Articular Manifestations Research Articles

A Practical Overview of the Articular Manifestations of Systemic Lupus Erythematosus.

Although it is widely known that joint involvement is the most frequent and prevalent manifestation of systemic lupus erythematosus (SLE), not having a validated organ-specific index for this domain in order to guide its treatment has been a major limitation. In addition, its clinical importance had been underestimated since it was not a vital risk domain; it was never the center of treatment, under the premise that in most cases its progression was slow and did not lead to significant functional disability.However, this concept has been changing due to the greater description of erosions both in ultrasonography and in osteoarticular magnetic resonance, so their identification can establish a more appropriate treatment time and thus avoid joint deformities, which in some cases can become irreversible.Recently, anifrolumab and belimumab have been able to significantly reduce the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and British Isles Lupus Assessment Group (BILAG) index scores, along with improvement in quality of life indices and a significant decrease in the required dose of glucocorticoids. Despite this, the ideal moment to consider biological therapy in this domain is not clear, since the clinical examination can sometimes be biased by the pain associated with fibromyalgia or the fatigue associated with SLE.For this reason, perhaps ultrasonography or magnetic resonance imaging, apart from differentiating the joint phenotype, can identify patients in time to define the onset of disease-modifying antirheumatic drugs and rationalize the use of glucocorticoids.The objective of this review is to characterize in detail the joint manifestations of SLE to offer the clinician a practical view of its diagnosis and treatment.

Vascular and Neurological Manifestations of Behcet’s Disease in the Eastern Province of Saudi Arabia

Abstract Context: Behcet’s disease (BD) is a form of vasculitis with multisystem and multiorgan involvement. Aims: To examine vascular and neurological manifestations in patients with BD in the Eastern Province of Saudi Arabia. Settings and Design: A single-center prospective study conducted at the rheumatology outpatient clinic Military of King Fahad Medical Complex in Dhahran between January 2016 and December 2022. Thirty-one patients, with minimum of three visits per year to a rheumatology outpatient clinic, were included in the study and followed up for 6 years. Patients and Methods: Data on patients’ demographic characteristics, age at BD onset, disease duration, multiorgan manifestation, and ongoing treatment were obtained using patient records and interviews during clinic visits. Statistical Analysis Used: The Mann–Whitney U-test was used to find the significance level among variables such as sex, vascular involvement, central nervous system involvement, and family history. Qualitative variables were compared using the Fisher’s exact and Z tests for proportion analysis. Results: Despite the high rate of consanguineous marriages in Saudi Arabia, only 25.8% of patients had a positive family history of BD. The most common manifestations of BD were oral ulcers, followed by genital ulcers and articular manifestations. Eleven and 12 patients with BD experienced vascular and central nervous system events, respectively; deep-venous thrombosis in the lower extremities was the most common vascular event. Conclusions: Vascular and neurological manifestations in BD were associated with significant morbidity and mortality, with an increased tendency of occurrence in men in the Eastern Province of Saudi Arabia.

Systemic Lupus Erythematosus: Female Versus Male

Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease, uncommon in men. The study of gender differences in SLE has been the subject of several publications. The main aim of our study is to identify gender differences in terms of clinical manifestations, immunological profile, activity score, and disease evolution in our Moroccan population. Results: We analyzed 279 patients (21 men versus 258 women), with a mean age at diagnosis of 37.54 +/- 23.39 years for women and 42.74 +/- 16.33 years for men. A comparison of clinical and immunological manifestations showed that male patients had a higher prevalence of renal involvement, lymphopenia, leukopenia, and general signs, but the difference was not significant. However, we did establish a significant correlation between male sex and cardiac involvement (P=0.013) as well as neuropsychiatric involvement (P=0.05). We also observed a significant association with anti-SSA antibodies (p=0.007). Dermatological and articular events were more frequent in women than in men, with a statistically significant association between female gender and photosensitivity (p=0.037). There was also a statistically significant correlation between women with RNP antibodies (p = 0.016) and anti-native DNA antibodies (p=0.047). The outcome was good in 67% of men (vs. 86.2% p=0.802), with response to treatment and control of the disease, and poor in 33% of men (vs. 13.9% %, p=0.019). Conclusions: Our study reveals the influence of gender on certain clinical manifestations of SLE. Dermatological and articular manifestations are more frequent in women, whereas men present a more severe disease, with a higher frequency of renal, cardiac, and neurological involvement.

