Anakinra for Myocarditis in Juvenile Idiopathic Arthritis

A 20-year-old pregnant woman with a history of juvenile idiopathic arthritis presented with flu-like symptoms, systemic inflammation with myocarditis, and severe cardiomyopathy. Six weeks earlier, her chronic-arthritis therapy had been changed from anakinra, an interleukin-1β receptor antagonist, to etanercept. When she resumed taking anakinra, her condition improved dramatically, including a complete recovery of ventricular function.Myocarditis is a well-recognized complication of systemic vasculitides. This unusual case emphasizes the important pathophysiologic role of interleukin receptors in the successful treatment of myocarditis. We suggest that clinical cardiologists be aware of the therapeutic usefulness of biological agents such as anakinra in patients with rheumatic conditions.Key words: Anti-inflammatory agents/therapeutic use, antirheumatic agents/therapeutic use, arthritis, juvenile rheumatoid/complications/drug therapy, disease susceptibility, heart/drug effects, interferon-beta/therapeutic use, interleukin 1 receptor antagonist protein, myocarditis/diagnosis, treatment outcome, ventricular dysfunction, left/drug effectsCardiac involvement in chronic rheumatic conditions is well recognized and is possibly associated with higher cardiovascular morbidity and mortality rates. The cardiac manifestations include vasculitis, accelerated atherosclerosis, congestive heart failure, valvular abnormalities, pulmonary hypertension, conduction system abnormalities, pericarditis, and myocarditis. Immunologic mediators such as interleukin-1, tumor necrosis factor (TNF), and other cytokines play a key role in the pathophysiologic course of chronic rheumatic conditions and their cardiac manifestations. It is crucial to recognize cardiac abnormalities and to initiate anti-inflammatory therapies promptly. We describe the case of a young woman with a history of juvenile idiopathic arthritis who presented with myocarditis, and we discuss the outcome of her therapy with an interleukin-1β receptor antagonist.     

Pain Intensity Variability and Its Relationship With Quality of Life in Youths With Juvenile Idiopathic Arthritis

Objective

To describe variability of pain intensity experienced by youths with juvenile idiopathic arthritis (JIA) and examine factors related to within‐day patterns of pain and the relationship between magnitude of pain variability and quality of life.

Methods

Pain intensity was self‐reported on a visual analog scale (VAS; range 0–100) by 112 youths with JIA ages 8–18 years using electronic diaries 3 times per day for 7 days. Average absolute change in pain (AAC) was computed as a measure of the magnitude of pain variability for each participant. Logistic regression was used to examine the relationship between demographic and disease characteristics and the probability of having high pain variability (AAC ≥10 VAS units). Linear regression was used to examine the relationship between quality of life (assessed by the Pediatric Quality of Life Inventory) and AAC. The generalized estimating equations approach was used to examine the relationship between the time of day and pain intensity.

Results

The mean ± SD AAC was 15.6 ± 10.5. The majority of youths (65%) had high AAC (≥10 VAS units). Disease severity predicted high pain variability (β = 0.02, P = 0.044). Higher AAC predicted lower quality of life (adjusted R² = 0.194, β = ?0.59, P = 0.003). Within‐day patterns of pain intensity varied by JIA subtype and sex.

Conclusion

This study characterized the pain intensity variability experienced by youths with JIA. Pain variability throughout the day was common, varied by JIA subtype and sex, and was related to quality of life. These findings have implications for future pain research, patient education, and development of clinical interventions for this population.

     

Impact of Uveitis on Quality of Life in Adult Patients With Juvenile Idiopathic Arthritis

Objective

To establish the impact of uveitis on the quality of life (QoL) in adult patients with juvenile idiopathic arthritis (JIA ).

Methods

Adult patients with a history of JIA , both with (n = 31) or without (n = 51) chronic anterior uveitis, were included. Their scores on 3 validated QoL questionnaires (National Eye Institute Visual Functioning Questionnaire [NEI VFQ ‐25], Medical Outcomes Study 36‐Item Short Form health survey [SF ‐36], and EuroQol 5‐domain questionnaire [EQ ‐5D]) were analyzed to find factors that could influence QoL.

