Joint Involvement Research Articles

Impact of gender on baseline presentation and outcome in adult IgA vasculitis.

Adult IgA vasculitis (IgAV) is more common in males, but the potential impact of gender remains unclear. We aimed to describe the impact of gender on presentation and outcome in adult IgAV. We retrospectively analysed data from a multicentre retrospective cohort of 260 patients (IGAVAS). Comparisons were made according to gender status. Data from 259 patients (95 females and 164 males) were analysed. Compared with females, baseline presentation in males was similar for cutaneous involvement (100% vs 100%, p= 1.0), joint involvement (60% vs 63%, p= 0.7), gastrointestinal involvement (57% vs 45%, p= 0.093) and glomerulonephritis (73% vs 64%, p= 0.16). Glomerulonephritis was more severe at baseline in males than in females, with a lower median estimated glomerular filtration rate (eGFR) (90 [IQR 59-105] vs 97 ml/min/1.73m2 [76-116], p= 0.015) and increased median proteinuria (0.84 vs 0.58 g/day, p= 0.01). There were no differences in histological findings in patients who had a kidney biopsy. Methylprednisolone was more frequently used in males (40% vs22%, p= 0.015), as were immunosuppressants, especially cyclophosphamide 24% vs 6%, p= 0.0025) and azathioprine (10% vs 2%, p= 0.038). Analysis of treatment response showed that males had more frequent refractory disease (30% vs 13%, p= 0.004). Long-term outcomes (mortality and progression to chronic kidney failure) did not differ. Kidney involvement in IgAV appears to more severe in males, which is supported by more intensive treatment contrasting with a lower response rate. This study raises the question of gender as a new prognostic factor in adult IgAV.

Radiological progression and predictive factors in psoriatic arthritis: insights from a decade-long retrospective cohort study.

Radiological alterations in psoriatic arthritis (PsA) are an established phenomenon frequently observed throughout the disease course. Our goal was to investigate the changes in the bone structure of PsA patients by conventional radiography. This study designed as a retrospective cohort study and cross-sectional evaluation for disease activity. The disease activity and the severity of skin and nail involvement were assessed. The Simplified Psoriatic Arthritis Radiographic Score (SPARS) was used to investigate the radiological progression. Logistic regression analysis was used to determine the predictors of radiological changes. Joint space narrowing and bone proliferation in hands (p = 0.001 and p = 0.001, respectively) and joint space narrowing in feet (p = 0.047) were more common at the final evaluation than at the baseline assessment. Total scores of joint space narrowing and bone proliferation in hands and feet were higher at the last visit than at the initial assessment (p < 0.001). Male gender (p = 0.030, OR 4.32 (95%CI 1.15-16.15)], older age (for joint space narrowing [p = 0.026 OR 1.08 (95%CI 1.01-1.56)] and for proliferation [p = 0.025 OR 1.08 (95%CI 1.01-1.44)]), high Disease Activity index for Psoriatic Arthritis (DAPSA) scores at baseline [p = 0.032 OR 6.21 (95%CI 1.17-32.92)], and symmetrical polyarticular involvement at baseline [p = 0.025 OR 5.3 (95% CI 1.23-22.4)] were found as predictors of structural changes. By the end of the decade, joint space narrowing and proliferation were observed to be more common than erosion. Male gender, older age, higher initial DAPSA scores, and initial polyarticular involvement were identified as predictors of radiological damage. Key Points • The radiological changes of Psoriatic arthritis are a well-known entity. However, studies investigating the progression of joint involvement over time are scarce. • This study reveals that joint space narrowing and proliferation are the most prominent radiological alterations in Psoriatic Arthritis patients at the end of the decade. • Male gender, older age, higher baseline DAPSA scores, and initial polyarticular involvement are predictive factors influencing the progression of bone destruction in Psoriatic Arthritis patients.

Clinical analysis of 10 cases of multi-center tumor necrosis factor receptor-associated periodic syndrome

Objective: To summarize the clinical characteristics of tumour necrosis factor receptor-associated periodic syndrome (TRAPS) in children. Methods: The clinical manifestations, laboratory tests, genetic testing and follow-up of 10 children with TRAPS from May 2011 to May 2021 in 6 hospitals in China were retrospectively analyzed. Results: Among the 10 patients with TRAPS, including 8 boys and 2 girls. The age of onset was 2 (1, 5) years, the age of diagnosis was (8±4) years, and the time from onset to diagnosis was 3 (1, 7) years. A total of 7 types of TNFRSF1A gene variants were detected, including 5 paternal variations, 1 maternal variation and 4 de novo variations. Six children had a family history of related diseases. Clinical manifestations included recurrent fever in 10 cases, rash in 4 cases, abdominal pain in 6 cases, joint involvement in 6 cases, periorbital edema in 1 case, and myalgia in 4 cases. Two patients had hematological system involvement. The erythrocyte sedimentation rate and C-reactive protein were significantly increased in 10 cases. All patients were negative for autoantibodies. In the course of treatment, 5 cases were treated with glucocorticoids, 7 cases with immunosuppressants, and 7 cases with biological agents. Conclusions: TRAPS is clinically characterized by recurrent fever accompanied by joint, gastrointestinal, skin, and muscle involvement. Inflammatory markers are elevated, and autoantibodies are mostly negative. Treatment mainly involves glucocorticoids, immunosuppressants, and biological agents.

