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.

Anterior Chest Wall Non-traumatic Arthropathies: A Crucial but Often Overlooked Site.

OBJECTIVE: The purpose of this study was to describe the distribution of Anterior Chest Wall (ACW) arthropathies in a tertiary care center and identify clinical, biological and imaging findings to differentiate osteoarthritis (OA) from non-osteoarthritis (N-OA) etiologies. METHODS: Search from medical records from January 2009 to April 2022, including patients with manubriosternal and/or sternoclavicular and/or sternocostal joint changes confirmed by ultrasonography, computed tomography or magnetic resonance imaging. The final study group was divided into OA and N-OA subgroups. RESULTS: A total of 108 patients (34 males and 74 females, mean age: 47.3 ± 13 years) were included. Twenty patients had findings of OA, while 88 were diagnosed with N-OA pathologies. SpA was the most common etiology in the N-OA group (n = 75). The other N-OA etiologies were less common: rheumatoid arthritis (n = 4), Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome (n = 3), infectious arthritis (n = 3) and microcrystalline arthropathies (n = 3). Regarding the distinctive features, ACW pain was the inaugural manifestation in 50% of patients in OA group and 18.2% of patients in N-OA group (p = 0.003); high inflammatory biomarkers were more common in N-OA group (p = 0.033). Imaging findings significantly associated with OA included subchondral bone cysts (p < 0.001) and intra-articular vacuum phenomenon (p < 0.001), while the presence of erosions was significantly associated with N-OA arthropathies (p = 0.019). OA was independently predicted by the presence of subchondral bone cysts (p = 0.026). CONCLUSION: ACW pain is a common but often underestimated complaint. Knowledge of the different non-traumatic pathologies and differentiation between OA and N-OA etiologies is fundamental for appropriate therapeutic management.

Management of Blau syndrome: review and proposal of a treatment algorithm.

Blau syndrome is a rare genetic granulomatosis affecting children. It could be responsible for vision-threatening complications and articular deformation. Due to the rarity of this disease, there are no standardized guidelines for its management. This work aimed to provide an updated overview of the different therapeutic options for Blau syndrome. We conducted research in the PubMed database for the different treatments used in Blau syndrome patients, and we proposed a therapeutic algorithm for disease management. High doses of corticosteroids are considered as a bridging therapy in Blau syndrome. Methotrexate should be initiated if the patient has articular or ocular involvement. An anti-tumor necrosis factor α should be added for patients with uveitis or residual arthritis. If the patient remains symptomatic, a switch to another anti-tumor necrosis factor α is the best option. In non-responders to the first- and second-line biotherapies, a switch to an anti-interleukin 1, an anti-interleukin 6, or tofacitinib is necessary. CONCLUSION: This article suggested an algorithm for the treatment of Blau syndrome. Other studies are necessary to confirm the efficacy of these treatments. WHAT IS KNOWN: • Blau syndrome is a rare but severe granulomatosis that could be responsible for vision-threatening complications and articular deformation. • Blau syndrome seems to be refractory to treatments. WHAT IS NEW: • High doses of corticosteroids are usually insufficient and should be considered only as a bridging therapy. • Blau syndrome could be considered as a poor factor for uveitis, thus, an anti-tumor necrosis factor α should be initiated for patients with uveitis or with residual arthritis.

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.

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.

Predictors factors of atlanto-axial subluxation in Tunisian patients with rheumatoid arthritis.