Results

The median overall composite score (OCS ) of the NEI VFQ ‐25 was significantly worse in the uveitis group compared to the non‐uveitis group (respectively, 83.4 [range 15.2–94.7] and 94.9 [range 46.3–100]; P < 0.001). Nearly all subscale scores were lower in patients with uveitis than in patients without uveitis (P < 0.001 for all). After adjusting for duration of arthritis, JIA subtype, arthritis onset before or after 1990, and the use of systemic immunomodulatory medication, the QoL was still worse in patients with uveitis (NEI VFQ ‐25 OCS regression coefficient = ?11.7; P = 0.002). No significant differences were found between the groups for the SF ‐36 and the EQ ‐5D. In the total JIA group, the use of systemic medication appeared to negatively influence some general QoL scores.

Conclusion

Having a history of uveitis has a substantial negative effect on the vision‐related QoL in JIA in adulthood, despite good visual acuity. General QoL scores did not differ between uveitis and non‐uveitis patients, but the use of systemic immunomodulatory treatment, independent of uveitis, did negatively influence general QoL scores in adult JIA patients.

     

Remission in Juvenile Idiopathic Arthritis: Current Facts

Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthritic disease affecting children. Despite the availability of potent disease-modifying antirheumatic medications, most children still experience a chronic course with long periods of active disease. Goals of treatment should include achievement of disease remission with optimal physical functioning that allows children to lead normal lives with no structural joint damage. The term remission implies a complete lack of disease activity. This article focuses on recently developed preliminary criteria for inactive disease and remission in JIA. Recent studies using these new definitions demonstrate only modest rates of achievement of remission favoring children with persistent oligoarticular JIA. Children with rheumatoid factor-positive polyarticular JIA are least likely to achieve remission. Therapeutic strategies to achieve remission are also discussed.     

Brief Report: Incidence of Selected Opportunistic Infections Among Children With Juvenile Idiopathic Arthritis

Objective

To compare incidence rates of selected opportunistic infections among children with and children without juvenile idiopathic arthritis (JIA).

Methods

Using US national Medicaid administrative claims data from 2000 through 2005, we identified a cohort of children with JIA based on physician diagnosis codes and dispensed medications. We also identified a non‐JIA comparator cohort of children diagnosed as having attention deficit hyperactivity disorder (ADHD). We defined 15 types of opportunistic infection using physician diagnosis or hospital discharge codes; criteria for 7 of these types also included evidence of treatment with specific antimicrobial agents. We calculated infection incidence rates. The rates in the ADHD comparator cohort were standardized to the age, sex, and race distribution of the JIA cohort. We calculated incidence rate ratios (IRRs) with 95% confidence intervals (95% CIs) to compare infection rates.

Results

The JIA cohort included 8,503 children with 13,990 person‐years of followup. The ADHD comparator cohort included 360,362 children with 477,050 person‐years of followup. When all opportunistic infections were considered together as a single outcome, there were 42 infections in the JIA cohort (incidence rate 300 per 100,000 person‐years; IRR 2.4 [95% CI 1.7–3.3] versus ADHD). The most common opportunistic infections among children with JIA were 3 cases of Coccidioides (incidence rate 21 per 100,000 person‐years; IRR 101 [95% CI 8.1–5,319] versus ADHD), 5 cases of Salmonella (incidence rate 35 per 100,000 person‐years; IRR 3.8 [95% CI 1.2–9.5]), and 32 cases of herpes zoster (incidence rate 225 per 100,000 person‐years; IRR 2.1 [95% CI 1.4–3.0]).

Conclusion

Opportunistic infections are rare among children with JIA. Nevertheless, children with JIA had a higher rate of opportunistic infections, including an increased rate of Coccidioides, Salmonella, and herpes zoster compared to children with ADHD.