The Effect of Two Years of Secukinumab Treatment on Bone Metabolism in Patients with Radiographic Axial Spondyloarthritis: Results from Daily Clinical Practice.

Our objective was to explore bone-related outcome and bone turnover markers (BTM) during 2 years of secukinumab treatment in patients with radiographic axial spondyloarthritis (r-axSpA) in daily clinical practice. Included were consecutive r-axSpA outpatients from the Groningen Leeuwarden axSpA (GLAS) cohort treated with secukinumab for 2 years. At baseline and 2 years, spinal radiographic damage was assessed using the modified Stoke Ankylosing Spondylitis Spinal Score (mSASSS; 0-72), cervical facet joint involvement according the "de Vlam" scoring method (0-15) and radiographic vertebral fractures (VF) using the "Genant" method (grade 0-3). At all visits, BTM reflecting collagen resorption (serum type I collagen C-telopeptide; sCTX), collagen formation (procollagen type 1 N-terminal peptide; PINP) and bone mineralization (bone-specific alkaline phosphatase; BALP) were measured and expressed in Z-scores to correct for the normal influence of age and gender. 17 r-axSpA patients were included; 53% male, mean age was 47±15 years, mean Ankylosing Spondylitis Disease Activity Score (ASDAS) 3.9±1.2, and 53% was biological naïve. The median 2-year progression rates were 1.1 for mSASSS and 0.5 for facet joints, which was less than the smallest detectable change. One traumatic VF (grade 3) occurred. Serum levels of sCTX and PINP remained stable during secukinumab treatment and BALP decreased significantly after 2 years, with median 0-2 year change in Z-scores of +0.1, -0.4, and -1.2, respectively. This explorative study of r-axSpA patients treated with secukinumab in daily clinical practice showed low radiographic spinal progression during 2 years of follow-up. Collagen resorption and formation markers remained stable, whereas mineralization marker BALP decreased significantly after 2 years. Our results are in line with the results of in vitro studies demonstrating that inhibition of IL17-A resulted in suppression of osteogenic differentiation with significant decrease in mineralization.

The relation between synovitis of individual finger joints and grip force over the first 5 years in early rheumatoid arthritis — a cohort study

Objective The objective of this study was to investigate the relation between swelling and tenderness of individual finger joints and grip force in patients with early rheumatoid arthritis (RA).Methods In an inception cohort of patients with early RA (symptom duration < 12 months), all patients were examined by the same rheumatologist, and grip force was measured using the Grippit instrument at inclusion, 1 and 5 years. The average grip force values of each hand were evaluated and expressed as % of expected values, based on age- and sex-specific reference values. Linear regression analyses were used to assess the cross-sectional relation between the involvement of individual finger joints and grip force. In generalized estimating equations, the impact of time-varying synovitis/tenderness on grip force over time was estimated. Analyses were adjusted for wrist involvement, erythrocyte sedimentation rate, and patient-reported pain.ResultsIn 215 patients with early RA, grip force was 39% of expected at diagnosis, and increased to 56% after 5 years. Synovitis of the first metacarpophalangeal (MCP) joint (60% and 69% at baseline in the right and left hand) was associated with reduced grip force at inclusion (adjusted ß − 9.2 percentage unit of expected grip force; 95% CI − 13.6 to − 4.8 for both hands combined) and at all follow-up evaluations. Synovitis of MCP I and MCP IV (12% at baseline) was significantly associated with reduced grip force over time in both hands. Proximal interphalangeal (PIP) joint swelling, and tenderness of MCP or PIP joints, had less impact on grip force.ConclusionMCP I synovitis is the major contributor to reduced grip force in patients with early RA. This underlines the importance of the involvement of the thumb for impaired hand function in RA. MCP IV synovitis, but not PIP involvement or finger joint tenderness, also has a substantial impact on grip force.

EROSIVE TEMPOROMANDIBULAR JOINT DISORDER IN A PATIENT WITH ULCERATIVE COLITIS: RAREMANIFESTATION OF ARTHROPATHIES, CASE REPORT

Temporomandibular Joint (TMJ) involvement in patients with ulcerative colitis (UC) is a rare manifestation. To the best of our knowledge there are only two cases in the literature reported the presence of TMJ involvement in a patient with UC. We report a case of 23-year-old female with a two-year history of UC she was complaining of unilateral temporomandibular jaw pain, the pain was severe enough to affect her daily activities, she was not able to speak and there was a limitation of mouth opening. The patient was only able to open her mouth for 2.5 cm. C-reactive protein and erythrocyte sedimentation rate were high, rheumatoid factor was negative. The computed tomography (CT) of the mandible and teeth reveals flattening with erosion of the condylar process of the left mandibular joint without the presence of joint effusion or soft tissue swelling. Our patient was managed by biological treatment Ustekinumab for 6 months, as a result the patient improved and she was able to open her mouth up to 4 cm. Clinicians should be aware of the importance of biological treatment in these circumstances, TMJ arthritis in UC patients is considered a rare and unusual picture of extraintesinal IBD manfistations.

