Demographic, clinical, and treatment characteristics of the juvenile primary fibromyalgia syndrome cohort enrolled in the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry

Abstract

Background To describe the demographic, clinical, and treatment characteristics of youth diagnosed with juvenile primary fibromyalgia syndrome (JPFS) who are seen in pediatric rheumatology clinics. Methods Information on demographics, symptoms, functioning, and treatments recommended and tried were obtained on patients with JPFS as part of a multi-site patient registry (the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry). Data were summarized using descriptive statistics. In a subset of patients completing registry follow-up visits, changes in symptoms, pain, and functioning were evaluated using growth modeling. Results Of the 201 patients with JPFS enrolled in the registry, most were Caucasian/White (85%), non-Hispanic (83%), and female (84%). Ages ranged from 9 to 20 years (M = 15.4 + 2.2). The most common symptoms reported were widespread musculoskeletal pain (91%), fatigue (84%), disordered sleep (82%), and headaches (68%). Pain intensity was rated as moderate to severe (M = 6.3 + 2.4/10). Scores on measures of functioning indicated mild to moderate impairment, with males observed to report significantly greater impairments. For the 37% of the initial cohort having follow-up data available, indicators of function and well-being were found to either worsen over time or remain relatively unchanged. Conclusions The symptoms of JPFS remained persistent and disabling for many patients treated by pediatric rheumatologists. Further study appears warranted to elucidate gender differences in the impact of JPFS symptoms. Work also is needed to identify accessible and effective outpatient treatment options for JPFS that can be routinely recommended or implemented by pediatric rheumatology providers.

Juvenile primary Fibromyalgia Syndrome: epidemiology, etiology, pathogenesis, clinical manifestations and diagnosis

“…Pain severity, as well as measures of function and well-being, are significantly worse in youth with JPFS, as compared to those with other pediatric rheumatic diseases, as was shown in a study of 7753 patients enrolled in a multinational registry [ 81 ]. Functional impairment was significantly greater in males [ 76 ].…”

“…Sleep disturbances are another common manifestation of JPFS, including general fatigue, poor sleep, and feeling tired and unrefreshed upon waking in the morning [ 3 , 10 – 12 ]. In a study of 201 JPFS patients enrolled in a multi-site patient registry, fatigue and disordered sleep were reported by 84 and 82% of patients, respectively [ 76 ]. Sleep disorders can have a direct negative impact on FM patients’ quality of life.…”

“…The characteristic symptom of JPFS is widespread musculoskeletal pain, usually with a very high subjective severity score [3,[74][75][76] . In adolescents with JPFS, pain has a negative effect on well-being and is associated with functional impairment, commonly leading to avoidance of regular daily activities [77,78], school absenteeism [79], and poor social functioning [1,80].…”

Juvenile primary fibromyalgia syndrome: A Review- Treatment and Prognosis

“…Interestingly, in addition to physical and emotional impairment, the JPFS group did not achieve similar levels of education and fewer were married as compared to healthy control subjects of similar ages. In a summary of data on youth with JPFS, enrolled in the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry, indicators of function and well-being were found to either worsen over time or remain relatively unchanged [ 103 ].…”

Clinical Characterization of Juvenile Fibromyalgia in a Multicenter Cohort of Adolescents Enrolled in a Randomized Clinical Trial

“…There has been little effort to comprehensively phenotype JFM symptom characteristics in a representative pediatric population. Two published studies (Connelly et al [10] and Weiss et al [11]) described the clinical characteristics of JFM in a relatively large sample of youth with JFM using data from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) registry of North American Rheumatology clinics (12). JFM patients in the registry were found to have several comorbid symptoms along with the highest levels of pain and pain‐related disability compared to youth with rheumatic diseases (e.g., juvenile arthritis, childhood‐onset lupus, etc.).…”