HLA-B27 Research Articles

E084 Analysis of the Early Inflammatory Back Pain Clinic at the Royal National Hospital for Rheumatic Diseases

Abstract Background/Aims The Royal National Hospital for Rheumatic Diseases (RNHRD) has a long-standing history of excellence in the care and management of axial spondyloarthritis (axSpA). Research has shown that early diagnosis of axSpA is associated with health-related benefits. However, because diagnosis earlier in the disease trajectory can be clinically challenging, there is often a significant delay. New referrals of possible axSpA are reviewed in the early inflammatory back pain clinic, and this audit of the demographics and diagnoses of clinic attendees aims to follows-up the patients post clinical evaluation to assess the frequency of missed diagnoses. Methods Electronic medical records of 100 consecutive new referrals attending for back pain between 03/09/2015 and 27/07/2016 were retrospectively reviewed. The information abstracted included sex, age, smoking status, investigations, and diagnosis. Clinic letters and follow-up records at the Royal United Hospitals until July 2022 were reviewed. Results The average age of the patients was 37.4 years; 42% were male and 58% female. The diagnostic breakdown of the 100 patients is presented in Table 1. Of the 21 patients diagnosed with axSpA, 14 (66%) were human leukocyte antigen B27 (HLA-B27) +ve; compared to only 7 (8.8 %) of the 79 patients who did not have an axSpA diagnosis. The electronic records of these 7 HLA-B27+ve patients were reviewed in July 2022, and no indication of a subsequent axSpA diagnosis was found; notably, one patient subsequently developed enteropathic arthritis. Of the 71 HLA-B27-ve patients categorised as having a non-inflammatory cause of their symptoms, three were re-referred to the RNHRD, but none were diagnosed with axSpA. Conclusion We found no evidence of a subsequent diagnosis of axSpA in patients who did not have the disease diagnosed at the time of initial referral. This audit identified a small cohort of HLA-B27+ve patients without evidence of axSpA; a more in-depth analysis of this patient group is planned. Disclosure M. Cowan: None. R. Sengupta: None.

P177 A UK survey evaluation of first contact practitioner and physiotherapist confidence, recognition and referral of suspected axial spondyloarthritis

Abstract Background/Aims Axial spondyloarthritis (axSpA) is an inflammatory disease associated with significant diagnostic delays. A previous evaluation of confidence, awareness and knowledge of axial spondyloarthritis (Steen et al, 2021) found physiotherapists lacked adequate consideration of axSpA in back pain assessments and of awareness of features that raise suspicion. Since the previous survey, professional education on axSpA has occurred. Furthermore, first contact practitioners (FCPs) are now widely established in general practice and key in supporting earlier recognition. The aims of this study were to evaluate FCP and physiotherapists' and re-evaluate musculoskeletal physiotherapists' awareness, knowledge, and confidence in recognising features of axSpA and of criteria supporting referral to rheumatology to compare to previous research which suggested the need for further professional education. Methods Using the previous strategy (Steen et al., 2021), an online survey was undertaken with a combined approach. This included back pain presentation vignettes with features of axSpA, non-specific low back pain (NSLBP) and radicular syndrome to evaluate screening knowledge, clinical reasoning and recognition, and section two with questioning on features of suspected axSpA. Analysis used descriptive statistics and content analysis informed by advocated criteria and resources, including ASAS and NICE guidance, that support recognition and referral of axSpA in clinical practice. Results There were 165 completed responses analysed. Only 73% (n = 120/165) of respondents recognised the axSpA vignette compared to NSLBP (91% [n = 80/88]) and radicular syndrome (88% [n = 68/77]). A trend towards improvement in axSpA recognition was demonstrated compared with previous data. FCPs performed slightly better, with 77% (n = 67/87) of respondents recognising the axSpA vignette. Adequate awareness of national referral guidance was evident in only 55% of ‘clinical reasoning’ and 6% of ‘further subjective screening’ responses. There was reduced awareness of NICE guidelines on spondyloarthritis compared with back pain guidelines; however, more awareness of all guidelines was found amongst FCPs. More knowledgeable responses were associated with familiarity with the NICE spondyloarthritis guidance (2017) and National Back and Radicular Pain Pathway (2017), CPD on LBP and spondyloarthritis, NHS employment, FCP role, ≥60% LBP caseloads, GP referrals and professional experience. There is still misplaced confidence in recognising clinical features of axSpA and when to refer compared to knowledge levels demonstrated. High importance is still given to inflammatory markers, HLA B27 positivity; however, appropriately, less emphasis is given to gender. Conclusion Musculoskeletal physiotherapists demonstrated some improved knowledge and awareness of axSpA compared with previous findings. Consideration of axSpA is still not universal in musculoskeletal physiotherapists' or FCPs' approaches to persistent back pain assessments, along with a lack of awareness of national referral guidance for suspected axSpA. This study highlights the need for ongoing professional education to make screening and referral criteria for suspected axSpA core knowledge in musculoskeletal clinical practice to support earlier diagnosis and better outcomes. Disclosure E. Steen: None. M. Cairns: None. C. McCrum: None.

