Syndrome ◽

Systemic Glucocorticoids

Cogan’s syndrome is a rare autoimmune disease characterized by ocular inflammation and audiovestibular manifestations. Treatment consists of systemic glucocorticoids and other immunosuppressive agents including methotrexate, cyclophosphamide and TNF-α-inhibitors. Due to potential ovarian or fetal toxicity immunosuppressive treatment options are limited during pregnancies. Thus far there is a paucity of reports on pregnancies in Cogan’s syndrome. With minimal transplacental transfer, Certolizumab pegol is considered to be safe for the use in pregnant patients with underlying inflammatory diseases. However, there is no literature on the use of this TNF-α-inhibitor in Cogan’s syndrome in general and especially during gestation. Here we report three pregnancies in two Cogan’s Syndrome-patients treated with Certolizumab pegol. Treatment with Certolizumab pegol was effective and well tolerated in patients with Cogan’s syndrome and seems to be a safe treatment option during pregnancy.

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