Chronic recurrent multifocal osteomyelitis is a rare auto-inflammatory disease in children, with only a few reports of its association with other inflammatory diseases, such as systemic juvenile idiopathic arthritis. A 15-year-old boy was admitted due to fever, skin rash, arthritis, and high inflammatory factors and was finally diagnosed with systemic juvenile idiopathic arthritis. After 6 months of recovery from the disease, the patient was referred due to local pain and swelling in the arms and left thigh. In radiography, bone lesions were seen in the shoulders, left humerus, and left femoral diaphysis. A whole-body bone scan showed increased absorption in these areas, which suggested a tumor or osteomyelitis. A biopsy of the bone lesion of the left humerus confirmed sterile osteomyelitis. Although the co-incidence of chronic recurrent multifocal osteomyelitis with systemic juvenile idiopathic arthritis is rare, it should be considered in differential diagnosis.

UNASSIGNED: Chronic nonbacterial osteomyelitis (CNO) is a rare disease of unknown etiology and most commonly occurs during childhood or adolescence. The purpose of this study is to collect data on the clinical features, outcomes, and management of the disease and to identify the factors affecting recurrence.
UNASSIGNED: This is a retrospective multicenter cross-sectional study of pediatric patients diagnosed with CNO. A total of 87 patients with a diagnosis of CNO followed for at least 6 months in 8 pediatric rheumatology centers across the country between January 2010 and December 2021 were included in this study.
UNASSIGNED: The study included 87 patients (38 girls, 49 boys; median age: 12.5 years). The median follow-up time was 20 months (IQR: 8.5-40). The median time of diagnostic delay was 9.9 months (IQR: 3-24). Arthralgia and bone pain were the most common presenting symptoms. Multifocal involvement was detected in 86.2% of the cases and a recurrent course was reported in one-third of those included in the study. The most commonly involved bones were the femur and tibia. Vertebrae and clavicles were affected in 19.5% and 20.6% of cases, respectively. The erythrocyte sedimentation rate (ESR) values of 60.9% of the patients were above 20 mm/h and the C-reactive protein values of 44.8% were above 5 mg/L. The remission rate was 13.3% in patients using nonsteroidal antiinflammatory drugs and 75.0% in those using biological drugs. Vertebral and mandibular involvement and high ESR values at the time of diagnosis were associated with recurrence.
UNASSIGNED: In this multicenter study, CNO with vertebral and mandibular involvement and high ESR at diagnosis were associated with recurrence.

(1) Background: Whole-body magnetic resonance imaging (WB-MRI) is central to defining total inflammatory burden in juveniles with arthritis. Our aim was to determine and compare the initial distribution of lesions in the WB-MRI in patients with chronic recurrent multifocal osteomyelitis (CRMO), juvenile idiopathic arthritis (JIA), their overlapping syndrome (OS), and with Non-specific Arthropathy (NA). (2) Methods: This retrospective single center study was performed on an Avanto 1.5-T MRI scanner with a dedicated multichannel surface coil system. A total of 173 pediatric patients were included with the following final diagnoses: CRMO (15.0%), JIA (29.5%), OS (4.6%), and NA (50.9%). (3) Results: Bone marrow edema (BME) was the most common abnormality, being seen in 100% patients with CRMO, 88% with OS, 55% with JIA, and 11% with NA. The bones of the lower extremities were the most affected in all compared entities. Effusion was seen in 62.5% children with OS, and in 52.9% with JIA, and in CRMO and NA, the exudate was sporadic. Enthesitis was found in 7.8% of patients with JIA and 3.8% with CRMO, and myositis was seen in 12.5% of patients with OS and in 3.9% with JIA. (4) Conclusions: The most frequent indication for WB-MRI in our center was JIA. The most common pathology in all rheumatic entities was BME, followed by effusion mainly seen in in OS and JIA. Enthesitis and myositis were less common; no case was observed in NA.

Chronic non-bacterial osteomyelitis (CNO) is an autoinflammatory bone-disease of unknown origin. The National Pediatric Rheumatologic Database (NPRD) collects long-term data of children and adolescents with rheumatic diseases including CNO.
To assess characteristics, courses, and outcomes of CNO with onset in childhood and adolescence and to identify outcome predictors.
From 2015 to 2021 patients with a confirmed diagnosis of CNO, who were registered in the NPRD during their first year of disease and at least one follow-up visit, were included in this analysis and observed for up to 4 years.
Four hundred patients with recent diagnosis of CNO were enrolled in the NRPD during the study period. After 4 years, patient data documentation was sufficient to be analyzed in 81 patients. A significant decline of clinical and radiological lesions is reported: at inclusion in the registry, the mean number of clinical lesions was 2.0 and 3.0 MRI lesions per patient. A significant decrease of manifestations during 4 years of follow-up (mean clinical lesions 0.5, p < 0.001; mean MRI lesions 0.9 (p < 0.001)) was documented. A significant improvement of physician global disease activity (PGDA), patient-reported overall well-being, and childhood health assessment questionnaire (C-HAQ) was documented. Therapeutically, an increase of disease-modifying anti-rheumatic drugs over the years can be stated, while bisphosphonates rather seem to be considered as a therapeutic DMARD option in the first years of disease. Only 5-7% of the patients had a severe disease course as defined by a PGDA >  = 4. Predictors associated with a severe disease course include the site of inflammation (pelvis, lower extremity, clavicle), increased erythrocyte sedimentation rate, and multifocal disease at first documentation. The previously published composite PedCNO disease activity score was analyzed revealing a PedCNO70 in 55% of the patients at 4YFU.
An improvement of physician global disease activity (PGDA), patient reported overall well-being and imaging-defined disease activity measures was documented, suggesting that inactivity of CNO disease can be reached. PedCNO score and especially PGDA, MRI-defined lesions and in a number of patients also the C-HAQ seem to be reliable parameters for describing disease activity. The identification of risk factors at the beginning of the disease might influence treatment decision in the future.