Changes in C-reactive protein, erythrocyte sedimentation rate, human leukocyte antigen-B27, and immunoglobulins A, G, M in patients with ankylosing spondylitis

Abstract Background To explore the changes in C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), human leukocyte antigen-B27 (HLA-B27), and immunoglobulin (Ig) (IgA, IgG, and IgM) in patients suffering from ankylosing spondylitis (AS). Methods A total of 232 patients with axial spondyloarthritis admitted from January 2020 to December 2021 were selected, among whom 132 were AS patients (AS group) and 100 were non-AS cases (non-AS group). Meanwhile, 100 healthy subjects undergoing physical examination were included as a control group. Peripheral blood was collected to detect the levels of CRP, ESR, HLA-B27, IgA, IgG, and IgM. Results The positive rates and levels of CRP, ESR, HLA-B27, IgG, and IgM in non-AS and AS groups were significantly higher, and the IgA level of the AS group was higher than those of the control group (P<0.05). The AS group had significantly higher positive rates and levels of CRP, ESR, HLA-B27, and IgA than those of the non-AS group (P<0.05). The levels of HLA-B27, ESR and CRP had positive correlations with those of IgA, IgG and IgM (P<0.05). The area under the curve (AUC) of HLA-B27 with a cut-off value of 53.18 U/mL for the diagnosis of AS was 0.879. AUC of ESR with a cut-off value of 23.83 mm/h for the diagnosis of AS was 0.811. AUC of CRP with a cut-off value of 14.35 mg/L for the diagnosis of AS was 0.745. With the cut-off values of 2.75 g/L, respectively, AUC of IgA for the diagnosis of AS were 0.668. AUC of the combined diagnosis was 0.929 (P<0.05). Conclusions AS patients have abnormally elevated levels of HLA-B27, ESR, CRP, and IgA, and the combined detection has a higher diagnostic value. The abnormal increase in IgA, IgG, and IgM is positively correlated with rheumatic inflammation marker HLA-B27, CRP, and ESR.

Diagnosis, Treatment, and Prevention of Noninfectious Acute Anterior Uveitis with or without Human Leukocyte Antigen B27 in Adults - Expert Consensus in Taiwan

