Navigating the Factors Affecting Functional Impairment in Spondyloarthritis.

Objectives: This study aimed to assess the predictive factors of functional impairment in spondyloarthritis (SpA) patients assessed with bath ankylosing spondylitis functional index (BASFI) and Lequesne Index (LI). Methods: This retrospective study was conducted at the Rheumatology Department of Mohamed Kassab Institute of Orthopedics, Manubah, Tunisia, and collected data from 2008 to 2019 over a period of 4 months (August to November 2019). Socio-demographic and disease-related data of SpA patients were collected. Disease activity was assessed using the bath ankylosing spondylitis-global score (BASG-s) and the bath ankylosing spondylitis disease activity index (BASDAI). The spinal mobility was evaluated by the bath ankylosing spondylitis metrology index (BASMI). Structural progression was evaluated with the bath ankylosing spondylitis radiologic index (BASRI) and modified stoke ankylosing spondylitis spine score (mSASSS). A multivariate analysis was done to search for predictive factors associated with BASFI and LI. Results: A total of 263 patients were included. The mean age was 38.9 ± 12.7 years and the gender ratio was 2.7. The mean age of onset of SpA was 27.6 ± 10.8 years and disease duration was 11.3 ± 9.5 years. Occupation was significantly associated with BASFI and LI scores. A significant functional impact was notably correlated with a long duration of the disease. The two scores were correlated with a limitation of spinal mobility (BASMI), a greater disease activity (BASDAI and erythrocyte sedimentation rate) and a greater impact of the disease on health status (BASG-s). Significant functional impairment was also correlated with structural impairment (mSASSS, BASRI and sacroiliitis grade). The variables independently related to BASFI were the mSASSS score and the BASDAI. The variables independently related to LI were profession (unemployed subjects had higher scores), the mSASSS score and the BASMI. Conclusion: Occupation, disease activity, mobility and structural progression predicted functional impairment in Tunisian SpA patients.

Does sacroiliitis is a mandatory criterion for enthesitis-related arthritis diagnosis?

INTRODUCTION AND OBJECTIVES: Magnetic resonance imaging (MRI) sensitivity and specificity seem to be less studied in enthesitis-related arthritis (ERA). We aimed to determine the ability of sacroiliac MRI to diagnose ERA patients. MATERIALS AND METHODS: We conducted a retrospective study including 44 patients with juvenile idiopathic arthritis (JIA). Each patient had a sacroiliac joint MRI. We divided patients into two groups: G1 patients with ERA and G2 patients with non-ERA subtype. RESULTS: ERA was noted in 61% of the cases. Sacroiliac joints were painful in 15 patients (34%). MRI was normal in 25 patients (57%) (G1:11 versus G2:14) and showed bone marrow edema in the sacroiliac joints in 19 patients (34%) (G1=16 versus G2=3, p=0.005). Sacroiliac joints MRI's sensitivity and specificity in the ERA diagnosis were 61.54% and 82.35%, respectively. Positive and negative predictive values were 84.21% and 58.33%, respectively. Furthermore, sacroiliac joint pain in the clinical examination was able to predict sacroiliac bone edema in MRI with an odds ratio of 6.8 (95% CI 1.68-28.09; p=0.006). CONCLUSION: Our study showed that sacroiliac joint MRI has good specificity and positive predictive value in the diagnosis of ERA patients among JIA patients. This underlines the usefulness of sacroiliac joint MRI in the early diagnosis of ERA patients.

Ultrasound Assessment of Psoriatic Arthritis Patients With Clinically Normal Nails and Evaluation of its Correlation with the Disease Activity: A Case-Control Study.

OBJECTIVES: Nail unit is one of the targets of ultrasound (US) assessment. We aimed to compare ultrasound parameters of clinically normal nail unit in psoriatic arthritis (PsA) patients with healthy controls (HC) and evaluate their correlations with disease activity. METHODS: This was a cross-sectional study including patients with PsA and matched HC. Tender (TJC) and swollen joint count (SJC), Psoriasis Area and Severity Index (PASI), and Disease Activity in Psoriatic Arthritis (DAPSA) were collected in PsA patients. Patients underwent US assessment of fingernails with a study of morphological changes and measurement of the thickness of nail bed (NBT), nail plate (NPT), and adjacent skin (ST). Correlation between nail unit parameters and disease activity was studied. RESULTS: We evaluated 22 PsA patients (219 nails) and 21 HC (210 nails). Mean DAPSA was 21.56 ± 14.36 and mean PASI was 2.19 ± 3.8. PsA patients had more US morphological changes than HC (16.89 vs 3.33%, P = .03). NPT comparison between identical fingernails of PsA and HC did not reveal significant difference. However, NBT was significantly higher in HC (1.77 vs 2.07 mm, P = .027) as well as ST (2.26 vs 2.59 mm, P = .003). TJC and ST were positively correlated (r = .46, P = .03). No correlation was noted between disease activity scores and NPT, NBT, or ST in PsA patients. In biologic parameters, ESR was negatively correlated with ST (r = -.41, P = .05). CONCLUSIONS: Nail bed and adjacent skin US morphological changes were contributive to distinguish psoriatic from healthy nails. Adjacent skin thickness measurement was positively correlated with TJC and ESR, suggesting that it could be used as an indicator of disease activity in PsA.

