To characterize the epidemiology and clinical course of children with juvenile idiopathic arthritis-associated uveitis (JIA-U) in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) ...Registry and explore differences between African American (AA) and non-Hispanic white (NHW) children.
There were 4983 children with JIA enrolled in the CARRA Registry. Of those, 3967 NHW and AA children were included in this study. Demographic and disease-related data were collected from diagnosis to enrollment. Children with JIA were compared to those with JIA-U. Children with JIA-U were also compared by race.
There were 459/3967 children (11.6%) with JIA-U in our cohort with a mean age (SD) of 11.4 years (± 4.5) at enrollment. Compared to children with JIA, they were younger at arthritis onset, more likely to be female, had < 5 joints involved, had oligoarticular JIA, and were antinuclear antibody (ANA)-positive, rheumatoid factor (RF)-negative, and anticitrullinated protein antibody-negative. Predictors of uveitis development included female sex, early age of arthritis onset, and oligoarticular JIA. Polyarticular RF-positive JIA subtype was protective. Nearly 3% of children with JIA-U were AA. However, of the 220 AA children with JIA, 6% had uveitis; in contrast, 12% of the 3721 NHW children with JIA had uveitis.
In the CARRA registry, the prevalence of JIA-U in AA and NHW children is 11.6%. We confirmed known uveitis risk markers (ANA positivity, younger age at arthritis onset, and oligoarticular JIA). We describe a decreased likelihood of uveitis in AA children and recommend further exploration of race as a risk factor in a larger population of AA children.
Objective
To formulate consensus treatment plans (CTPs) for induction therapy of newly diagnosed proliferative lupus nephritis (LN) in juvenile systemic lupus erythematosus (SLE).
Methods
A ...structured consensus formation process was employed by the members of the Childhood Arthritis and Rheumatology Research Alliance after considering the existing medical evidence and current treatment approaches.
Results
After an initial Delphi survey (response rate = 70%), a 2‐day consensus conference, and 2 followup Delphi surveys (response rates = 63–79%), consensus was achieved for a limited set of CTPs addressing the induction therapy of proliferative LN. These CTPs were developed for prototypical patients defined by eligibility characteristics, and included immunosuppressive therapy with either mycophenolic acid orally twice per day, or intravenous cyclophosphamide once per month at standardized dosages for 6 months. Additionally, the CTPs describe 3 options for standardized use of glucocorticoids, including a primarily oral, a mixed oral/intravenous, and a primarily intravenous regimen. There was consensus on measures of effectiveness and safety of the CTPs. The CTPs were well accepted by the pediatric rheumatology providers treating children with LN, and up to 300 children per year in North America are expected to be candidates for the treatment with the CTPs.
Conclusion
CTPs for induction therapy of proliferative LN in juvenile SLE based on the available scientific evidence and pediatric rheumatology group experience have been developed. Consistent use of the CTPs may improve the prognosis of proliferative LN, and support the conduct of comparative effectiveness studies aimed at optimizing therapeutic strategies for proliferative LN in juvenile SLE.
Juvenile idiopathic arthritis-associated uveitis (JIA-U) can lead to poor visual outcomes and impact a child's quality of life (QOL) and function. Our aim is to identify risk markers of JIA-U and ...examine differences in the QOL of children with JIA and JIA-U.
Rheumatology and ophthalmology record reviews and questionnaires were completed every 4-6 months on 287 children with JIA. We collected arthritis, uveitis, and QOL data. We examined data through last study visit.
There were 52/287 (18%) children with JIA-U who were younger at arthritis diagnosis, had oligoarticular persistent JIA, and ANA positive. Confirmed uveitis predictors were age at JIA diagnosis (OR = 0.86) and oligoarticular subtype (OR = 5.92). They had worse vision specific QOL and function, but similar general QOL. Blindness occurred in 17.5% of children but was more common in African American children compared to non-Hispanic Caucasian children ((5/7 (71%) vs. 2/29 (7%), p <0.001) despite a similar uveitis prevalence (22% vs. 16%). Both races had similar complications, although band keratopathy was more frequent in African Americans (75% vs. 15.6%, p = 0.003).
We confirm young age at JIA diagnosis and the oligoarticular JIA subtype as predictors of uveitis development. Although we were unable to identify predictors of ocular complications or blindness, AA children appeared to have a more severe disease course manifested by increased ocular complications, vision loss and blindness. Potential causes that warrant additional study include underlying disease severity, access to medical care and referral bias. Further investigation of the risk factors for vision-compromising uveitis and its' long-term effects should be conducted in a large racially diverse population.
