Clinical Characteristics of Adolescents With Juvenile Idiopathic Arthritis Transitioning to Adult Rheumatology Care in Canada: Results From the CAPRI Registry.

OBJECTIVE: Using Canadian Alliance of Pediatric Rheumatology Investigators (CAPRI) juvenile idiopathic arthritis (JIA) registry data, we describe (1) clinical characteristics of patients with JIA transitioning to adult care, (2) prevalence of disease-related damage and complications, and (3) changes in disease activity during the final year prior to transfer. METHODS: Registry participants who turned 17 years between February 2017 and November 2021 were included. Clinical characteristics and patient-reported outcomes (PROs) at the last recorded pediatric rheumatology visit, and changes observed in the year prior to that visit were analyzed. Physicians completed an additional questionnaire characterizing cumulative disease-related damage and adverse events by age 17 years. RESULTS: At their last visit, 88 of 131 participants (67%) had inactive and 42 (32%) had active disease. Overall, 96 (73%) were on medications and 41 (31%) were on biologic disease-modifying antirheumatic drugs. Among 80 participants for whom the additional questionnaire was completed, 26% had clinically detected joint damage, 31% had joint damage on imaging, 14% had uveitis, and 7.5% had experienced at least 1 serious adverse event. During the final year, 44.2% of patients were in remission, 28.4% attained inactive disease, and 27.4% became or remained active. Mean scores of PROs were stable overall during that last year, but a minority reported marked worsening. CONCLUSION: A substantial proportion of youth with JIA transitioning to adult care in Canada had a high disease burden, which was reflected by their degree of disease activity, joint damage, or ongoing medication use. These results will inform pediatric and adult providers of anticipated needs during transition of care.

Chronic recurrent multifocal osteomyelitis with a comprehensive approach to differential diagnosis of paediatric skull pain.

A girl in middle childhood was referred to rheumatology with a 1-month history of progressive skull pain, preceded by fleeting musculoskeletal symptoms. Apart from a scaly rash on her scalp, she was well, with moderately elevated inflammatory markers. Skull imaging (radiographs, CT and MRI) revealed osteolytic lesions, soft tissue swelling and pachymeningeal enhancement at frontal and temporal convexities. Langerhans cell histiocytosis, bone infection/inflammation or malignancy was considered. Skin and bone biopsies eventually ruled out mimicking diseases and confirmed the diagnosis of chronic recurrent multifocal osteomyelitis (CRMO). She was treated with intravenous pamidronate (IVPAM) for 9 months, with rapid resolution of pain and gradual resolution of bony abnormalities. She remains in remission at 15-month follow-up. While CRMO can affect any bone, skull involvement is extremely rare, with a broad differential diagnosis. We recommend bone biopsy to confirm skull CRMO. The patient achieved excellent clinical and radiological response to IVPAM.

Severe bilateral Legg-Calvé-Perthes resolved with pamidronate in combination with casts, physiotherapy and adductor tenotomy: a pictorial essay over 11 years.

We describe an 11-year prospective clinical and radiologic course of a 6-year-old boy with bilateral Legg-Calvé-Perthes disease, who was treated with intravenous pamidronate (IV-PAM). His baseline radiographs showed grade IV avascular necrosis/Catterall stage IV, and at worst he progressed to lateral pillar/Herring stage C bilaterally. His disease initially was extremely functionally limiting with expected poor outcome with eventual joint replacement. Because IV-PAM stops bone breakdown and allows for ongoing bone formation while revascularisation of bone occurs, we hypothesised that IV-PAM could act as an adjunct to traditional treatment to help heal the femoral heads. Our patient received nine once monthly doses of IV-PAM (1 mg/kg/dose) over 13 months, along with Petrie/broomstick casts and physiotherapy. Remarkably, over time, his femoral heads healed. Now, at 11-year follow-up, he has excellent functional and radiologic outcome with congruence between femoral head and acetabulum, no residual osteonecrosis and minimal loss of femoral head sphericity.

Widespread osteonecrosis in children with leukemia revealed by whole-body MRI.

BACKGROUND: Confirmation of early long-bone epiphyseal osteonecrosis in pediatric patients with leukemia allows for medical and surgical intervention before articular surface collapse. MRI detects early osteonecrosis, but multiple focused MR images are required to capture all lesions. QUESTIONS/PURPOSES: We determined whether whole-body MRI (WB-MRI) could (1) assist in diagnosing long-bone epiphyseal and other osteonecroses, (2) characterize articular surface involvement, and (3) detect preferential sites for osteonecrosis. PATIENTS AND METHODS: We retrospectively reviewed prospectively collected data on all 11 pediatric patients newly diagnosed with leukemia who had musculoskeletal pain develop that persisted 4 weeks or more during leukemia treatment. All were screened for osteonecrosis by WB-MRI, which consisted of a one-time scan of the entire body. Osteonecrosis was defined as circumscribed lesions with a distinct rim of low signal intensity in the normally high-intensity marrow on T1-weighted images and high signal intensity in the normally low-intensity marrow on short-tau inversion recovery images. RESULTS: WB-MRI confirmed osteonecrosis in nine of 11 patients. All patients had multisite lesions; eight had long-bone epiphyseal lesions, which comprised 66 of 129 (51%) of all lesions. Osteonecrosis involving greater than 50% of the epiphyseal surface was present in 57% of distal femoral and proximal tibial lesions. All humeral and femoral head lesions involved more than 1/3 of the medial surface volume but were asymptomatic. No articular collapse was present. All osteonecrotic lesions were more common in the lower extremities. CONCLUSIONS: WB-MRI confirmed early epiphyseal osteonecrosis, with quantification of articular surface involvement. Lower limbs were preferentially affected, but asymptomatic humeral head osteonecrosis was present in five of nine patients. LEVEL OF EVIDENCE: Level IV, diagnostic study. See Instructions for Authors for a complete description of levels of evidence.