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.

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.

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.

Sarcopenia: a new issue in juvenile idiopathic arthritis. A study Protocol.

INTRODUCTION: The decrease in muscle function and mass is defined as sarcopenia. Known for a long time as an age-related disorder, sarcopenia is nowadays well recognized in childhood. Juvenile idiopathic arthritis (JIA), a chronic inflammatory joint disease may be associated with loss of skeletal mass. AIM: This protocol aims to evaluate the prevalence rate of sarcopenia and its associated factors in JIA. METHODS: To evaluate the prevalence rate and factors associated with sarcopenia in juvenile idiopathic arthritis, we are enrolling 30 children with JIA and 30 healthy children aged between 4-and 16 years. Clinical data will report: age, sex, body mass index, disease duration, and therapeutic management. All participants will undergo the Whole-body Dual-energy X-ray absorptiometry to assess the skeletal muscle mass. The muscle strength will be measured using the handgrip dynamometer and adjusted to the body mass index. Data will be analyzed and compared to age and sex reference curves. RESULTS: This study aims to detect sarcopenia in JIA children and identify subsequently the main associated factors. By collecting anthropometric data and extracting the main features of the disease, specific metrics will be extracted. Body composition will be obtained using the DXA scans, including appendicular lean mass and skeletal muscle mass. Muscle strength will also be assessed. CONCLUSION: This study aims to assess sarcopenia in JIA patients, using the sarcopenia update definition. If we will provide conclusive results, it will be possible to better identify the associated factors of sarcopenia and to prevent children from this complication. Clinical trials registration NCT05291416.

Foot-related impairment in children with juvenile idiopathic arthritis.

AIM: Foot involvement is present in approximately 60%-90% of children with Juvenile idiopathic arthritis (JIA). It is a major cause of disability, which can lead to deterioration in daily activities and quality of life. However, it is often overlooked and can compromise patient management. PURPOSE: Our objective was to describe the ankle and foot involvement in JIA and to appraise the functional impact of this damage on the child's performance and quality of life by using validated scores. METHODS: The cross-sectional study included patients with JIA. Functional impairment was assessed through the Juvenile Arthritis Functionality Scale (JAFS) and the Oxford Ankle and Foot Questionnaire for children. RESULTS: Twenty-three patients aged 12.75 ± 3.9 and with 41 months of disease duration. After completing the Oxford score, the physical domain appeared to be the most altered. A long delay in diagnosis was associated with an alteration in the emotional domain of the Oxford score. Higher disease activity was significantly associated with impairment in both the physical activity and the footwear domains of the Oxford score. As regards clinical examination data, the presence of foot pain as well as the presence of tendinopathy were associated with an alteration of all Oxford score domains. The presence of flat feet significantly affected all domains of the Oxford score. The JAFS was reported to affect the child's performance ability and was associated with impairment in physical activity, school and play, and emotional domains. CONCLUSION: Ankle and foot involvement was common in our study. Functional ability was most impaired in the lower limbs. High disease activity, foot and ankle pain, tenosynovitis, and flat feet were associated with poorer quality of life and higher functional impact.

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.

Psoriatic Arthritis Quality of Life questionnaire: Translation, cultural adaptation and validation into Arabic language.

BACKGROUND: Psoriatic arthritis (PsA) is a multifaceted inflammatory disease that has a strong negative impact on the quality of life (QoL) of patients. The Psoriatic Arthritis Quality of Life (PsAQoL) questionnaire was the first disease-specific patient-derived instrument developed to measure the QoL in patients with PsA. Our objective was to translate the PsAQol into Arabic language and evaluate its reliability and validity in patients with PsA. METHODS: This was a cross-sectional study including patients with PsA. A clinical and biological assessment of the patients was performed at inclusion. The translation of the original PsAQoL into Arabic was performed by a professional bilingual and lay panel. Eight patients were interviewed to assess face and content validity. A separate sample of PsA patients (n = 30) were invited to participate in a test-retest postal study in order to investigate reproducibility and construct validity. One week separated the two administrations. The Arabic version of Health Assessment Questionnaire (HAQ) was used as a comparator instrument for convergent validity. RESULTS: Face and content validity were satisfactory. The Arabic version of the PsAQoL was found to be relevant, understandable and easy to complete in only a few minutes. One item was excluded (item 16). It had no correlation with either the other 19 items or the total score of PsAQol. The Arabic PsAQol had excellent internal consistency (Cronbach's a = 0.926), and test-retest reliability (r = 0.982). There was a positive correlation between the total score of the PsAQoL and the Arabic version of HAQ (Spearman's r = 0.838, p < 10-3 ). Exploratory factor analysis had extracted two factors explaining 55% of the total variance. CONCLUSION: Nineteen items were selected to compose the Arabic version of PsAQoL, which was found to be relevant and understandable and has excellent reliability and construct validity. The new measure will be a valuable new tool for use in routine care for patients' assessment.