E49 Behçet's disease associated with Warkany syndrome: a case report

Abstract Background Behçet's disease is a rare chronic, relapsing, multisystem vasculitis characterized by mucocutaneous lesions, articular, ocular, vascular, and central nervous system manifestations, of which pediatric forms may be syndromic. Warkany syndrome is a rare genetic anomaly defined by trisomy 8, characterized clinically by facial dysmorphia, mild intellectual disability, and skeletal anomalies. Aim We report the case of a 9-year-old boy diagnosed with Behçet's disease and whose examination revealed an associated Warkany syndrome. Observation the boy was from a non-consanguineous marriage, correctly vaccinated, with a history of congenital dislocation of the hip operated at the age of 3 years and obstruction of the lacrimal duct operated at the age of 6 years, who presented with chronic bilateral synechial pan uveitis, recurrent oral aphthosis (>3 times a year), multiple dysostosis with chronic diffuse arthralgias: hip, knees, shoulders. The clinical examination reveals a facial dysmorphia (elongated skull, high and prominent forehead, hypertelorism, sunken eyes, wide nose with anteverted nostrils, micrognathia with eversion of the lower lip, large ears with a thick rim), organomegaly (hepatosplenomegaly, large kidneys), a moderate intellectual deficit and deep palmar and plantar folds Auditory evoked potential finds bilateral deafness. Inflammatory workup was positive, HLAb51 typing was positive. MPS enzyme assay is normal. Genetic study: not done due to lack of means. Conclusion Behçet's disease in children can be associated with other genetic syndromes, it remains to be seen if it is a simple fortuitous association.

E66 Arthropathy as the initial presentation of HIV

Abstract Background Human immunodeficiency virus (HIV) has been associated with musculoskeletal disorders which has been estimated to occur in 11–72% of infected patients. Most of these reports however have been in adults with few published cases of HIV associated arthritis in children. The articular manifestations of HIV, especially overt arthritis, can occur at any stage of the disease but are more prevalent in advanced immunosuppression. With the increased access to early diagnosis and highly active antiretroviral therapy (HAART), such complications have increasingly become rare. However, HIV diagnosis may often be missed or delayed in slow progressors, and in such circumstances, these patients may manifest with arthritis as the initial manifestation of HIV. Therefore, there’s need for consideration of HIV associated arthritis in children presenting with arthropathy of undetermined cause. We hereby present a case of arthritis as the initial manifestation of HIV in a child with missed diagnosis of HIV. Methodology/case presentation A 12-year-old boy was admitted in the Kenyatta National Hospital (KNH) paediatric ward with a history of joint pain and swelling for three months. He had otherwise previously been well. He had no history of fever, trauma, preceding gastroenteritis or urine tract infection. He had no chorea, no erythematous rash and no cardiac complications of rheumatic fever. He was not wasted, and was able to walk with great difficulty. He had bilateral knee and right ankle swelling. The swollen joints were warm to touch with positive ballotment and fluid sweep test. Joint aspiration revealed straw coloured joint effusion. Investigations included a positive HIV rapid test with CD4% of 6, CRP 8.33 mg/l (n = <5), Elevated ESR (111 mm in first h) and negative ANA. Bilateral Knee -rays did not reveal any destructive or erosive changes. A diagnosis of HIV arthropathy was made and the patient was started on HAART, HCQ, prednisone and ibuprofen. He improved, with resolution of the joint swellings, was discharged home after 10 days and follow up in the rheumatology and comprehensive care clinics advised. Conclusion HIV arthropathy should be considered in patients manifesting with arthritis of unknown origin; and the patients evaluated for HIV as a strategy for identifying and including them in care. Highly active antiretroviral therapy, steroids, hydroxychloroquine and NSAIDS may be effective therapy for HIV arthropathy. Disclosure statement: The authors have no conflict of interest to declare.