Osteonecrosis as a rare musculoskeletal complication in Behcet’s disease- the largest case series with literature review

BackgroundBehcet disease (BD) as a variable vessel vasculitis is mainly characterized by ocular involvement, genital and oral aphthosis, and erythema nodosum. However, major organ involvements including gastrointestinal involvement, nervous system, and vascular involvement are among the severe complications. Osteonecrosis is a rare complication of patients with BD. We aim to report the largest series of BD patients suffering from osteonecrosis.MethodsWe have retrospectively reviewed all patients in Iran Behcet’s Disease Registry and reported those with osteonecrosis. Patients’ medication and clinical features, symptoms, and details of osteonecrosis will also be presented. Furthermore, previously reported cases will also be reviewed.ResultsSeven thousand eight hundred thirty-one patients were diagnosed with BD and registered. 18 patients developed ON with an incidence of 0.22%. The most common involvement during the disease progression was oral aphthosis which appeared in 100% of patients followed by ocular involvement in 85.7% and skin involvement in 71.4%. Vascular, ocular, and nervous system involvements are significantly higher in BD patients with osteonecrosis than the other BD patients. For the management of acute episode of uveitis, deep vein thrombosis, severe gastrointestinal involvement, arterial involvement, nervous system Involvement, and joint involvement high dose of glucocorticoids is indicated.ConclusionsON tends to appear as a multifocal involvement in BD patients, hence, after diagnosis of ON in one joint other possible sites of ON should be investigated.

Vitamin D Deficiency and Avascular Necrosis in Patients with Sickle Cell Disease: A Retrospective Cohort Study

Introduction Sickle cell disease (SCD) is an inherited hemoglobinopathy with complex systemic involvement. Avascular necrosis (AVN) is a devastating complication among patients with SCD. Vitamin D deficiency (VDD) is commonly found in SCD patients, but there is limited research on the potential role of VDD as a risk factor for AVN in SCD patients. This study aimed to assess the association between VDD and AVN and determine the patterns, risk factors, diagnosis, and treatment of AVN in SCD patients. Methods We conducted a retrospective cohort study of consecutive SCD patients diagnosed with VDD from January 2020 to December 2022 at Prince Sultan Medical Military City (PSMMC) in Riyadh, Saudi Arabia. Patients meeting the following criteria were included: (1) adults and children with confirmed SCD through hemoglobin electrophoresis and (2) tested for VDD. Patients who underwent bone marrow transplant were excluded. The main outcome measured was the occurrence of AVN. VDD was diagnosed in patients with 25-hydroxyvitamin D levels below 30 nmol/L. Magnetic resonance imaging was used to stage and confirm AVN diagnosis. Associations were assessed using the chi-square test, and logistic regression was performed to control for confounding. Results The study included 711 eligible SCD patients, with 271 (38%) being children. VDD affected 301 out of 711 patients (42.3%). Among these, 212 (70.4%) were adults (&amp;gt;18 years old). VDD was observed in 32.2% of children and 48.1% of adults. Of the total cohort, 127 (17.9%) patients experienced AVN, including 18 children. Among patients with VDD, 89 developed AVN, while VDD affected 212 patients without AVN. The time between VDD diagnosis and AVN presentation ranged from 3 to 12 months. Male patients accounted for 52% of AVN cases. The average age at AVN diagnosis was 30.9 ± 11 years. The most common clinical presentation of AVN was chronic joint pain in over 95% of patients. Other presentations included limping in 3.9% of patients and limited joint movement in one patient. Affected joints included the hip (86%), shoulder (16.5%), knee (4.7%), and sacroiliac joint (0.7%). Bilateral joint involvement was present in 50.4% of AVN patients. The majority of patients (76.4%) were diagnosed with advanced stage AVN (III/IV). Treatment of patients with AVN included joint replacement in 44 (34.6%) patients, while the remaining 83 patients managed with conservative therapy such as pain control and physiotherapy. Patients with a history of VDD were significantly associated with a higher risk of AVN (p &amp;lt; 0.001), as seen in Table 1. Multivariate logistic regression showed SCD patients with VDD had 9.79 times the odds of AVN compared to SCD patients without VDD (OR 9.79; 95% CI: 5.25-18.26). Other statistically significant risk factors for AVN included frequent vaso-occlusive crises (three or more episodes per year), older age, history of acute chest syndrome, and low hemoglobin F levels. Conclusion AVN is a significant cause of morbidity in our SCD cohort, with most patients having advanced AVN disease. VDD is an independent risk factor for AVN in patients with SCD. Well-designed randomized trials are needed to determine the effect of vitamin D supplementation in reducing AVN in children and adults with SCD.

