Pediatric and adult osteoporosis: a contrasting mirror.

Pediatric osteoporosis (PO) is a condition that is currently gaining recognition. Due to the lack of official definitions over the past few decades, the exact incidence of PO is unknown. The research does not provide a specific prevalence of PO in different world regions. However, this is expected to change with the latest 2019 guidelines proposed by the International Society of Clinical Densitometry. Although adult osteoporosis (AO) has been postulated a pediatric disease because its manifestation in adulthood is a result of the bone mass acquired during childhood, differences between PO and AO should be acknowledged. AO is defined as low bone density; however, PO is diagnosed based on existing evidence of bone fragility (vertebral fractures, pathological fractures). This is particularly relevant because unlike in adults, evidence is lacking regarding the association between low bone density and fracture risk in children. The enhanced capacity of pediatric bone for reshaping and remodeling after fracture is another difference between the two entities. This contrast has therapeutic implications because medication-free bone reconstitution is possible under certain conditions; thus, background therapy is not always recommended. In this narrative review, differences between PO and AO in definition, assessment, and medical approach were investigated.

Probability of the 10-year Risk of Hip and Major Osteoporotic Fracture in Non-radiographic Axial Spondyloarthritis.

BACKGROUND: Fracture risk in non-radiographic spondyloarthritis is underestimated. A reliable tool such as the Fracture Risk Assessment tool (FRAX) may assess this risk probability. This study aimed to assess the fracture risk by the FRAX score in patients with nr-axSpA and to determine factors associated with high fracture risk. METHODS: We conducted a retrospective study of nr-axSpA patients meeting the Assessment of SpondyloArthritis International Society (ASAS) classification criteria for spondyloarthritis. All patients had Bone Mineral Density (BMD) by dual-energy X-ray absorptiometry (DEXA). The 10- year probability of major osteoporotic fracture (MOF) and hip fracture (HF) was calculated using the Fracture Risk Assessment Tool (FRAX). RESULTS: Among 40 patients with nr-axSpA, 27 were women (67.5%). Their mean age was 43.7 ± 12.1 years. The mean disease duration was 3.15 ± 2.7 years. Eighteen patients (45%) had osteopenia, and 12 patients (30%) had osteoporosis. The median HF FRAX was 0% [0-1.2]. The median MOF FRAX was 0.5% [0.3-1.8]. MOF FRAX was positively correlated with age (p = 0.002), disease onset age (p = 0.006), disease duration (p = 0.024), and the modified Stoke Ankylosing Spondylitis Spine Score (mSASSS) (p < 0.0001), and negatively correlated with daily calcium intake (p < 0.0001). HF FRAX was positively correlated with mSASSS (p < 0.0001) and negatively correlated with daily calcium intake (p = 0.005). CONCLUSION: Our study confirmed the frequency of bone loss during nr-axSpA and showed that osteoporotic risk fracture was related not only to traditional risk factors for osteoporosis but also to disease-related factors.

The childhood arthritis radiographic score of the hip: the proposal cut-off value using cluster analysis.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease that affects children. It is crucial to detect and treat hip involvement in JIA early to prevent functional impairment and reduced quality of life. The Childhood Arthritis Radiographic Score of the Hip (CARSH) is a validated radiographic scoring system used to assess hip involvement in JIA. In this study, we aimed to determine cut-off values for CARSH scores using cluster analysis. METHODS: The study was conducted as a cross-sectional analysis and included JIA patients with hip involvement who underwent a pelvic radiograph. The same pelvic radiograph was interpreted by two experienced pediatric rheumatologists at baseline and after 3 weeks by both readers for reliability. The CARSH scores were calculated for each hip four times (twice by each reader). For the 50 hips, a total of 200 interpretations of the CARSH score were obtained. Model-based clustering was employed to identify distinct groups of CARSH score interpretations and characterize the phenotype of each cluster. RESULTS: Twenty-five children with hip involvement were included. The mean age was 13.9 ± 4.6 years. JIA subtypes were as follows: ERA in 64%, oligoarthritis in 16%, psoriatic arthritis in 12%, polyarthritis RF + in 4%, and RF - in 4% of patients. For the 200 hip interpretations, three clusters based on the level of the CARSH were identified by model-based clustering. Cluster 1 consisted of 17 CARSH score interpretations with a median score of 7 ± 3 (ranging from 1 to 15). This group primarily comprised patients with enthesitis-related arthritis (ERA) and psoriatic arthritis. Patients in cluster 1 were generally older, experienced longer diagnostic delays, and had a longer disease duration compared to the other clusters. Cluster 2 exhibited a moderate CARSH score, with an average score of 4 ± 3 (1 to 15). Patients in this group had significantly higher body weight compared to the other clusters. Cluster 3 represented the group with the least severe hip involvement, characterized by CARSH scores of 2 ± 1 (ranging from 0 to 9). This cluster had a higher proportion of male patients and higher C-reactive protein (CRP) levels than the other clusters. Regarding the individual items of the CARSH score, cluster 1 showed higher percentages of hip radiograph abnormalities such as joint space narrowing, erosions, growth abnormalities, and subchondral cysts. Cluster 2 was characterized by a high rate of acetabular sclerosis, with little to no abnormalities in other CARSH score items. Cluster 3 was the only group that exhibited hip subluxation, with minimal abnormalities in the other score items. In conclusion, this study identified three distinct groups of CARSH scores, representing varying levels of severity in hip involvement in JIA. These findings provide valuable insights for clinicians in assessing and managing JIA patients with hip involvement, enabling tailored treatment strategies based on the severity of the condition. Key Points • While a Childhood Arthritis Radiographic Score of the Hip (CARSH) is a valid and reliable tool in hip-related juvenile idiopathic arthritis, its use is limited in daily practice due to the lack of available cut-off values. • The cluster analysis defined three clusters based on the CARSH levels. • Cluster 1 exhibited the highest score with more damage and disability. Cluster 2 involved a moderate score and more overweight patients. Cluster 3 included the least level of the score but with an active disease parameter.