E25 The pattern of juvenile psoriatic arthritis in a tertiary centre in Libya

Abstract Background Juvenile psoriatic arthritis (JPsA) is a relatively rare condition in childhood as it represents ∼5% of the whole Juvenile Idiopathic Arthritis (JIA). There are fewer reports describing the characteristics and outcome of patients with JPsA. Aim To describe the characteristic features and the treatment modalities of JPsA among Libyan children in pediatric rheumatology clinic at the Tripoli Children Hospital which is the only referral clinic covering the western, northern and southern part of Libya and to compare our results with other populations worldwide. Methods All the medical records of the children who were diagnosed to have JPsA according to ILAR classification criteria or Vancouver criteria (definite or probable) from January 2001 to January 2020 were included and retrospectively reviewed. Data were collected about demographic, clinical, and laboratory features and treatment Results The study included a total of 30 cases of JPsA that represents a 12% of total JIA cases over the study period, the mean age at presentation was 5.8 ± 5.3 years with male to female ratio of 1:1, with the mean duration of symptoms prior to diagnosis of JIA of 9 ± 10 months. The foremost common type of arthritis was peripheral polyarthritis (83% & 58.3%), with the Small joints commonly affected, 30% of patients had hip joint involvement. 13% of the patient had psoriasis, half of them before presentation of arthritis. Nail changes secondary to psoriasis was seen in 1 of the cohort, and the percentage of patients with dactylitis among the group was 30%. Family history of psoriasis found in 83% of the cohort, 26% found to be ANA positive. Chronic uveitis occurred in 10%. Patients of the total cohort, ANA positive, Unilateral anterior uveitis being the most common presentation. The following table summarizes the Medications used during the course of the disease At the time of the first visit 100%of total patients fulfilled the Vancouver criteria for juvenile PsA, While 5 of the12 patients didn’t fulfilled the ILAR criteria for JPsA. Conclusions Juvenile psoriatic arthritis constituted 12% of our patients with juvenile idiopathic arthritis. Juvenile psoriatic arthritis constitutes 8% to20% of JIA patients worldwide with equal gender distribution and peak age at presentation of 6–13 years. More than four peripheral joints involvement were the most frequent presentation. Half of the patients had skin involvement before the development of arthritis. Prevalence of chronic anterior uveitis was higher in our cohort than worldwide (10% vs 5% respectively) as an extra articular manifestation. All patients were treated with NSAIDs at presentation and/or during follow-up. Methotrexate was the most commonly used second-line agent Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

Obliterative bronchiolitis in rheumatoid arthritis (clinical case)

Obliterative (constrictive) bronchiolitis (OB) is a rare disease characterized by destruction of the bronchiolar epithelium and subsequent progressive airway obstruction. OB is most common in rheumatoid arthritis (RA) compared to other systemic rheumatic diseases. Clinical manifestations of OB are found mainly with a long duration of RA and the absence of adequate therapy for articular manifestations. We present a clinical observation, demonstrating the distal respiratory tract involvement in a patient with RA during the first year of the disease, which is observed in no more than 10–20% of cases. The nonspecificity of respiratory symptoms on the background of immunosuppressive therapy led to a diverse differential diagnostic spectrum of pulmonary pathology. For timely diagnosis and optimization of therapeutic approaches, clinical suspicion for respiratory lesions in patients with RA and interdisciplinary cooperation are necessary.

Lyme borreliosis in pediatric population: Clinical, diagnostic and therapeutic features

IntroductionLyme borreliosis (LB) in the paediatric population is an understudied entity with certain peculiarities. The objective of this study is to describe the characteristics of paediatric patients with LB, and their diagnostic and therapeutic processes. MethodsDescriptive and retrospective study in patients up to 14 years old with suspected or confirmed LB between 2015 and 2021. ResultsA total of 21 patients were studied: 18 with confirmed LB (50% women; median age 6.4 years old) and 3 false positive of the serology. Clinical features in the 18 patients with LB were: neurological (3, neck stiffness; 6, facial nerve palsy), dermatological (6, erythema migratory), articular (1), and non-specific manifestations (5). Serological diagnosis was confirmatory in 83.3% of cases. A total of 94.4% patients received antimicrobial treatment (median duration, 21 days). All recovered with resolution of symptoms. ConclusionsLB diagnosis is difficult in the paediatric population and presents clinical and therapeutic peculiarities, with favourable prognosis.

