Clinical features and characteristics of uveitis associated with juvenile idiopathic arthritis in Japan: first report of the pediatric rheumatology association of Japan (PRAJ)

Abstract

Background Although there are many reports on Juvenile Idiopathic arthritis-associated uveitis (JIA-U) from various countries, especially from Europe and North America, there are few reports from Asia. Our aim was to investigate the epidemiology, characteristics and predictors of JIA-U in Japan. Methods Data were retrospectively collected on 726 patients with JIA from medical records as of April 2016 at 15 medical centers specialized in pediatric rheumatic diseases. Of these, patients with uveitis were further investigated for the specific characteristics of this manifestation. Results The prevalence of uveitis was 6.1% in the 726 JIA patients examined. Incidence of uveitis was significantly higher in patients with an earlier arthritis onset (2.6-vs.-5.8 years, P < 0.0001), oligoarthritis (16.1%-vs.-1.6%, P < 0.001), or anti-nuclear antibodies. On the contrary, it was significantly less common in patients with rheumatoid factor or anti-cyclic citrullinated peptide antibodies. A history of using methotrexate (MTX), infliximab or adalimumab was also associated with uveitis occurrence. The median age at uveitis diagnosis was 5 years, and the median time from arthritis onset to uveitis diagnosis was 2 years. The occurrence of anterior and bilateral uveitis was 79.3 and 53.7%, respectively. There were no symptoms at uveitis diagnosis in 58.5% of cases. Complications arising between the time of uveitis diagnosis and the last observation increased from 31.7 to 56.1%; in particular, cataract was increased 3-fold. While no patients lost their vision, 61.9% did not recover normal vision (≥ 1.0), and in many cases active uveitis persisted, especially in males. In addition to steroid eye drops (97.6%) and MTX (15.4%), biological agents were used for treating the uveitis in 41.5% of patients. Conclusions The epidemiology, characteristics and predictors of JIA-U in Japan are described here for the first time. Although the prevalence of JIA-U in Japan is lower than in predominantly Caucasian cohorts, as reported from North America and Europe, the epidemiology, characteristics and predictors were found to be similar.

Importance of pediatric rheumatologists and transitional care for juvenile idiopathic arthritis-associated uveitis: A retrospective series of 9 cases.

“…We conducted a retrospective analysis of the case records of 9 patients with JIA-U who received care (HLA-B27) positivity, and rheumatoid factor (RF) negativity [2,[15][16][17]. These factors were reflected in 4 our 9 JIA-U patients as follows: median age was 16.8 years (range 5.5-19.8 years), median disease duration was 12.5 years (range 3.5-24.7 years), the sex ratio was 1:8 (male:female), all patients presented with oligo-JIA (one presented with extended oligo-JIA), median age at uveitis onset was 5.0 years (range 3.0-13.0 years), the onset of uveitis preceded arthritis in 2 patients, 4 patients showed ANA positivity ( ≧ 1:160) (all patients presented with the homogeneous and speckled-pattern subtype), and all patients were negative for RF.…”

“…Although the prevalence rate of JIA is found to be 32.6 per 100,000 /person [3], JIA-U is rarer, and it occurs in 4.7-20.5% of JIA patients, with local differences noted (6.1% reported in Japan) [2,[4][5][6][7][8][9].…”

Importance of pediatric rheumatologists and transitional care for juvenile idiopathic arthritis-associated uveitis: a retrospective series of 9 cases

“…Figure 1 presents the 9 cases of JIA-U in order of age at onset, including 2 cases (Cases 4 and 9) in which uveitis preceded arthritis. Risk factors associated with the development of JIA-U included sex, JIA category, age at onset, the titer of anti-nuclear antibody (ANA), human leukocyte antigen B27 (HLA-B27) positivity, and rheumatoid factor (RF) negativity [2,[15][16][17]. These factors were reflected in our 9 JIA-U patients as follows: median age was 16.8 years (range 5.5-19.8 years), median disease duration was 12.5 years (range 3.5-24.7 years), the sex ratio was 1:8 (male:female), all patients presented with oligo-JIA (one presented with extended oligo-JIA), median age at uveitis onset was 5.0 years (range 3.0-13.0 years), the onset of uveitis preceded arthritis in 2 patients, 4 patients showed ANA positivity (≧ 1:160) (all patients presented with the homogeneous and speckledpattern subtype), and all patients were negative for RF.…”

“…However, pediatric rheumatologists rarely encounter cases of blindness due to JIA-U. Among the JIA-U cases reported in Japan, legal blindness was not noted among patients under 18 years of age [2].…”

“…Although the prevalence rate of JIA is found to be 32.6 per 100,000 /person [3], JIA-U is rarer, and it occurs in 4.7-20.5% of JIA patients, with local differences noted (6.1% reported in Japan) [2,[4][5][6][7][8][9]. Improved antiinflammation effects with biologics has resulted in a good prognosis for JIA-U [10,11].…”