Objective
The Effects of Youngsters’ Eyesight on Quality of Life (EYE‐Q) is a novel measure of vision‐related quality of life (QOL) and function in children. We aim to determine the validity of the ...EYE‐Q in childhood uveitis.
Methods
We ed medical record data on arthritis and uveitis in a convenience sample of children with juvenile idiopathic arthritis (JIA) and/or uveitis. In addition to the EYE‐Q, parents and patients completed questionnaires on overall QOL (Pediatric Quality of Life Inventory PedsQL), and physical functioning (Childhood Health Assessment Questionnaire C‐HAQ).
Results
Among 57 children (8 JIA, 24 JIA and uveitis, 25 uveitis alone), 102 ocular examinations were performed within 1 month of completing questionnaires. Uveitis patients had bilateral disease (69%), anterior involvement (78%), synechiae (51%), and cataracts (49%). Children with vision loss in their better eye (visual acuity VA 20/50 or worse) had worse EYE‐Q (P = 0.006) and PedsQL (P = 0.028) scores, but not C‐HAQ scores. The EYE‐Q moderately correlated with logMAR VA (rs = −0.43), PedsQL (rs = 0.43), and C‐HAQ (rs = −0.45), but was not correlated with anterior chamber cells or intraocular pressure. The PedsQL and C‐HAQ did not correlate with VA or cells. There were strong correlations between the parent and child EYE‐Q (rs = 0.62). Cronbach's α for the child report was 0.91. The EYE‐Q had strong test–retest reliability (rs = 0.75).
Conclusion
The EYE‐Q may be an important tool in the assessment of visual outcomes in childhood uveitis and an improvement over general measures in detecting changes in vision‐related function.
The Association of Race With Childhood Uveitis Angeles-Han, Sheila T; McCracken, Courtney; Yeh, Steven ...
American journal of ophthalmology,
11/2015, Letnik:
160, Številka:
5
Journal Article
Recenzirano
Odprti dostop
Purpose To identify risk factors for a severe uveitis course among children with noninfectious uveitis. Design Retrospective cohort study. Method This was a retrospective analysis of a prospectively ...collected database. Records of 94 children with uveitis were reviewed at enrollment and every 3–6 months (2011–2015). Severe uveitis was defined as a history of ocular complications or a visual acuity (VA) of ≤20/200. Children were compared by disease, VA, complications, and race. Regression models were used to model risk factors for severe disease. When examining race, we focused on non-Hispanic African-American and non-Hispanic white children only. Results Of 85 children with uveitis and complete ocular examinations, 27 (32%) had a history of a VA of ≤20/200. A subanalysis of non-Hispanic African-American and white children showed an increased prevalence of VA ≤20/200 in non-Hispanic African-Americans (18/25; 72% vs 4/43; 9%). Non-Hispanic African-Americans were more likely to be diagnosed at an older age ( P = .030) and to have intermediate uveitis ( P = .026), bilateral disease ( P = .032), a history of VA ≤20/50 ( P = .002), VA ≤20/200 ( P < .001), and a higher rate of complications ( P < .001). On multivariable analysis, non-Hispanic African-American race was a significant predictor of blindness (OR = 31.6, 95% CI 5.9–168.5, P < .001), after controlling for uveitis duration. Non-Hispanic African-Americans also developed 2.2 times more unique complications per year of disease than non-Hispanic whites when controlling for uveitis type and duration. Conclusions There appear to be racial differences in the outcomes of children with uveitis. Non-Hispanic African-American children with non–juvenile idiopathic arthritis–associated uveitis may have worse visual outcomes with increased vision loss and ocular complications. These findings highlight the need for future studies in minority populations.
Malakoplakia and Primary Immunodeficiency Archer, Sydney R., BS; Abramowsky, Carlos R., MD; Kobrynski, Lisa, MD ...
The Journal of pediatrics,
11/2014, Letnik:
165, Številka:
5
Journal Article
Recenzirano
Malakoplakia, a rare granulomatous disease caused by impaired macrophage response, has been reported only rarely in children. We report 3 unique cases, with lesions occurring in unusual locations in ...children with primary immune deficiencies.