Denosumab use in osteogenesis imperfecta: an update on therapeutic approaches.

Osteogenesis imperfecta (OI) is an inherited skeletal disorder that leads to bone fragility and multiple fractures. Given advances in the genetic understanding of existing phenotypes and newly discovered mutations, therapeutic management of OI has become challenging. Denosumab, a monoclonal antibody that inhibits the interaction between the receptor activator of nuclear factor kappa B ligand (RANKL) and its receptor RANK, has been approved to treat postmenopausal osteoporosis and emerged as an important therapy for malignancies and other skeletal disorders, including pediatric skeletal conditions such as OI. This review summarizes information about denosumab therapy in OI by exploring its mechanisms of action, main indications, and safety and efficacy. Several case reports and small series have been published about the short-term use of denosumab in children with OI. Denosumab was considered a strong drug candidate for OI patients with bone fragility and a high risk of fracture, particularly for patients with the bisphosphonate (BP)-unresponsive OI-VI subtype. The evidence for denosumab's effects in children with OI indicates that it effectively improves bone mineral density but not fracture rates. A decrease in bone resorption markers was observed after each treatment. Safety was assessed by tracking the effects on calcium homeostasis and reporting side effects. No severe adverse effects were reported. Hypercalciuria and moderate hypercalcemia were reported, suggesting that BPs be used to prevent the bone rebound effect. In other words, denosumab can be used as a targeted intervention in children with OI. The posology and administration protocol require more investigation to achieve secure efficiency.

Early Identification of Sacroiliitis in Patients with Suspected Spondyloarthritis: A Challenging Task.

OBJECTIVE: The purpose of this study was to assess the performance of computed tomography (CT) scan and magnetic resonance imaging (MRI) for detecting sacroiliitis in nonradiographic SpA (nr-SpA). METHODS: This cross-sectional monocentric double-blind study included 63 patients consulting for symptoms suggestive of SpA between February 2014 and February 2017. Patients with conventional radiographs showing a confirmed sacroiliitis (grade 3 or 4) were not included. Eligible patients underwent CT and MRI of sacroiliac joints (SIJ). CT and MR images were interpreted by 2 experienced musculoskeletal radiologists blinded to clinical and laboratory data. Two professors in rheumatology blinded to radiologists' conclusions analyzed clinical data, laboratory tests, HLA typing, X-rays, CT and MRI images, and divided the patients into 2 groups: confirmed nr-SpA or no SpA. This classification was considered the gold standard when analyzing the results. RESULTS: 46 women and 17 men were included in this study. 47 patients were classified as confirmed nr-SpA (74.6%) and 16 patients as no SpA (25.4%). Sensitivity, specificity, and positive and negative predictive values of CT and MRI for detecting sacroiliitis were, respectively, estimated at 71.7%, 71.4%, 89.2%, 43.5%, and 51.2%, 100%, 100%, and 40%. CT and MRI findings were found to be statistically associated (p<0.001). CONCLUSION: SIJ MRI is a highly specific method in the detection of sacroiliitis, but with a moderate sensitivity. SIJ CT scan, usually known as the third option after radiography and MRI, has much greater diagnostic utility than it has been documented previously.

Osteonecrosis of the Tarsal Navicular: Not Always Spontaneous!