Using of Emicizumab in Pediatric Hemophilia a Patients: KSA Single Center Experience

Introduction Emicizumab, a bispecific monoclonal antibody plays a crucial role in restoring adequate hemostasis by acting as a bridge between factors IX and X, effectively simulating the activity of factor VIII. Emicizumab is approved in several countries for bleeding episode prophylaxis in patients with HA with or without FVIII inhibitors regardless of severity. This study aimed to report results from an assessment of tolerability, efficacy, and safety of Emicizumab prophylaxis in pediatric patients with HA at a single center in Saudi Arabia. Methods This study was a retrospective study conducted between April 2019 and July 2023 at a Center for Blood Disorders for Pediatric Patients, Jeddah, Saudi Arabia. Eligible study participants were individuals &amp;lt; 16 years of age, weighing over 3 kg, presenting severe or moderate congenital hemophilia A with or without FVIII inhibitors and those who were treated with Emicizumab for at least 3months at our center. We decided to administer Emicizumab to patients with inhibitors, poor venous access, especially those of younger ages,and those with repeated breakthrough bleeds despite regular prophylaxis with FVIII concentrates. Parental drug preference was considered and discussed in all cases. The initial 2 loading injections of Emicizumab (3 mg/kg once weekly for four weeks) were administered at the center, and the patients were observed for 1 h for signs of any acute adverse events. Following discharge, the caregivers of the patients were educated about proper medication administration techniques and instructed to contact our center in case of any adverse events. Annual bleeding rate (ABR) were calculated at baseline pre and post-Emicizumab administration.Weight-based Emicizumab dosage adjustments were made at the end of each patient visit. Results 23 children with HA who started Emicizumab was initially included. A total of 6 patients were excluded as they didn't met the eligible criteria.10 patients with severe hemophilia (59%) and 7 patients with moderate hemophilia (41%). Patient were followed for median duration range of 217 weeks (15-232 weeks). All study patients were males and median (range) age at enrollment was 8.0 (11 months to 16 years).All of them were treated previously with factor either on demand or as prophylaxis. Two patients presented with inhibitors, while the rest were the non-inhibitor type. Among these patients, 2 received a maintenance dose of 3 mg/kg of Emicizumab every 2 weeks, while 16 patients received a monthly dose of 6 mg/kg. Selection of regimen was based guardian preference and also approximate the dose to available drug concentration to avoid drug wastage. Prior to enrollment, 9 patients had target joint involvement; among theses patients, 6 patients demonstrated improvement after Emicizumab initiation (66% reduction), while 3 patients still had persistent target joint involvement. The initial baseline ABRs median (Range) was 48 (Range 5-120). Compared to the ABRs after initiation of Emicizumab which showed median of 2 (Range 0-18), the ABR mean value decreased from 38.5 at baseline to 4.6 after Emicizumabtreatment. All bleeding events were traumatic and resolved with less than 48 h. one or two doses of Factor was/were required in four cases while bleeding was self-limited in others. 11 (64.7%) patients experienced zero bleeding. We observed unusual side effect of asymptomatic persistent neutropenia was observed in 10 (58.8%) out of 17 patients. The absolute neutrophil count (ANC) ranged from 0.6 to 1.45 cells/µL in these cases. There was no other adverse event was observed. Two patients underwent a tooth extraction without post-surgery bleeding, and neither required factor VIII treatment. Additionally, one patient underwent facial laceration repair and received a prophylaxis dose of factor VIII, leading to a successful outcome without any bleeding during and after the procedure. Conclusion Our study demonstrates the effectiveness of Emicizumab prophylaxis for treating patients with moderate to severe HA.The participants reported high adherence to the treatment, improved satisfaction rates and the concept of subcutaneous injections was well accepted. Although neutropenia is considered a unusual side effect of this treatment, the treatment progressed smoothly and favorable safety profile was observed.

Prevalence of temporomandibular disorders in juvenile idiopathic arthritis evaluated with diagnostic criteria for temporomandibular disorders: A systematic review with meta-analysis.

Juvenile idiopathic arthritis (JIA) is a very common systemic inflammatory rheumatic disorder affecting the musculoskeletal system in children below 16 years of age. Joint inflammation and tissue destruction is the prime characteristic of the disease. Along with the systemic involvement in the long joints, several studies are mentioning the increased association of temporomandibular disorders (TMDs) in JIA. This current systematic review intends to find the prevalence rate of TMD in JIA-affected individuals as compared to healthy controls. We have searched in PubMed, Scopus and Ovid SP for articles published between the timeframe 1 January 1990 and 1 June 2023. All the searched articles were subjected to the Population, Exposure, Comparison, and Outcome model (PECO) based on which inclusion or exclusion is carried out. Participants (P) are children below 18 years of age, Exposure (E) is children or adolescents with a diagnosis of JIA, Comparator is age and gender-matched healthy controls who has no JIA or any systemic disorder, Outcome (O) is the prevalence of TMD. Only the studies that evaluated TMD using diagnostic criteria for evaluation of TMD (DC/TMD) were included in the analysis. We have set the exclusion to the following reasons- diagnostic sensitivity studies, case reports, and systematic reviews. The software Review Manager Version 5.4 (Cochrane Collaboration) was used to perform the pooled analysis. We measured the risk ratio (RR) between the two groups (JIA and no JIA) for the outcome TMD. The pooled total included subjects were 366 in this review with an established diagnosis of JIA as evaluated by DC/TMD. The overall effect of the pooled data suggests that there is a significant difference in the TMD prevalence in the JIA group when compared to the control, results suggest that TMD is more prevalent in the JIA group RR 3.86; 95% CI [2.59, 5.76]. Overall, based on the data we can suggest a positive relationship between JIA and TMD, hence presence of JIA can be a risk factor for the development of TMD. The sensitivity of DC/TMD is low when compared to magnetic resonance imaging.