BACKGROUND: The aim of this study was to describe clinical and imaging features of atlantoaxial subluxation (AAS) and the associated risk factors in patients with rheumatoid arthritis (RA). METHODS: We conducted a retrospective and comparative study including 51 RA patients with AAS and 51 RA patients without AAS. Atlantoaxial subluxation was defined by the presence of an anterior C1C2 diastasis on the cervical spine radiograph in hyperflexion and/or an anterior, posterior, lateral or rotatory C1C2 dislocation on MRI with/without inflammatory signal. RESULTS: In G1, clinical presentations revealing AAS were mainly neck pain (68.7%) and neck stiffness (29.8%). MRI revealed: diastasis C1C2 (92.5%), periodontoid pannus (92.5%), odontoid erosion (23.5%), vertical subluxation (9.8%) and spinal cord involvement (7.8%). A collar immobilisation and corticosteroid boluses were indicated in 86.3% and 47.1% of cases. C1-C2arthrodesis was performed in 15.4% of cases. Atlantoaxial subluxation was significantly associated with: age at disease onset (p = 0.009), history of joint surgery (p = 0.012), disease duration (p = 0.001), rheumatoid factor (p = 0.01), anti-cyclic citrullinated peptide (p = 0.02), erosive radiographic status (p < 0.005), coxitis (p < 0.001), osteoporosis (p = 0.012), extra-articular manifestations (p < 0.001), and high disease activity (p = 0.001). Multivariate analysis identified RA duration (p < 0.001, OR = 1.022 CI[1.01-1.034]) and erosive radiographic status (p = 0.01, OR = 21.236 CI[2.05-219.44]) as predictive factors of AAS. CONCLUSION: Our study showed that longer disease duration and joint destruction are the major predictive factors of AAS. Early treatment initiation, tight-control and regular monitoring of cervical spine involvement are required in these patients.

Primary pachydermoperiostosis associated with pigmented villonodular synovitis: An unknown association?

Primary pachydermoperiostosis is a rare genetic disease affecting the skin and musculoskeletal system. In contrast to secondary hypertrophic osteoarthropathy, primary pachydermoperiostosis is considered a benign condition. While a variety of associated abnormalities have been described in this form, any association with tumors was previously reported in the literature. We hereby describe the first case of a 20-year-old man with primary pachydermoperiostosis revealed by a knee synovial tumor.

Non-pharmacological therapies in Fibromyalgia: New horizons for physicians, new hopes for patients.

INTRODUCTION: Fibromyalgia (FM) is a chronic musculoskeletal condition characterised by reduced quality of life and severe limitations in daily living activities. Considering the wide spectrum of symptoms and the ineffectiveness of a single pharmacological approach, the latest clinical guidelines recommend non-pharmacological therapies as both an alternative and a better-tolerated approach. Several studies have been conducted to determine the effectiveness of non-pharmacological therapies in the management of FM. AIMS: Through a literature review, this paper aims to describe the different complementary therapies and investigate their potential sustainability and effectiveness on FM symptoms in the short and/or long term. METHODS: We searched the PubMed and Google Scholar databases using broad search terms up to June 2022, to identify all types of study designs restricted to human subjects on non-pharmacological therapies in FM. RESULTS: Recent evidence demonstrated that physical activity is the mainstay of therapeutic management, highlighting the relevance of walking as the best method of exercise in FM patients. Nevertheless, adherence to physical activity remains fraught with obstacles that could be overcome with a multimodal and multidisciplinary approach involving a wide range of passive therapies. The effectiveness of passive non-pharmacological therapies remains however unproven in the long term. They can be therefore suggested as 'adjunct' or 'bridge' therapy to improve adherence to physical activity. CONCLUSION: To conclude, FM management requires a multimodal and symptom-based approach, guided by the predominant bothersome symptom on the one hand, and the preferences of each patient on the other hand.

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.

Management of chronic recurrent multifocal osteomyelitis: review and update on the treatment protocol.

INTRODUCTION: Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder primarily affecting children. It is characterized by a peripheral involvement of the metaphysis of long bones rather than axial involvement. Due to the scarcity of the disease, there are no guidelines regarding its management. AREAS COVERED: This review aims to provide an overview of the different therapeutic alternatives and recent protocols. For this reason, first-line and second-line treatment, as well as the impact of new therapies, are discussed in depth. We conducted a search through PubMed on the different aspects of CRMO. Outcomes were categorized as first and second-line treatments. EXPERT OPINION: Non-steroidal anti-inflammatory drugs remain the keystone of CRMO management and are proposed as the first-line treatment. In the case of vertebral involvement, bisphosphonate should be considered, even as a first-line treatment. Several case series and retrospective studies highlight the efficacy of anti-TNF agents. Their use could be an optimal treatment choice for CRMO with comorbid immune-mediated diseases. The potentially favorable effect of interleukin-1 antagonists remains to be determined.

