Table 2 Clinical characteristics, treatment and treatment outcomes of paediatric CS patients. Patients from the literature and our patient. N = 55
Number of patients | 55 |
|---|---|
Age, years, median (min–max) | 12 (3–18) |
Gender, male, n (%) | Male 31/54 (57) |
Follow-up period, years (min–max) | 2 (0.2–16) |
Clinical findings, n (%) | |
Ocular findings | 55/55 (100) |
Interstitial keratitis | 34/55 (62) |
Uveitis | 20/55 (36) |
Conjunctivitis | 10/55 (18) |
Sensorineural hearing deficit | 55/55 (100) |
Vestibular symptoms | 37/54 (69) |
Any systemic symptoms | 32/55 (58) |
Fever | 16/55 (29) |
Musculoskeletal symptoms | 25/55 (45) |
Neurological signs | 17/55 (31) |
Aortitis | 9/55 (16) |
Skin involvement | 10/55(18) |
Gastrointestinal symptoms | 6/55 (11) |
Elevated CRP and/or ESR | 29/36 (81) |
Treatment, n (%) | |
Systemic corticosteroids | 44/54 (81) |
MTX | 14/54 (26) |
AZA | 5/54 (9) |
CYC | 5/54 (9) |
MMF | 3/54 (6) |
Cyclosporine | 3/54 (6) |
Cochlear implantation | 7/55 (13) |
Outcome, hearing, n (%) | |
Deafness/ unilateral deafness/ severely impaired | 28/50 (56) |
Moderately impaired | 6/50 (12) |
Complete/partial remission/mildly impaired | 16/50 (32) |
Outcome, ocular symptoms, n (%) | |
Blindness/ unilateral blindness | 2/48 (4) |
Decreased visual acuity | 5/48 (10) |
Recurrences | 6/48 (13) |
Remission | 33/48 (69) |
Mortality | 2/55 (4) |