ABSTRACT Background Anterior uveitis is the most common anatomical type of uveitis. Patients with noninfectious anterior uveitis may develop various ocular complications and eventually visual impairment. Appropriately differentiating the etiologies can help clinicians to predict the outcome, arrange clinical follow-up, and decide the treatment or prevention strategy. Adequate treatment and effective prevention strategies can reduce the frequency of recurrence and the risk of developing complications. Human leukocyte antigen (HLA)-B27 is the most common positive finding in patients with noninfectious AAU in many countries including Taiwan. Purpose To report a consensus from experienced uveitis specialists and rheumatologists was made in Taiwan. Methods A panel of nine ophthalmologists from nine different referral centers with expertise in the management of uveitis and an experienced rheumatologist was held on January 16, 2022. A comprehensive literature review was performed. Differential diagnoses for etiologies, general treatments, and prevention strategies were discussed. Each statement in the consensus was made only if more than 70% of the experts agreed. Results A flow chart and seven statements regarding the differential diagnoses for etiologies, treatments and preventions, and co-management with rheumatologists were included in the consensus. Conclusions This article discusses the general diagnosis, treatment, and prevention of noninfectious acute anterior uveitis, with or without HLA-B27, in adults for general ophthalmologists to improve overall outcomes of these patients

Vitamin D Status and Psoriatic Arthritis: Association with the Risk for Sacroiliitis and Influence on the Retention Rate of Methotrexate Monotherapy and First Biological Drug Survival-A Retrospective Study.

A growing body of evidence on the importance of vitamin D in immune modulation has increased the interest in its possible impact on the course of rheumatological diseases. The scope of our study is to assess if the presence of different statuses of vitamin D could interfere in the clinical subsets, in methotrexate monotherapy discontinuation, and biological drug (b-DMARDs) survival in psoriatic arthritis patients (PsA). We conducted a retrospective study on PsA patients and split them into three groups based on their vitamin D status: the group with 25(OH)D ≤ 20 ng/mL, the group with levels of 25(OH)D between 20 and 30 ng/mL, and the group with serum levels of 25(OH)D ≥ 30 ng/mL. All patients were required to fulfill the CASPAR criteria for psoriatic arthritis and to have the evaluation of vitamin D serum levels at baseline visit and at clinical follow-up visits. The exclusion criteria were ages less than 18 years old, the presence of HLA B27, and satisfaction of rheumatoid arthritis classification criteria (during the study time). Statistical significance was set at p ≤ 0.05. Furthermore, 570 patients with PsA were screened and 233 were recruited. A level of 25(OH)D ≤ 20 ng/mL was present in 39% of patients; levels of 25(OH)D between 20 and 30 ng/mL presented in 25% of patients; 65% of patients with sacroiliitis presented 25 (OH)D ≤ 20 ng/mL. Methotrexate monotherapy discontinuation for failure was higher in the group with 25 (OH)D ≤ 20 ng/mL (survival time: 92 ± 10.3 weeks vs. 141.9 ± 24.1 weeks vs. 160.1 ± 23.6 weeks; p = 0.02) with higher discontinuation risk (HR = 2.168, 95% CI 1.334, 3.522; p = 0.002) than those with 25(OH)D between 20 and 30 ng/mL and those with 25(OH)D ≥ 30 ng/mL. Significantly shorter survival of first b-DMARDs was assessed in the group with 25 (OH)D ≤ 20 ng/mL versus the other groups (133.6 ± 11 weeks vs. 204.8 ± 35.8 weeks vs. 298.9 ± 35.4; p = 0.028) (discontinuation risk 2.129, 95% CI 1.186, 3.821; p = 0.011). This study highlights significant differences in clinical presentation, in particular sacroiliac involvement and on drug survival (methotrexate and b-DMARDs) in PsA patients with vitamin D deficiency. Further prospective studies, including a larger sample of patients, are needed to validate these data and to assess if the supplementation of vitamin D could improve the b-DMARDs response in PsA patients.

Spondyloarthritis Associated with Uveitis: A Review.

Spondyloarthritis (SpA) is a group of chronic inflammatory rheumatism characterized by common clinical, radiological, and biological manifestations occurring on a predisposing genetic background dominated by the HLA-B27 antigen. Acute anterior uveitis is the most common extra-articular feature of SpA. The objective of this review is to describe the prevalence, demographic characteristics, factors favoring the occurrence of uveitis in patients with SpA, clinical manifestations, and their therapeutic management.