1281. Ongoing Transmission of Clonal Strains of Invasive Nontypeable Haemophilus influenzae Disproportionately Affecting Persons with HIV in Metropolitan Atlanta, 2019—2022

Abstract Background Nontypeable Haemophilus influenzae (NTHi) is a genetically diverse pathogen that typically causes mucosal infections or invasive NTHi (iNTHi) disease in persons at the extremes of age. In 2017-2018, we reported a significant increase in incident iNTHi in Atlanta among virally-suppressed persons with HIV (PWH) and identified two clonal strains associated with septic arthritis/polyarthritis among Black men who have sex with men. Methods Surveillance data from the CDC-funded Georgia Emerging Infections Program was used to assess interim iNTHi epidemiology in adults ≥ 18 years old in metropolitan Atlanta, including the potential for continued transmission of clonal strains in adults 18-55 years old. Overall incidence of iNTHi and by HIV status from 2019-2022 was determined and compared with prior years; and demographic and clinical data were collected. Whole genome sequencing assessed genetic relatedness of isolates, which were categorized as belonging to cluster 1 or 2 (C1, C2) or non-clustered (NC). Results From 2019-2022 in Atlanta, there were 157 iNTHi cases in adults ≥ 18 years old (median age 56 [Q1-Q3 36-72] years, 59% male, 44% Black), including 29 in PWH. The iNTHi incidence among PWH in 2019-2022 was lower than in 2017-2018 (18.8 vs 41.7 cases per 100,000, respectively, p&amp;lt; 0.01), and higher than in 2008-2016 (9.6 per 100,000; p&amp;lt; 0.01) (Figure). Among iNTHi cases 18-55 years old (2019-2022), 94/95 had isolates available for cluster analysis: 7/94 (7%) and 18/94 (19%) were identified as C1 and C2, respectively. Characteristics of cases differed by clonal status (Table 1): among C1, C2, and NC cases, 6/7 (86%), 7/18 (39%), and 15/69 (22%) involved PWH, respectively; and septic arthritis was present in 1/7 (14%), 8/18 (44%), and zero, respectively. Table 2 provides detailed data on PWH with clonal strains. Conclusion Ongoing transmission of two clonal NTHi strains that are associated with a relatively high prevalence of joint involvement in invasive disease and disproportionately affect Black men and PWH was noted in Atlanta. The C2 clonal strain became more prevalent in 2022. Additional studies are needed to better understand modes of transmission, geographic distribution of clones, and to explore the unusual clinical manifestations to optimize prevention and treatment measures. Disclosures Lauren F. Collins, MD, MSc, Curio Science: Honoraria

Are there causal mucosal drivers in the preclinical development of rheumatoid arthritis?

BackgroundThe causal pathways which drive the development of seropositive rheumatoid arthritis (RA) are incompletely understood, especially in the period of time prior to the first development of signs and symptoms of joint involvement. That asymptomatic period, designated herein as pre-RA, is characterized by the presence of RA-related autoantibodies for many years and is the subject of an increasing number of studies as well as a focus of efforts to prevent the onset of clinically apparent arthritis. ObjectivesTo review the potential causal pathways in pre-RA by examining results of studies which evaluate the systemic peripheral blood and mucosal alterations that have been identified in individuals who are genetically at-risk, and/or who elaborate RA-related autoantibodies, and are defined as in a pre-RA period. MethodsPublished studies by the author and his colleagues, as well as publications by other groups, which describe the presence of biomarkers at mucosal sites and in the blood were reviewed. From these studies, a hypothesis related to the presence of pre-RA causal drivers was constructed. ResultsThe author and his colleagues, as well as other groups, have shown that there are multiple mucosal sites, primarily gut, lung and oral/peridontial, which appear in subsets of individuals in the pre-RA to exhibit inflammation and/or the presence of local production of IgA and IgG RA-related autoantibodies, including anti-citrullinated protein antibodies (ACPA). These findings are reviewed herein. There remain a large number of unanswered questions, though, related to the immune mechanisms that are operative at each site, as well as how these local findings evolve to causal systemic autoimmunity and eventually inflammatory arthritis. Author's ConclusionsComprehensive natural history studies are required to understand how multiple mucosal sites which appear to be involved in pre-RA are causally involved in the development of arthritis. Questions remain as to whether there are independent, serially involved, or inter-related causal immune pathways originating from these sites. In addition, the microbiota which may be involved in local immune inflammation and autoantibody production should be identified and characterized.

Interaction effects of significant risk factors on low bone mineral density in ankylosing spondylitis.

