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Table 4 Clinical outcome with respect to systemic treatment modality. Patients from the literature supplemented by our patient. N = 55

From: Paediatric Cogan´s syndrome - review of literature, case report and practical approach to diagnosis and management

Treatment modality

Outcome

Auditory

Ocular

Other

No of patients (%)

 No systemic treatment N = 11/55 (20)

Deaf: 7/11 (64)

Moderately impaired: 3/11 (27)

Remission: 0/11 (0)

Recurrences 2/11 (18)

Remission 6/11 (55)

Death 1/11 (9)

Arthritis 1/11 (9)

 Systemic CS N = 14/55 (25)

Deaf/severely impaired: 6/14 (43)

Complete/partial remission: 8/14 (57)

Impaired vision 2/14 (14)

Remission 11/14 (79)

Arthritis 1/14 (7)

 Systemic CS and cDMARDs N = 26/55 (47)

Deaf/severely impaired 11/26 (42)

Moderately impaired: 7/26 (27)

Complete/partial remission: 7/26 (27)

Impaired vision: 3/26 (12)

Recurrences: 4/26 (15)

Secondary complications: 2/26 (8)

Remission: 14/26 (54)

Death 1/26 (4)

Vestibular 2/26 (8)

Vascular 1/26 (4)

Renal 2/26 (8)

 Systemic CS, cDMARD and biologics N = 2/55 (4)

Severely impaired 1/2 (50)

Complete/partial remission: ½ (50)

Decreased vision: 1/2 (50)

Remission: ½ (50)

None

 Systemic CS and colchicine N = 2/55 (4)

Deaf/severely impaired: 2/2 (100)

Decreased vision: ½ (50)

Remission: ½ (50)

Vestibular: ½ (50)

  1. Conventional DMARDs included: MTX, AZA, CsA, MMF, CYC. Secondary ocular complications included a glaucoma and a cataract. All patients received topical steroids