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.

Low back pain in pregnant women: A necessary or an avoidable evil?

OBJECTIVES: The aim of this study was to determine the prevalence, the main features and the potential risk factors of LBP in pregnant women. METHODS: This cross-sectional study included 173 pregnant women in the third trimester. Non-inclusion criteria were severe mental disability and known previous history of musculoskeletal diseases. The participants were categorised into two groups: women with pregnancy-related LBP and pain-free women. Demographic, socio-professional, clinical and obstetrical data were compared between the two groups using the appropriate statistical tests. RESULTS: The mean age was 32.2 ± 5.4 years [17-45]. Among them, 108 (62.4%) reported one or more episodes of LBP during at least 7 days, mostly in the third semester (n = 71). The presence of LBP was significantly associated with history of LBP in previous pregnancies and jobs requiring prolonged standing. Active jobs and the presence of gestational complications were significantly more common in pain-free women. In the multivariate analysis, LBP was independently predicted by the history of LBP in previous pregnancies and the absence of gestational complications. CONCLUSIONS: The association of LBP with gestational complications as a protective factor has not yet been reported in previous studies. These complications are a common cause of hospitalisation, which represents a period of relative rest during pregnancy. Our results revealed that history of LBP in previous pregnancies, sedentary lifestyle prior to pregnancy and prolonged standing are the main risk factors of LBP. In contrast, rest and avoidance of physical overstrain during pregnancy may be protective factors.

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.

Factors associated with poor prognosis of hip arthritis in juvenile idiopathic arthritis: Data from the JIR cohort.

OBJECTIVES: Hip involvement remains a predictor of severe juvenile idiopathic arthritis (JIA) course and carries a high risk of disability. This study aims to determine the factors of poor prognosis of hip involvement in patients with JIA and to assess the treatment response. METHODS: This is a multicenter observational cohort study. Patients were selected from the JIR Cohort database. Hip involvement was defined as clinically suspected and confirmed by an imaging tool. Follow-up data were collected during 5 years. RESULTS: Among the 2223 patients with JIA, 341(15%) patients had hip arthritis. Male gender, enthesitis-related arthritis, and North African origin were factors associated with hip arthritis. Hip inflammation was associated with disease activity parameters during the first year, particularly Physician Global Assessment, joint count, and inflammatory marks. Structural hip progression was associated with early onset of the disease, a longer time to diagnosis, geographic origin, and JIA subtypes. Anti-TNF therapy was found to be the only treatment able to effectively reduce structural damage progression. CONCLUSION: The early onset diagnostic delay, origin, and systemic subtype of JIA predict a poor prognosis of hip arthritis in children with JIA. The use of anti-TNF was associated with a better structural prognosis.

Impact of FCGR2A R131H, FCGR3A F158V and FCGR3B NA1/NA2 polymorphisms on response to Fc-containing TNF inhibitors in Tunisian rheumatoid arthritis patients.

OBJECTIVES: Single nucleotid polymorphisms (SNPs) of Fc-gamma receptors (FcgRs), by inducing a variation of their affinity to the Fc-region of immunoglobulins, might influence the efficacy of Fc-containing biologics prescribed in rheumatoid arthritis (RA). Our aim was to investigate associations of FCGR2A, FCGR3A and FCGR3B SNPs with TNF-inhibitors (TNFi)' response in Tunisian RA patients. METHODS: A cross-sectional, observational and analytic multicentric cohort study was conducted in a group of 47 Tunisian RA patients treated with (etanercept [ETA], adalimumab [ADL] and infliximab [IFX]). Treatment outcome was evaluated after 6 months. R131H-FCGR2A, F158V-FCGR3A and NA1/NA2-FCGR3B SNPs were genotyped. RESULTS: The analytic study including all types of TNFi showed that FCGR3A-F/F low-affinity receptor was associated with a greater decrease of DAS28, while FCGR3B-NA1/NA1 high-affinity receptor was associated with a lower decrease of DAS28 in ADL group. Furthermore, both of high affinity receptors FCGR3B-NA1/NA1 and FCGR3A-V/V were more prevalent in non-responders to ADL, according to EULAR criteria. CONCLUSIONS: Identifying reliable biomarkers of response to biologics in RA is necessary to improve responsiveness, preserve joints' functions and structure, and reduce treatment's cost. Our study showed that FCGR3A and FCGR3B polymorphisms might have an impact on TNFis' response in RA Tunisian patients since bad response was more frequent in homozygous carriers of high affinity alleles FCGR3A-V and FCGR3B-NA1.

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.