Successful treatment of JAK1-associated inflammatory disease

Gain-of-function variants of JAK1 drive a rare immune dysregulation syndrome associated with atopic dermatitis, allergy, and eosinophilia. This study sought to describe the clinical and immunological characteristics associated with a new gain-of-function variant of JAK1 and report the therapeutic efficacy of Janus kinase (JAK) inhibition. The investigators identified a family affected by JAK1-associated autoinflammatory disease and performed clinical assessment and immunological monitoring on 9 patients. JAK1 signaling was studied by flow and mass cytometry in patients' cells at basal state or after immune stimulation. Amolecular disease signature in the blood was studied at the transcriptomic level. Patients were treated with 1 of 2 JAK inhibitors: either baricitinib or upadacitinib. Clinical, cellular, and molecular response were evaluated over a 2-year period. Affected individuals displayed a syndromic disease with prominent allergy including atopic dermatitis, ichthyosis, arthralgia, chronic diarrhea, disseminated calcifying fibrous tumors, and elevated whole blood histamine levels. Avariant of JAK1 localized in the pseudokinase domain was identified in all 9 affected, tested patients. Hyper-phosphorylation of STAT3 was found in 5 of 6 patients tested. Treatment of patients' cells with baricitinib controlled most of the atypical hyper-phosphorylation of STAT3. Administration of baricitinib to patients led to rapid improvement of the disease in all adults and was associated with reduction of systemic inflammation. Patients with this new JAK1 gain-of-function pathogenic variant displayed very high levels of blood histamine and showed a variable combination of atopy with articular and gastrointestinal manifestations as well as calcifying fibrous tumors. The disease, which appears to be linked to STAT3 hyperactivation, was well controlled under treatment by JAK inhibitors in adult patients.

Epidemiology and Economic Burden of Chikungunya: A Systematic Literature Review.

Chikungunya (CHIK) is a re-emerging viral infection endemic in tropical and subtropical areas. While the typical clinical presentation is an acute febrile syndrome, long-term articular complications and even death can occur. This review characterizes the global epidemiological and economic burden of chikungunya. The search included studies published from 2007 to 2022 in MEDLINE, Embase, LILACS, and SciELO for a thorough evaluation of the literature. Rayyan software was used for data analysis, and data were summarized descriptively and reported following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Seventy-six publications were included. Chikungunya is widely distributed in the tropics, including Africa, Asia, South America, and Oceania/the Pacific Islands, and co-circulates with other simultaneous arboviruses such as DENV, ZIKV, and YFV. Chikungunya infection can lead to chronic articular manifestations with a significant impact on the quality of life in the long term. In addition, it generates absenteeism and economic and social losses and can cause fatal infections in vulnerable populations, mainly in high-risk patients with co-morbidities and at the extremes of age. Reported costs associated with CHIKV diseases are substantial and vary by region, age group, and public/private delivery of healthcare services. The chikungunya disease burden includes chronicity, severe infections, increased hospitalization risks, and associated mortality. The disease can impact the economy in several spheres, significantly affecting the health system and national economies. Understanding and measuring the full impact of this re-emerging disease is essential.

Clinical features, treatment and outcomes of Italian children with enthesitis-related arthritis and juvenile psoriatic arthritis: a cross-sectional cohort study.