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.

Non-secretory myeloma in young man mimicking the Gorham disease: case report and the literature review.

Multiple myeloma is a neoplasm of plasma cells affecting mostly the elderly with incidence peaks between 60 and 70 years. This disease is exceedingly rare in younger people, especially in adults under 30-year-old. Non-secretory multiple myeloma accounts for 1-5% of all cases of multiple myeloma. It is also a rare condition in young adult patients, and only six cases have been reported [1]. We herein describe a rare case of non-secretory myeloma in a 22-year-old male, explaining from chest wall pain, without general manifestation. Plain radiography and CT scans revealed diffuse osteolytic lesion mimicking the Gorham disease. A bone marrow biopsy was conducted, revealing the diagnosis of myeloma.

Intra-articular hip neurofibromas in von Recklinghausen's disease.

Neurofibromatosis is a genetic disorder with osteo-articular manifestations. The intra-articular location of neurofibroma is scarce.

Complications of Paget Bone Disease: A Study of 69 Patients.

INTRODUCTION: Paget bone disease (PBD) is characterized by a disorder in the bone remodeling activity at sites of involvement. This can produce dramatic alterations of local bone architecture and causes most of the complications. We aimed to focus on the characteristics of complications of PDB among hospitalized patients. MATERIAL AND METHODS: A retrospective study was conducted, on PBD patients hospitalized in two rheumatology centers from 1994 to 2019. Characteristics of the PBD complications were studied. RESULTS: Sixty-nine patients were collected with a sex ratio of 0.76 and a mean age of 75.4±6.4 years [43-101]. The diagnosis of PBD was established in the average age of 64.2±11.5 years. The primary reason for consultation was pain (78.3%). The PBD was localized in the pelvis (58%), lower limb (42%), spine (36.2%), skull (23.2%) and upper limb (5.8%). It was polyostotic in 44.9% of cases. Dosage of ALP was 324 [68-8390]. The PDB complication rate was 52.2% and it decreased over time. The main complication was osteoarthritis (23.2%), followed by deafness (17.4%), fracture (15.9%), hydrocephalus (7.2%), neurological disease (7.2%) and osteosarcoma (1.4%). The presence of complications was significantly associated with the polyostotic form (p=0.01), the skull localization (p=0.04), an increased ALP (p=0.02). CONCLUSION: According to our study, the incidence rate of PBD among hospitalized cases is higher among elderly women and decreases over time. Complications related to PDB are frequent (52%). It concerns patients with a polyostotic form, skull localization and high ALP.

Progressive pseudorheumatoid dysplasia: A rare entity mimicking juvenile idiopathic arthritis.

Progressive pseudorheumatoid dysplasia can be confused with juvenile idiopathic arthritis. Treatment is mainly symptomatic and the prescription of immunosupressive agents is unnecessary. Surgery may be indicated at advanced stages of the disease.

Paget's Disease of Bone in Patients under 40 Years: Two case reports and review of the literature.

Paget's disease of bone (PDB) is a focal disorder of accelerated skeletal remodelling that is uncommon in patients under the age of 40 years; it is more prevalent in older individuals. We report two cases of PDB diagnosed in early adulthood at the Mohamed Kassab Institute of Orthopedics, La Manouba, Tunisia. The first case was a 35-year-old male patient who presented in 2011 with a seven-month history of hip pain. The second case was a 39-year-old female patient who presented 2014 with chronic lower back pain. The PDB diagnosis was confirmed with clinical, biological and radiological investigations. Both patients were doing well on follow-up. Some previous cases have been reported in the literature, differing from the presented cases in some aspects; data of PDB features at differing ages is still insufficient. Early recognition of this clinical entity in young patients is important as early treatment can affect the progression of the disease.

Atypical osteomalacia mimicking radiological features of spondyloarthritis: Never judge a book by its cover.

Longstanding osteomalacia, by its proliferative enthesopathic changes, may mimic the advanced features of SpA. Despite the typical radiological findings, the lack of response to anti-TNF should encourage clinicians to reconsider the diagnosis.