BACKGROUND: Mueller-Weiss disease, a rare and complex foot condition, is defined as spontaneous and progressive navicular fragmentation leading to midfoot pain and deformity. However, its exact etiopathogenesis remains unclear. We report a case series of tarsal navicular osteonecrosis to describe the clinical and imaging characteristics and etiologic profile of the disease. METHODS: This retrospective study included five women diagnosed as having tarsal navicular osteonecrosis. The following data were extracted from medical records: age, comorbidities, alcohol and tobacco consumption, history of trauma, clinical presentation, imaging modalities performed, treatment protocol, and outcomes. RESULTS: Five women with a mean age of 51.4 years (range, 39-68 years) were enrolled in the study. Mechanical pain and deformity over the dorsum of the midfoot was the main clinical presentation. Rheumatoid arthritis, granulomatosis with polyangiitis, and spondyloarthritis were reported by three patients. Radiographs revealed bilateral distribution in one patient. Three patients underwent computed tomography. It showed a fragmentation of the navicular bone in two cases.Magnetic resonance imaging was performed in one patient showing flattening of the lateral aspect of the navicular bone with signal abnormalities. Talonaviculocuneiform arthrodesis was performed in all of the patients. CONCLUSIONS: Mueller-Weiss disease-like changes may occur in patients with an underlying inflammatory disease such as rheumatoid arthritis and spondyloarthritis.

The concept of telemedicine in pediatric rheumatology in Tunisia: Parents' perceptions.

INTRODUCTION: With the COVID-19 pandemic, health care systems are facing challenges in delivering proper patient care. Children and adolescents with juvenile idiopathic arthritis require specialized and comprehensive attention. In this context, telemedicine is an alternative that has the potential to improve access to healthcare in addition to cost savings. The objective of our study was to evaluate parents' willingness for telemedicine and factors helping to adopt this alternative in the era of COVID-19. METHODS: We undertook a cross-sectional study via structured phone interviews of parents' JIA patients as well as those with no established diagnoses. We evaluated their point of view and willingness to adhere to TM. RESULTS: The study included 40 parents. The main reasons for favoring TM were avoiding hospitals during the pandemic (32.5%), time saving (27.5%) as well as avoiding school absenteeism (27.5%). The main reasons for preferring a live consultation were the fear of a possible discrepancy between physical and distant evaluation (47.5%) and the fear of the trivialization of the disease (38.5%). There was no association between preference for TM and a family history of COVID-19 (p = 0.704) as well as electronic devices afforded (p = 0.263). However, patients who lived away from hospital, not familiar with the concept of TM and with higher income adhered less to TM. CONCLUSION: Unlike the literature data, our study showed the low prevalence of parents willing to accept TM as a model of care. This imply an urgent need for parent and patient education to promote TM especially in pediatric rheumatology.

Factors associated with the inflammatory process in pain in ankylosing spondylitis.

Introduction: sleep disorders, closely related to any chronic pain process, are frequent among patients with rheumatic diseases, mainly ankylosing spondylitis (AS). Our study aimed to determine the association between sleep disturbances and the inflammatory process in pain in AS patients compared with lower back pain (LBP) patients. We have additionally examined factors associated with sleep disorders among AS patients. Methods: we conducted a cross-sectional study among AS patients. Sociodemographic data, patient reported outcomes and disease characteristics were recorded. Sleep was assessed using the medical outcomes study sleep scale measure (MOS-SS). For psychological assessment, Beck anxiety (BAI) and depression index (BDI) was used. A multivariate logistic regression was performed to identify factors associated with sleep disorders. Results: the study included 50 patients with AS and 40 patients with low back pain. The most common affected domains among AS patients were inadequacy, sleep disturbance, and daily somnolence. The MOS-SS index was significantly higher in the AS group than in the control group (p<0.001). Sleep disorder was associated with age, female gender, analphabetism, patient-reported outcomes (all p<0.05), but was not associated with profession, comorbidities and smoking habits. In multivariate analysis, factors associated with sleep disruption were the duration of morning stiffness (MS), disease activity, bath ankylosing spondylitis metrology index (BASMI), ASQol, as well as anxiety and depression (odds ratio: 5.4(CI 95% 1.6-18.3), 9.9 (CI95%1.1-86); 6 (CI95%1.1-32); 13 (CI 95% 1.4-143.8); 15.7 (CI 95% 2.6-94.3); 14 (CI 95% 2-105.7) respectively, p<0.05 for each). Conclusion: our study highlighted the importance of sleep disorders among patients with AS with a predilection for inadequacy, sleep disturbance, and daily somnolence. Factors associated with sleep disruption were high disease activity, a longer duration of MS, an altered function and quality of life as well as anxiety and depression.