Juvenile idiopathic arthritis (JIA) affects children of all races. Prior studies suggest that phenotypic features of JIA in African American (AA) children differ from those of non-Hispanic white ...(NHW) children. We evaluated the phenotypic differences at presentation between AA and NHW children enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry, and replicated the findings in a JIA cohort from a large center in the southeastern United States.
Children with JIA enrolled in the multicenter CARRA Registry and from Emory University formed the study and replication cohorts. Phenotypic data on non-Hispanic AA children were compared with NHW children with JIA using the chi-square test, Fisher's exact test, and the Wilcoxon signed-rank test.
In all, 4177 NHW and 292 AA JIA cases from the CARRA Registry and 212 NHW and 71 AA cases from Emory were analyzed. AA subjects more often had rheumatoid factor (RF)-positive polyarthritis in both the CARRA (13.4% vs 4.7%, p = 5.3 × 10(-7)) and the Emory (26.8% vs 6.1%, p = 1.1 × 10(-5)) cohorts. AA children had positive tests for RF and cyclic citrullinated peptide antibodies (CCP) more frequently, but oligoarticular or early onset antinuclear antibody (ANA)-positive JIA less frequently in both cohorts. AA children were older at onset in both cohorts and this difference persisted after excluding RF-positive polyarthritis in the CARRA Registry (median age 8.5 vs 5.0 yrs, p = 1.4 × 10(-8)).
Compared with NHW children, AA children with JIA are more likely to have RF/CCP-positive polyarthritis, are older at disease onset, and less likely to have oligoarticular or ANA-positive, early-onset JIA, suggesting that the JIA phenotype is different in AA children.
To examine in a randomize controlled feasibility clinical trial the efficacy of a cognitive-behavioral intervention designed to manage pain, enhance disease adjustment and adaptation and improve ...quality of life among female adolescents with systemic lupus erythematosus.
Female adolescents (n = 53) ranging in age from 12 to 18 years were randomly assigned to 1 of 3 groups including a cognitive-behavioral intervention, an education-only arm and a no-contact control group. Participants were assessed at baseline, postintervention and at 3- and 6-month intervals after completion of the intervention.
No significant differences were revealed among the 3 treatment arms for any of the dependent measures at any of the assessment points. For the mediator variables, a posthoc secondary analysis did reveal increases in coping skills from baseline to postintervention among the participants in the cognitive-behavioral intervention group compared with both the no-contact control group and the education-only group.
Although no differences were detected in the primary outcome, a possible effect on coping of female adolescents with systemic lupus erythematosus was detected in this feasibility study. Whether the impact of training in the area of coping was of sufficient magnitude to generalize to other areas of functioning, such as adjustment and adaptation, is unclear. Future phase III randomized trials will be needed to assess additional coping models and to evaluate the dose of training and its influence on pain management, adjustment and health-related quality of life.
Rheumatoid factor-positive polyarthritis (RF+ poly) is the juvenile idiopathic arthritis (JIA) category that resembles adult seropositive rheumatoid arthritis (RA). We studied children with RF+ ...and/or anticyclic citrullinated peptide antibody (anti-CCP)+ JIA to determine what proportion of those children meet International League of Associations for Rheumatology (ILAR) criteria for RF+ poly JIA and to assess for significant differences between children who meet RF+ poly criteria and those who are classified in other categories.
Charts of children with JIA who were RF+ and/or anti-CCP+ were reviewed. Children with RF+ poly JIA were compared to children in other categories. Statistical analysis was performed using chi-square, Fisher's exact test, and the Student's t-test.
Of 56 children with RF+ and/or anti-CCP+ JIA, 34 (61%) met ILAR criteria for RF+ poly JIA. Twelve children had RF-/anti-CCP+ JIA with low anti-CCP titers. When these 12 children were excluded, there were few significant differences between children who met criteria for RF+ poly and those who were classified in other categories. The American College of Rheumatology/European League Against Rheumatism criteria for RA identified more RF+ children than did the ILAR RF+ poly classification (100% vs 77%).
A number of children with RF+ arthritis were excluded from the RF+ poly JIA classification, though many demographic features and disease measures were similar to those of children who met criteria for RF+ poly JIA. We propose prioritization of RF/anti-CCP positivity over specific exclusions, along with inclusion of anti-CCP, in future revisions of the JIA classification criteria, to improve the sensitivity of diagnosing childhood-onset RA.
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