Limited information is available on the clinical features, treatment modalities and outcomes of the juvenile idiopathic arthritis (JIA) categories of enthesitis-related arthritis (ERA) and juvenile psoriatic arthritis (JPsA). This study was aimed to describe the characteristics of Italian children with ERA and JPsA and to compare them with those of patients with the other categories of JIA. Patients were part of a multinational sample included in a study aimed to investigate the prevalence of disease categories, treatment approaches, and disease status in patients from across different geographical areas (EPOCA Study). All patients underwent a retrospective assessment, based on the review of clinical chart, and a cross-sectional evaluation, which included assessment of physician- and parent-reported outcomes and laboratory tests, and recording of ongoing therapies. Of the 9081 children with JIA enrolled in the EPOCA Study, 1300 were recruited at 18 paediatric rheumatology centres in Italy. 45 (3.5%) had ERA and 49 (3.8%) had JPsA. Several remarkable differences in demographic features and frequency of articular and extra-articular manifestations, disease damage, impairment in physical function and health-related quality of life, school-related problems, comorbidities, and ongoing treatments were observed between ERA and JPsA and the other JIA categories. We described the characteristics of Italian children with ERA and JPsA and highlighted their peculiarities and their differences from the other JIA subsets. These data provide useful insights for future revisions of JIA classification and a benchmarking against which the features from other cohorts may be compared.

Biologics in Rheumatoid Arthritis: When & Why?

Among the autoimmune rheumatic diseases RA is common. Not only articular extra-articular manifestations are common including involvement of skin, ocular, haematological, pulmonary and renal systems. A heightened cardiovascular risk also exists which closely follows disease activity stresses to obtain remission or low disease activity. Therapeutic landscape of RA treatment most significantly shiftedwith the introduction of biological disease modifying anti-rheumatic drugs (bDMARDs). There are five classes of bDMARDs currently available, each with a different molecular target and subtle differences in their efficacy and safety profile. Patient with poor prognostic factors and failed to csDMARDs are the candidate of anti-TNF agents and or other biologics. Clinicians should screen for latent infections with hepatitis B and C serology, chest radiograph plus interferon gamma release assay or tuberculin skin test. In the indicated cases, HIV serology should be requested as well. To reduce infection risk, guidelines specify that patients on bDMARDs should continue with national vaccination schedules including influenza and pneumococcal immunisations as well as the HPV vaccine for cervical cancer. Live attenuated vaccines (like, live herpes zoster vaccine, yellow fever, measles, mumps, rubella), are contraindicated in patients taking a bDMARD. The contraindications of anti-TNF agent’s initiations are serious or untreated infections including TB, current malignancy, multiple sclerosis, severe heart failure and hypersensitivity. In case of tocilizumab contraindications are serious or untreated infections, hypersensitivity and diverticulitisand for rituximab reactivation of hepatitis B.Coadministration of one class of bDMARD with another from a different class is not recommended due to an increased risk of infection. A DAS28 reduction of <1.2 should be considered as improvement at 3 and 6 monthvisits. A close monitoring is advocated for the adverse effects of biologic agents. TNF inhibitors may be continued during pregnancy. Rituximab, abatacept and IL-6 inhibitors are not safe in first trimester. Bangladesh J Medicine 2023; Vol. 34, No. 2(1) Supplement: 193-194

Borreliosis de Lyme en población pediátrica: peculiaridades clínicas, diagnósticas y terapéuticas

IntroductionLyme borreliosis (LB) in the paediatric population is an understudied entity with certain peculiarities. The objective of this study is to describe the characteristics of paediatric patients with LB, and their diagnostic and therapeutic processes. MethodsDescriptive and retrospective study in patients up to 14 years old with suspected or confirmed LB between 2015 and 2021. ResultsA total of 21 patients were studied: 18 with confirmed LB (50% women; median age 6.4 years old) and 3 false positive of the serology. Clinical features in the 18 patients with LB were: neurological (3, meningitis; 6, facial nerve palsy), dermatological (6, erythema migrans), articular (one), and non-specific manifestations (2). Serological diagnosis was confirmatory in 83.3% of cases. A total of 94.4% patients received antimicrobial treatment (median duration 21 days). All recovered with resolution of symptoms. ConclusionsLB diagnosis is difficult in the paediatric population and presents clinical and therapeutic peculiarities, with favourable prognosis.

Serum Interleukin-36 α as a Candidate Biomarker to Distinguish Behçet's Syndrome and Psoriatic Arthritis.