Enthesitis-related arthritis: monitoring and specific tools

Abstract Objective: In this article, the authors aimed to review the different tools used in the monitoring of enthesitis-related arthritis. Sources: The authors performed a literature review on PubMed, Google Scholar, and Scopus databases. The dataset included the original research and the reviews including patients with enthesitis-related arthritis or juvenile spondylarthritis up to October 2020. Summary of finding: Enthesitis-related arthritis is a category of juvenile idiopathic arthritis. It is characterized by the presence of enthesitis, peripheral arthritis, as well as axial involvement. The only validated tool for disease activity measurement in juvenile idiopathic arthritis is the Disease Activity Score: It has proven its reliability and sensitivity. Nevertheless, due to an absence of validated evaluation tools, the extent of functional impairment, as well as the children and parents' perception of the disease, could not be objectively perceived. Despite the great progress in the field of imaging modalities, the role they play in the evaluation of disease activity is still controversial. This is partially due to the lack of validated scoring systems. Conclusion: Further work is still required to standardize the monitoring strategy and validate the outcome measures in enthesitis-related arthritis.

Which Metrological Index, the BASMI or EDASMI, is Best Correlated with Disease-Related Parameters in Spondylarthritis Patients?

OBJECTIVE: This study aimed at comparing the Edmonton Ankylosing Spondylitis Metrology Index (EDASMI) and Bath Ankylosing Spondylitis Metrology Index (BASMI) to determine which of the two is best correlated with disease-related parameters in axial spondyloarthritis (axSpA) patients. METHODS: A cross-sectional study was carried out involving 86 patients with radiographic axSpA. Sociodemographic data, the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), the Ankylosing Spondylitis Disease Activity Score (ASDAS), the Bath Ankylosing Spondylitis Functional Index (BASFI), and the Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire were applied. Spinal mobility was assessed by two indices: the BASMI and the EDASMI. Structural damage of the spine was also evaluated by two indices: the Bath Ankylosing Spondylitis Radiology Index (BASRI) and the modified Stoke Ankylosing Spondylitis Spine Score (mSASSS). RESULTS: Eighty-six patients with an average age of 43.21 ± 11.43 years (20-79) were included. Impaired spinal mobility, which corresponds to higher BASMI scores, was correlated with prolonged disease duration (p < 0.01, r = 0.310), higher ASDAS-CRP (p < 0.001, r = 0.386), severe functional disability on the BASFI (p < 0.01, r = 0.505) and poorer quality of life according to the ASQoL (p < 0.01, r = 0.369). However, the EDASMI score did not correlate with any disease parameter. The BASMI was correlated with the total BASRI (p < 0.01, r = 0.634) and mSASSS (p < 0.01, r = 0.388). Unlike the BASMI, the EDASMI was neither correlated with the BASRI (p = 0.520, r = 0.245) nor the mSASSS (p = 0.252, r = -0.120). CONCLUSION: Our results indicate that among the studied metrological indices, the BASMI is more contributory since it is correlated with clinical disease parameters and structural damage, unlike the EDASMI.

Vertebral sarcoidosis: diagnosis to management.

Sarcoidosis is a systemic inflammatory granulomatous disease that can develop in almost any organ system. Rheumatologists may encounter sarcoidosis in different situations varying from arthralgia to bone involvement. While the peripheral skeleton was a frequent location, data regarding axial involvement is scarce. Most patients with vertebral involvement have a known diagnosis of intrathoracic sarcoidosis. They tend to report mechanical pain or tenderness over the involved area. Imaging modalities, particularly Magnetic Resonance Imaging (MRI), are a mainstay of axial screening. It helps exclude differential diagnoses and delineate the extent of bone involvement. Histological confirmation combined with the ap- propriate clinical and radiological presentation is the key of diagnosis. Corticosteroids remain the cornerstone of treatment. In refractory cases, methotrexate is the steroid- sparing agent of choice. Biologic therapies may be used, although the evidence base for their efficacy is bone sarcoidosis controversial.

Enthesitis-related arthritis: monitoring and specific tools.

OBJECTIVES: In this article, the authors aimed to review the different tools used in the monitoring of enthesitis-related arthritis. SOURCES: The authors performed a literature review on PubMed, Google Scholar, and Scopus databases. The dataset included the original research and the reviews including patients with enthesitis-related arthritis or juvenile spondylarthritis up to October 2020. SUMMARY OF FINDING: Enthesitis-related arthritis is a category of juvenile idiopathic arthritis. It is characterized by the presence of enthesitis, peripheral arthritis, as well as axial involvement. The only validated tool for disease activity measurement in juvenile idiopathic arthritis is the Disease Activity Score: It has proven its reliability and sensitivity. Nevertheless, due to an absence of validated evaluation tools, the extent of functional impairment, as well as the children and parents' perception of the disease, could not be objectively perceived. Despite the great progress in the field of imaging modalities, the role they play in the evaluation of disease activity is still controversial. This is partially due to the lack of validated scoring systems. CONCLUSIONS: Further work is still required to standardize the monitoring strategy and validate the outcome measures in enthesitis-related arthritis.