To analyze individually and interactively critical risk factors, which are closely related to low bone mineral density (BMD) in patient with ankylosing spondylitis (AS). A total of 249 AS patients who visited China-Japan Friendship Hospital were included in this training set. Patients with questionnaire data, blood samples, X-rays, and BMD were collected. Logistic regression analysis was employed to identify key risk factors for low BMD in different sites, and predictive accuracy was improved by incorporating the selected significant risk factors into the baseline model, which was then validated using a validation set. The interaction between risk factors was analyzed, and predictive nomograms for low BMD in different sites were established. There were 113 patients with normal BMD, and 136 patients with low BMD. AS patients with hip involvement are more likely to have low BMD in the total hip, whereas those without hip involvement are more prone to low BMD in the lumbar spine. Chest expansion, mSASSS, radiographic average grade of the sacroiliac joint, and hip involvement were significantly associated with low BMD of the femoral neck and total hip. Syndesmophytes, hip involvement and higher radiographic average grade of the sacroiliac joint increases the risk of low BMD of the femoral neck and total hip in an additive manner. Finally, a prediction model was constructed to predict the risk of low BMD in total hip and femoral neck. This study identified hip involvement was strongly associated with low BMD of the total hip in AS patients. Furthermore, the risk of low BMD of the femoral neck and total hip was found to increase in an additive manner with the presence of syndesmophytes, hip involvement, and severe sacroiliitis. This finding may help rheumatologists to identify AS patients who are at a high risk of developing low BMD and prompt early intervention to prevent fractures.

The effect of polypharmacy on rheumatoid and psoriatic arthritis treatment: retrospective study.

Rheumatoid arthritis (RA) and psoriatic arthritis (PsA) are chronic, progressive inflammatory diseases that can be accompanied by other diseases. In recent years, with the increase in the lifespan of individuals, the concept of polypharmacy has become more prominent. We aimed to show the prevalence of polypharmacy and the effects of polypharmacy on disease activity in RA and PsA. This study included PsA patients who had peripheral joint involvement and, RA patients. Since PsA has a heterogeneous clinical picture, only patients with peripheral joint involvement were included in the study and patients with inflammatory low back pain or radiological sacroiliitis or spondylitis, dactylitis or enthesitis were not included in the study due to homogeneity concerns. The numbers of medications used by the patients at the onset of their treatment and at sixth months into their treatment were recorded. Polypharmacy was accepted as the simultaneous use of at least five medications by the person. The Disease Activity Score 28 joints C-Reactive Protein (DAS-28 CRP) was used to assess disease activity for both disease. The modified Charlson Comorbidity Index (CCI) scores of the patients were calculated based on their chronic diseases. The sample of the study included 232 RA and 73 PsA patients. Polypharmacy was present at the treatment onset in 115 (49.6%) of the RA patients and 28 (38.4%) of the PsA patients. At the sixth month of treatment, polypharmacy was present in the sixth month of the treatment in 217 (93.5%) RA and 61 (83.6%) PsA patients. The mean ages of the RA and PsA patients who were receiving polypharmacy treatment at the beginning were significantly older than the mean ages of those who were not receiving polypharmacy treatment. In both the RA and PSA groups, the patients with polypharmacy at the beginning had statistically significantly higher DAS-28 CRP scores at six months of treatment than those without polypharmacy at the beginning (p<0.001). Polypharmacy was present both at the time of diagnosis and in the treatment process in the RA and PsA patients, and the presence of polypharmacy at the beginning of the treatment was among the factors that affected the treatment of these patients by significantly affecting their 6th-month DAS-28 CRP values.

Whole-body MRI for the investigation of joint involvement in inflammatory arthritis

ObjectivesThis study aimed to develop a novel whole-body MRI protocol capable of assessing inflammatory arthritis at an early stage in multiple joints in one examination.Materials and methodsForty-six patients with inflammatory joint symptoms and 9 healthy volunteers underwent whole-body MR imaging on a 3.0 T MRI scanner in this prospective study. Image quality and pathology in each joint, bursae, entheses and tendons were scored by two of three radiologists and compared to clinical joint scores. Participants were divided into three groups based on diagnosis at 1-year follow-up (healthy volunteers, rheumatoid arthritis and all other types of arthritis). Radiology scores were compared between the three groups using a Kruskal-Wallis test. The clinical utility of radiology scoring was compared to clinical scoring using ROC analysis.ResultsA protocol capable of whole-body MR imaging of the joints with an image acquisition time under 20 min was developed with excellent image quality. Synovitis scores were significantly higher in patients who were diagnosed with rheumatoid arthritis at 12 months (p < 0.05). Radiology scoring of bursitis showed statistically significant differences between each of the three groups—healthy control, rheumatoid arthritis and non-rheumatoid arthritis (p < 0.05). There was no statistically significant difference in ROC analysis between MRI and clinical scores.ConclusionThis study has developed a whole-body MRI joint imaging protocol that is clinically feasible and shows good differentiation of joint pathology between healthy controls, patients with rheumatoid arthritis and patients with other forms of arthritis.