Behçet's syndrome (BS) is a rare systemic vasculitis characterized by different clinical manifestations. As no specific laboratory tests exist, the diagnosis relies on clinical criteria, and the differential diagnosis with other inflammatory diseases can be challenging. Indeed, in a relatively small proportion of patients, BS symptoms include only mucocutaneous, articular, gastrointestinal, and non-typical ocular manifestations, which are frequently found also in psoriatic arthritis (PsA). We investigate the ability of serum interleukin (IL)-36α-a pro-inflammatory cytokine involved in cutaneous and articular inflammatory diseases-to differentiate BS from PsA. A cross-sectional study was performed on 90 patients with BS, 80 with PsA and 80 healthy controls. Significantly lower IL-36α concentrations were found in patients with BS as compared to PsA, although in both groups IL-36α was significantly increased compared to healthy controls. An empirical cut-off of 420.6 pg/mL displayed a specificity of 0.93, with a sensitivity of 0.70 (AUC 0.82) in discriminating PsA from BS. This cut-off displayed a good diagnostic performance also in BS patients lacking highly specific BS manifestations. Our results indicate that IL-36α might be involved in the pathogenesis of both BS and PsA, and might be a candidate biomarker to support the differential diagnosis of BS.

Cellular indexing of transcriptomes and epitopes by sequencing (CITE-Seq) of peripheral blood to dissect the effect of different spondyloarthropathies

Abstract Spondyloarthritis is a family of immune mediated inflammatory disorders with overlapping articular and extraarticular clinical manifestations and a strong association to human leukocyte antigen (HLA)-B27. Despite a strong association of HLA-B27 with prototypic spondyloarthropathies such as axial spondyloarthritis (axSpA) and acute anterior uveitis (AAU), the underlying mechanisms are not known. Here, we aim to determine whether HLA-B27 associated axSpA and AAU are associated with common or divergent immunological markers in the blood. Previously, we did single cell RNA-seq analysis on a small number of patients with AAU and axSpA, which revealed increase in T cell, MAIT cells and NK cell clusters in AAU patients, whereas axSpA patients had increase in MAIT cells, gd-T cells, ILCs and Tregs and decrease in naïve CD8 T cell clusters, suggesting that distinct signatures maybe associated with axSpA and AAU. Changes in ILCs or MAIT cells may suggest involvement of the gut mucosal surface in pathogenesis. This is consistent with our previous studies showing gut microbial perturbation in clinical samples and in rat model of spondyloarthritis. Leveraging on our preliminary data, we are performing CITE-seq to capture paired transcriptome and cell surface epitope expression in individual PBMCs from 5 subjects each with axSpA, AAU and axSpA+AAU in comparison with healthy individuals (5 HLA-B27 negative and 5 HLA-B27 positive). Since, HLA-B27 allele is also present in healthy population, these analyses will allow us to dissect the effect of host genetics from the disease. These studies represent potential advances in the understanding of spondyloarthritis and may reveal genetic and disease biomarkers associated with axSpA and AAU. TG (principal investigator) is a recipient of research awards from the Spondylitis Association of America, SPARTAN (Spondyloarthritis Research and Treatment Network), GRAPPA (Group for Research and Assessment of Psoriasis and Psoriatic Arthritis), The Collins Medical Trust, Medical Research Foundation of Oregon and, Innovation Award from OHSU. This work was funded in part by the Grandmaison Fund for Autoimmunity Research. JTR is a recipient of the Innovative Research Award, Rheumatology Research Foundation.

Extra Articular Manifestations and its management in Rheumatoid Arthritis: An Overview

Extra Articular Manifestations and its management in Rheumatoid Arthritis: An Overview

Post-covid Henoch-schonlein Purpura: A Pediatric Case Report

We report the case of a 28-month-old boy who displayed symptoms of Henoch-Schonlein purpura (HSP), a type of small-vessel vasculitis that affects multiple systems in childhood and is caused by IgA. The symptoms include dermatological, abdominal, articular, and renal manifestations. HSP can occur due to several factors, such as upper respiratory tract infections, medications, vaccinations, and malignancies. The patient had positive COVID-19 serology but no other trigger factor for HSP, indicating a possible correlation between the two. The boy received oral prednisolone, leading to a swift clinical improvement. Our findings align with other research that suggests a link between the SARS-CoV-2 coronavirus and infantile HSP.