The Presence of Pigmentation on Central Anterior Lens Capsule in the Human Leukocyte Antigen B27-Associated Anterior Uveitis and Its Value in Clinical Diagnosis

ABSTRACT Purpose To report on the value of presence of pigmentation on central anterior lens capsule (PioLe) in HLA B27- associated anterior uveitis (HLA B27-AU). Methods 268 patients (320 eyes) with AU were reviewed. Two diagnostic models to predict probability of HLA-B27-AU were developed. The first model included 6 variables (age, gender, unilaterality, presence of non-granulomatous keratic precipitates, hypopyon, and intraocular pressure (IOP). The second model was developed to investigate the added value of PioLe into the first model. Results Unilaterality, presence of hypopyon, IOP <21 mmHg and PioLe were characteristic for HLA-B27 positive patients (P≤0.003 for all). All of 6 variables had area under receiver operating characteristic curves (AuROC) ≤ 60, but PioLe reached even higher value (65.5). Diagnostic model I and II had AuROC 76.3% (95%CI, 68.4%-84.2%) and 80.0% (95%CI, 72.6%-87.5%), respectively. Conclusions Unilaterality, hypopyon, IOP <21 mmHg and presence of PioLe are clinical signs suggesting HLA B27- AU.

Ankylosing Spondylitis and Right Lower Limb Lymphoedema A Rare Association: A Case Report and Review of the Literature

Introduction: Ankylosing spondylitis or rheumatic pelvispondylitis is a chronic inflammatory rheumatism characterized by an axial localization predominantly in the spine and sacroiliac joints, an involvement of the entheses and a tendency to bone ankylosis by ossification of these entheses. We report an observation of right lower limb lymphedema associated with ankylosing spondylitis. Observation: This was a 63-year-old patient with a history of inflammatory rheumatism (his older sister) and osteoporosis (his mother), He was diagnosed with ankylosing spondylitis based on enthesis involvement (bilateral tilted gluteal pain and low inflammatory back pain) with a positive HLA-B27 antigen by the 8-point Amor criteria for spondyloarthritis (right unilateral sacroiliitis, low inflammatory back pain, bilateral tilted gluteal pain, HLA-B27+ and NSAID sensitivity). The unilateral lymphedema with positive Stemmer's sign affected the lower 2/3 of the right lower limb. It occurred one year after the onset of ankylosing spondylitis. The echo-Doppler scan showed no signs of thrombosis or venous infiltration of the lower limbs, and diffuse infiltration of the subcutaneous tissue of the right leg without inflammatory character. Treatment of ankylosing spondylitis gradually improved the lymphedema. Conclusion: Lymphedema is a rare complication of ankylosing spondylitis. Dermatologists should be alert to early swelling of the extremities in patients with ankylosing spondylitis so as not to delay specific multidisciplinary treatment and measures to avoid irreversible lymphatic damage.

Ankylosing Spondylitis and Autoimmune Thrombocytopenia: the First Senegalese Case

Introduction: Ankylosing spondylitis (AS) and autoimmune thrombocytopenia are two distinct diseases, and they can occur rarely. Objective: To determine the epidemiological, clinical, therapeutic profiles and impact of this rare association in the literature Observation: This was a 57-year-old teaching patient received on September 07, 2020. The examination of the musculoskeletal system revealed lumbar pain. The onset was 26 years ago and was marked by the progressive onset of pain located in the dorsal spine, lumbar and manubrio-sternal region, then a progressive extension to both wrists and the left knee with a VAS of 6/10. Associated signs were stiffness and synovitis of the left knee. The biology showed a non-specific biological inflammatory syndrome with accelerated blood pressure at 70 minutes in the first hour and CRP increased to 15 mg/l, normocytic normochromic anemia (hemoglobin 7.6 g/dl), thrombocytopenia at 51,000/mm3 (N : leukopenia at 3,100/mm3 and neutropenia at 1115/mm3 (pancytopenia). The transaminases ALAT was 60 IU/L and Gamma GT was increased to 265 IU/L, the immunological tests were negative with the presence of the HLA B27 positive antigen, and the fixed anti-platelet antibodies showed the presence of fixed autoantibodies directed against the glycoprotein complex IIb and IIIa and the absence of circulating antibodies directed against the glycoprotein complexes IaIIb, IIbIIIa and IbIX. The lumbar CT scan shows syndesmophytes and osteophytes (Fig 2) and the CT scan of the pelvis shows Forestier stage III sacroiliitis (Fig 1). Conclusion: The association of ankylosing spondylitis and autoimmune thrombocytopenia is rare but possible, and should be considered whenever a known spondyloarthritis patient presents with a platelet collapse. Treatment with methotrexate and anti-inflammatory drugs may be effective in some cases.

