Study provides the first practical guide to diagnostic work-up and treatment in children with Cogan’s syndrome

Therapeutic decision on pediatric Cogan’s syndrome, a multisystem autoimmune disease affecting the ocular and audiovestibular system, is highly challenging due to the rarity of the disease. According to a recent study published in Pediatric Rheumatology, children with Cogan’s syndrome should receive complete examinations as soon as they are diagnosed, including tests for viral and autoimmune illnesses, echocardiography, and brain MRI. Based on the data collected, the researchers have come up with the first practical manual for a diagnostic work-up and treatment in children with Cogan’s syndrome. 

Dr. Rucklova and colleagues performed a literature analysis of case reports using PubMed. The instances from the literature review were supplemented by data from a child with Cogan’s syndrome, who was treated at the hospital by the researchers. The researchers identified 55 published case reports of children with Cogan’s syndrome and each patient featured in the case report had ‘sensorineural hearing deficit and ocular involvement’. Among these cases, the most frequently reported manifestation was keratitis (62% of reports), followed by uveitis (36% of reports). Vestibular symptoms, such as tinnitus and dizziness, were reported by 69% of patients, and the authors occasionally connected these symptoms to vomiting. Fevers, arthralgias or arthritis, neurological involvement, and skin involvement were recorded in 29%, 45%, 31%, and 18% of patients, respectively. The researchers found that systemic corticosteroids were the most frequently used therapy, administered to 81% of patients. Methotrexate was the second most popular treatment, utilized by 26% of patients, while only two individuals received infliximab, a biologic medication. 

Orsoni et al. (2004) indicated that the diagnosis of Cogan’s syndrome may be considered if persistent ocular inflammation is observed together with sensory neuronal hearing loss and any other systemic autoimmune inflammation symptoms. Immunosuppressive therapy was recommended to be started as soon as feasible to prevent permanent hearing loss and vision impairment. Experts reported more widespread lesions on the cornea than those seen in adults with Cogan’s syndrome. Systemic and ocular inflammation were successfully treated with immunosuppressive drugs, but the involvement of the pupillary area resulted in amblyopia in one case and lifelong poor vision in the other. 

The present study represents the most extensive analysis to date of children diagnosed with Cogan’s syndrome, offering a pioneering and comprehensive diagnostic work-up. This includes essential assessments such as laboratory screening for autoimmune and infectious markers, as well as echocardiography and MRI with gadolinium enhancement of the brain and inner ear in these young patients. These findings pave the way for a practical and reliable diagnostic approach, ultimately leading to more accurate and timely management of Cogan’s syndrome in pediatric cases. 

References: 

  1. Rucklova K, von Kalle T, Koitschev A, Gekeler K, Scheltdorf M, Heinkele A, et al. Paediatric Cogan´ s syndrome-review of literature, case report and practical approach to diagnosis and management. Pediatric Rheumatology. 2023 Dec;21(1):1-9.
  2. Orsoni JG, Zavota L, Vincenti V, Pellistri I, Rama P. Cogan syndrome in children: early diagnosis and treatment is critical to prognosis. American journal of ophthalmology. 2004 Apr 1;137(4):757-8. 

 

 

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