Chronic recurrent multifocal osteomyelitis (CRMO) is a non-infectious, inflammatory disorder of the bones. CRMO typically affects children, with a predisposition to females. Bone-related pain is often felt in the metaphysis of long bones, particularly of the lower extremities, but it can also target other sites at varied time intervals. Patients are likely to complain of tenderness and swelling that may cause considerable disability and adversely impact quality of life. There are three main pathophysiological mechanisms that have been hypothesized to drive CRMO including imbalanced cytokine expression, increased inflammasome activation, and enhanced osteoclast differentiation. Therapies have been based on targeting and suppressing these key players in CRMO patients. The first step in management involves pain control. Non-steroidal anti-inflammatory drugs should provide initial relief, albeit temporarily. It is imperative to initiate immunosuppressive medication that will help limit bone involvement and thereby prevent the development of fractures or leg-length discrepancies, for example. The purpose of this literature review is to study the pathophysiology of CRMO and carefully dissect the agents that have been previously employed in the management of CRMO patients. This could allow for the purposeful formulation of individualized care plans and improving the overall well-being of patients. The authors included a multitude of PubMed-indexed articles published from 2000 onwards in this review.

BACKGROUND: Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by chronic vasculitis involving small to medium sized arteries, granulomatous inflammation of the upper and lower respiratory tracts, pauci-immune necrotizing glomerulonephritis, as well as vasculitis of other organs. Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory syndrome characterized by sterile bone inflammation.
METHODS: We report a case of CRMO that was doing well on non-steroidal anti-inflammatory drugs (NSAID for 6 years and then developed ANCA positive limited GPA presenting with pyoderma gangrenosum, persistent bilateral otalgia with serous otitis, otorrhea, then sensorineural hearing loss.
CONCLUSIONS: This is the first report of limited GPA initially presenting as pyoderma gangrenosum in a patient with underlying CRMO. It is unclear how the pathology of an autoimmune and an autoinflammatory condition can overlap.

暂无摘要。

Prospective comparative effectiveness research (CER) in chronic nonbacterial osteomyelitis (CNO) is lacking. Our objectives were to (1) determine the use and safety of each consensus treatment plan (CTP) regimen for CNO, (2) assess the feasibility of using the Chronic Nonbacterial Osteomyelitis International Registry (CHOIR) data for CER, and (3) develop and validate a CNO clinical disease activity score (CDAS) using CHOIR.
Consenting children or young adults with CNO were enrolled into CHOIR. Demographic, clinical, and imaging data were prospectively collected. The CNO CDAS was developed through a Delphi survey and nominal group technique. External validation surveys were administered to CHOIR participants.
One hundred forty (78.2%) CHOIR participants enrolled between August 2018 and September 2020 received at least 1 CTP regimen. Baseline characteristics from different CTP groups were well matched. Patient pain, patient global assessment, and clinical CNO lesion count were key variables included in the CNO CDAS. The CDAS showed a strong correlation with patient/parent report of difficulty using a limb, back, or jaw and patient/parent report of disease severity, but a weak correlation with patient/parent report of fatigue, sadness, and worry. The change in CDAS was significant in patients reporting disease worsening or improvement (P < 0.001). The CDAS significantly decreased after initiating second-line treatments from median 12.0 (IQR 8.0-15.5) to 5.0 (IQR 3.0-12.0; P = 0.002). Although second-line treatments were well tolerated, psoriasis was the most common adverse event.
The CNO CDAS was developed and validated for disease monitoring and assessment of treatment effectiveness. CHOIR provided a comprehensive framework for future CER.

Autoinflammatory bone disorders are a group of diseases characterized by sterile osteomyelitis. This includes chronic nonbacterial osteomyelitis and the monogenic forms, Majeed syndrome and deficiency of the interleukin-1 receptor antagonist. These disorders result from innate immune system dysregulation and cytokine imbalance that triggers inflammasome activation causing downstream osteoclastogenesis and excessive bone remodeling. In this review, we will summarize the immunopathogenesis of pediatric autoinflammatory bone diseases with a special focus on the genetics and inborn errors of immunity, while briefly touching on the clinical manifestations and management of each disease as well as areas for future research.

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory disease, clinically characterized by chronic and recurrent episodes of osteoarticular inflammation, that generally presents in children and adolescents. From a dermatological point-of-view, CMRO can be associated with skin rashes mainly including psoriasis, palmoplantar pustulosis and acne. Pyoderma gangrenosum (PG) is a rare immune-mediated inflammatory skin disease classified within the spectrum of neutrophilic dermatoses that, in some cases, has been reported as cutaneous manifestation in CMRO patients. This paper presents a 16-year female patient diagnosed with CMRO, who presented PG lesions located on the lower leg, that arose after the administration of the tumour necrosis factor (TNF)-α inhibitor adalimumab. Cases of PG have been reported in patients being treated with certain medications, including TNF-α antagonists, leading to classified them in a setting aptly termed \"drug-induced PG.\" In this paper, we discuss the co-occurrence of PG and CRMO, in the light of recent evidence on the pathogenesis of both diseases and giving ample space to a literature review on drug induced PG. In our case, it is plausible that PG could be considered a cutaneous manifestation of CRMO, although the mechanisms underlying this intriguingly relationship remain to be fully unraveled.