Oral health-related quality of life, impaired physical health and orofacial pain in children and adolescents with juvenile idiopathic arthritis – a prospective multicenter cohort study

BackgroundKnowledge on oral health-related quality of life (OHRQoL) in children and adolescents with juvenile idiopathic arthritis (JIA) is limited, and longitudinal studies are lacking. We aimed to describe OHRQoL in children and adolescents with JIA compared to controls, and to explore the validity and internal consistency of the Early Childhood Oral Health Impact Scale (ECOHIS) and the Child Oral Impact on Daily Performance (Child-OIDP). Furthermore, we wanted to investigate associations between OHRQoL and orofacial pain, physical health, disease activity, and temporomandibular joint (TMJ) involvement in JIA.MethodsThe Norwegian prospective, multicenter cohort study recruited participants with JIA between 4 and 16 years of age and corresponding controls from three pediatric university hospital departments and public dental health services. In the present study, we analyzed OHRQoL in all children < 12 years with the ECOHIS and adolescents ≥ 12 years with the Child-OIDP at the first visit and the two-year follow-up. Associations between OHRQoL and JIA characteristics, collected in clinical exam and questionnaires, were analyzed in logistic regressions.ResultsThe same OHRQoL questionnaire was completed both at first visit and two-year follow-up in 101 children < 12 years (47 JIA, 54 controls) and 213 adolescents ≥ 12 years (111 JIA, 102 controls). The frequency of OHRQoL impacts in children was similar at the first visit and the two-year follow-up (ECOHIS > 0: JIA group 81% and 85%, p = 0.791; control group 65% and 69%, p = 0.815), while adolescents with JIA reported fewer impacts at the two-year follow-up (Child OIDP > 0: JIA group 27% and 15%, p = 0.004; control group 21% and 14%, p = 0.230). The internal consistency of the OHRQoL instruments was overall acceptable and the criterion validity indicated that the instruments were valid at both visits. Orofacial pain was more frequent in children and adolescents with JIA than in controls. We found associations between OHRQoL impacts and orofacial pain, impaired physical health, disease activity, and TMJ involvement.ConclusionsChildren and adolescents with orofacial pain or impaired physical health were more likely to report impacts on daily life activities than those without. Pediatric rheumatologists and dentists should be aware of impaired OHRQoL in individuals with JIA with active disease or temporomandibular joint involvement.Trial registrationRegistered on clinicaltrials.gov (NCT03904459, 05/04/2019).

The assessment of atlantoaxial joint involvement in patients with rheumatoid arthritis, results from an observational “real-life” study

Atlantoaxial joint is a possible affected site during rheumatoid arthritis (RA) and, in this work, we evaluated its occurrence and associated characteristics in a “real-life” cohort. By a medical records review study of RA patients longitudinally followed-up, the occurrence of severe atlantoaxial joint involvement was estimated (incidence proportion and incidence rate per 1000 person-years at risk). Regression analyses were also exploited to evaluate possible associated factors. Based on these findings, a prospective recruitment was performed to build a descriptive cross-sectional study in evaluating a subclinical atlantoaxial joint involvement in patients with the same clinical characteristics. Retrospectively, 717 patients (female 56.6%, age 64.7 ± 12.3 years) were studied. The incidence proportion of severe atlantoaxial joint involvement was 2.1% [1.5–2.5], occurring in 15 out of 717 patients, and identified by both MRI and CT scan. Considering over 3091 person-years, an incidence rate of 5.2 × 1000 [2.9–8.3] person-years was estimated. Regression analyses suggested that male gender, a longer disease duration, ACPA positivity and extra-articular manifestations resulted to be significantly associated with a severe atlantoaxial joint involvement. Given these findings, 30 asymptomatic patients were selected according to these clinical characteristics and underwent MRI of cervical spine. To date, almost 50% of these asymptomatic patients showed a subclinical atlantoaxial joint involvement. The occurrence of the severe atlantoaxial joint involvement in RA patients was estimated in a “real-life” setting. Male gender, ACPA positivity, long disease duration, and extra-articular manifestations could be associated with the severe atlantoaxial joint involvement in RA. MRI could provide a useful clinical tool to early evaluate the atlantoaxial joint involvement in RA, also in asymptomatic patients.

Safety and efficacy of canakinumab treatment for undifferentiated autoinflammatory diseases: the data of a retrospective cohort two-centered study.