Increased Prevalence of Generalized Joint Hypermobility Observed in Patients With Recurrent Temporomandibular Joint Dislocation

Generalized joint hypermobility (GJH), determined by the Beighton score, is a fundamental part of diagnosing benign joint hypermobility syndrome (BJHS), which may also present extra-articular manifestations, and is determined by the Brighton criteria. This study was designed to investigate whether there is an association between recurrent temporomandibular joint (TMJ) dislocation and these disorders. A retrospective cross-sectional study was conducted. Hospital-based patients with a history of recurrent TMJ dislocation were compared to population-based patients that did not experience TMJ dislocations or any other TMJ disorders. Age and sex matching were performed between the study groups. All subjects reached the age of skeletal maturity. A history of recurrent TMJ dislocations. Measurements of Beighton score (range from 0 to 9 with a score of ≥ 4 indicating GJH) and correspondence to the Brighton criteria (with at least two "major" criteria or one "major" criterion plus two "minor" criteria or four "minor" criteria indicating BJHS). Included age and sex. Mann-Whitney U-test for continuous variables and the χ2 test or Fisher's exact test for categorical variables. Statistical significance was set at P<.05. A total of 68 participants were included, of whom 34 patients presented with recurrent TMJ dislocations compared with a control population of 34. The Mean participants were 31.35±8.06years, and 29.4% (n=20) were males. Of the dislocation group, 16 (47.0%) patients had a Beighton score of 4 or higher. The Beighton sum score was significantly higher, with a TMJ dislocation group mean score of 3.06±2.8, compared with a control score of 0.82±1.1 (P=.001). A total of 58.8% (n=20) of the TMJ dislocation group participants met the Brighton criteria versus none (0.0%) of the control group (P=.001). We found an association between recurrent TMJ dislocation and GJH. An association with BJHS was also found, based mainly on articular manifestations. Early detection of these disorders in patients suffering from recurrent TMJ dislocation may help identify individuals at increased risk for joint instabilities and allow the implementation of appropriate preventive management strategies.

ARTHRITIS AND ARTHRALGIA AS EXTRAINTESTINAL MANIFESTATIONS IN PATIENTS WITH INFLAMMATORY BOWEL DISEASE TREATED WITH VEDOLIZUMAB: A META-ANALYSIS

Vedolizumab is a humanized gut selective drug that specifically targets α4β7 integrin and has been used successfully in the treatment of Inflammatory Bowel Disease (IBD). Although Vedolizumab is an effective treatment for IBD and acts specifically on the gastrointestinal system, some studies have reported extraintestinal manifestations as adverse events. One of the most frequently reported non-infectious adverse events is articular manifestations. However, it is difficult to know for sure whether the joint symptoms are due to the vedolizumab adverse event or due to extraintestinal symptoms of the disease itself.

Canakinumab for Adult-Onset Still Disease

&#x0D; This review identified limited evidence from 2 systematic reviews about the clinical effectiveness and safety of canakinumab for patients with adult-onset Still disease (AOSD). Most studies in the systematic reviews on AOSD did not have a control group; therefore, the findings related to the effectiveness and safety of canakinumab in this population are uncertain.&#x0D; For adults with AOSD, treatment with canakinumab may be associated with a complete or partial response to treatment, corticosteroid-sparing effect, and a decrease or normalization of key laboratory parameters, such as leukocyte count, erythrocyte sedimentation rate, and serum levels of ferritin and C-reactive protein, compared with baseline.&#x0D; In adults with AOSD, limited evidence suggested there was no statistically significant difference between canakinumab and placebo in articular manifestations.&#x0D; Adverse events associated with canakinumab treatment included infections, leukopenia, musculoskeletal and connective tissue disorders, skin disorders, thoracic and mediastinal disorders, gastrointestinal disorders, and site injection reactions. Severe adverse events included macrophage activation syndrome, hepatocytotoxicity, and serious infections that led to discontinuation of treatment.&#x0D; No studies were found comparing canakinumab and alternative pharmacologic treatments for AOSD.&#x0D; No studies were found on the cost-effectiveness of canakinumab for the treatment of AOSD.&#x0D; No evidence-based guidelines were found on the use of canakinumab for the treatment of ASOD.&#x0D;