The blockade of interleukine-1 (anakinra and canakinumab) is a well-known highly effective tool for monogenic autoinflammatory diseases (AIDs), such as familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, hyperimmunoglobulinaemia D syndrome, and cryopyrin-associated periodic syndrome, but this treatment has not been assessed for patients with undifferentiated AIDs (uAIDs). Our study aimed to assess the safety and efficacy of canakinumab for patients with uAIDs. Information on 32 patients with uAIDs was retrospectively collected and analyzed. Next-generation sequencing and Federici criteria were used for the exclusion of the known monogenic AID. The median age of the first episode was 2.5 years (IQR: 1.3; 5.5), that of the disease diagnosis was 5.7 years (IQR: 2.5;12.7), and that of diagnostic delay was 1.1 years (IQR: 0.4; 6.1). Patients had variations in the following genes: IL10, NLRP12, STAT2, C8B, LPIN2, NLRC4, PSMB8, PRF1, CARD14, IFIH1, LYST, NFAT5, PLCG2, COPA, IL23R, STXBP2, IL36RN, JAK1, DDX58, LACC1, LRBA, TNFRSF11A, PTHR1, STAT4, TNFRSF1B, TNFAIP3, TREX1, and SLC7A7. The main clinical features were fever (100%), rash (91%; maculopapular predominantly), joint involvement (72%), splenomegaly (66%), hepatomegaly (59%), lymphadenopathy (50%), myalgia (28%), heart involvement (31%), intestinal involvement (19%); eye involvement (9%), pleuritis (16%), ascites (6%), deafness, hydrocephalia (3%), and failure to thrive (25%). Initial treatment before canakinumab consisted of non-biologic therapies: non-steroidal anti-inflammatory drugs (NSAID) (91%), corticosteroids (88%), methotrexate (38%), intravenous immunoglobulin (IVIG) (34%), cyclosporine A (25%), colchicine (6%) cyclophosphamide (6%), sulfasalazine (3%), mycophenolate mofetil (3%), hydroxychloroquine (3%), and biologic drugs: tocilizumab (62%), sarilumab, etanercept, adalimumab, rituximab, and infliximab (all 3%). Canakinumab induced complete remission in 27 patients (84%) and partial remission in one patient (3%). Two patients (6%) were primary non-responders, and two patients (6%) further developed secondary inefficacy. All patients with partial efficacy or inefficacy were switched to tocilizumab (n = 4) and sarilumab (n = 1). The total duration of canakinumab treatment was 3.6 (0.1; 8.7) years. During the study, there were no reported Serious Adverse Events (SAEs). The patients experienced non-frequent mild respiratory infections at a rate that is similar as before canakinumab is administered. Additionally, one patient developed leucopenia, but it was not necessary to stop canakinumab for this patient. The treatment of patients with uAIDs using canakinumab was safe and effective. Further randomized clinical trials are required to confirm the efficacy and safety.

Association between vaccination and the risk of immunoglobulin A vasculitis in children

To study the effect of vaccination on the short-term risk of immunoglobulin A vasculitis (IgAV) in children. A retrospective analysis was conducted on the general data and the vaccination history within one year prior to onset in children with IgAV hospitalized in the Children's Hospital Affiliated to Zhengzhou University from November 2021 to January 2023. Vaccine exposure rates in the risk period (3 months prior to IgAV onset) and the control period were compared by autocontrol-case crossover analysis, and the odds ratio and 95% confidence interval (95%CI) were calculated. A sensitivity analysis for the one-month and two-month risk periods was conducted. A total of 193 children with IgAV were included, with a median age of 7.0 years. Among the 193 children, 36 (18.7%) received at least one dose of the vaccine within 1 year prior to IgAV onset, and 14 (7.3%) received at least one dose of the vaccine during the 3-month risk period. Compared to the unvaccinated IgAV group, the vaccinated IgAV group had a significantly younger age of onset (P<0.05). There were no significant differences in the proportions of children with gastrointestinal involvement, renal involvement, and joint involvement between the two groups (P>0.05). The odds ratio for developing IgAV after receiving any type of vaccine within 3 months prior to IgAV onset was 2.08 (95%CI: 0.82-5.27, P>0.05). Further sensitivity analysis for the 1-month and 2-month risk periods demonstrated that the odds ratios for developing IgAV after receiving any type of vaccine were 2.74 (95%CI: 0.72-10.48, P>0.05) and 2.72 (95%CI: 0.95-7.77, P>0.05), respectively. Vaccination dose not increase the risk of IgAV, nor does it exacerbate clinical symptoms in children with IgAV.

Joint Involvement Can Predict Chikungunya in a Dengue Syndemic Setting in India

Dengue and chikungunya have been endemic in India but have the tendency to cause periodic epidemics, often together, wherein they are termed ‘syndemic’. Such a syndemic was observed in 2016 in India which resulted in a further scarcity of already resource-poor specific diagnostic infrastructure even in many urban conglomerates. A cross-sectional study was thus conducted, on 978 fever patients that consulted the ICMR-NIMR fever clinic, New Delhi, in September 2016, with an objective to identify symptom/s that could predict chikungunya with certainty. The overall aim was to rationally channelize the most clinically suitable patients for the required specific diagnosis of chikungunya. Based on their clinical profile, febrile patients attending NIMR’s clinic, appropriate laboratory tests and their association analyses were performed. Bivariate analysis on 34 clinical parameters revealed that joint pain, joint swelling, rashes, red spots, weakness, itching, loss of taste, red eyes, and bleeding gums were found to be statistically significantly associated predictors of chikungunya as compared to dengue. While, in multivariate analysis, only four symptoms (joint pain in elbows, joint swelling, itching and bleeding gums) were found in statistically significant association with chikungunya. Hence, based on the results, a clinician may preferably channelize febrile patients with one or more of these four symptoms for chikungunya-specific diagnosis and divert the rest for dengue lab diagnosis in a dengue–chikungunya syndemic setting.