Recent updates in enthesitis-related arthritis.

Enthesitis-related arthritis (ERA) is a category of juvenile idiopathic arthritis which belongs to the spectrum of diseases that are included in juvenile spondyloarthropathy. In recent years, there have been significant advances in understanding pathogenesis, tools to assess disease activity, early recognition of the axial disease, and targeted therapy using IL-17 inhibitors and small molecule inhibitors. The current narrative review highlights these new advances. Among many hypotheses linking HLA B27 to ERA, one of them is the effect of HLA B27 on gut dysbiosis. However, recent data suggest that gut dysbiosis is probably not determined by HLA B27. Though children present with arthritis and enthesitis, axial disease is present in 50-60% on MRI. Using data-driven approach, discriminative MRI finding for active and chronic diseases has been defined for children. This will help in the early recognition of disease. An abridged version of juvenile spondyloarthropathy disease activity (JSpADA) score without the need for acute phase reactants and Schober test performed as well as the original score may increase its acceptance in routine practice. Secukinumab (anti-IL-17 antibody) has shown a more than 75% response rate in children with ERA and may be a good alternative to anti-TNF therapy. Initial data with tofacitinib also look promising. All these will translate into better outcomes for children with ERA.

Juvenile Idiopathic Arthritis and Its HLA Association: A Study from Bangladesh

Background: HLA alleles may have association with overall JIA including specific sub-types. Determation of HLA DRB1, DPB1, DQA1, DQB1 and B27, may be helpful to diagnose JIA cases where diagnostic dilemma is present. The aim of the study was to find out the association of HLA alleles with JIA and its subtypes. Methods: This cross sectional study was conducted in the department of Pediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, from April 2020 to March 2021. A total of 50 cases and 25 controls attending pediatric rheumatology clinic and pediatric OPD were enrolled in the study. Data were collected by a pre-designed questionnaire containing socio-demographic, clinical and laboratory parameters. HLA DR-B1, DQ-A1, DQ-B1 and B27 typing were done for the cases and controls from blood samples by polymerase chain reaction sequence specific primer (PCR-SSP) method. Data were analyzed by SPSS version 26. Frequency, percentage, chi square test and Fisher exact tests were done for statistical analysis. Results: HLA DR-B1_10 had significant positive association whereas HLA DR-B1_03 and B1_16 had negative association with overall JIA cases. Poly-articular JIA, oligo-articular JIA and ERA had association with HLA DRB1_01, B1_08 and B1_12 respectively. HLA DQA1-01 and A1_02 had positive association. However, DQ-A1_06 was negatively associated with JIA cases. Oligo JIA was positively associated with HLA DQ-A1_01, whereas poly JIA and ERA had association with A1_02. Though, HLA DQ_B1 had no association with overall JIA cases, analysis showed association of poly JIA with DQ-B1_04. Frequency of HLA DQ-B1_02 and 03 were higher among ERA cases, though not significant, but they had strong association with HLA B27. Conclusion: HLA associations were present with overall JIA and each sub-groups had different patterns of HLA associations including some protective roles.

DETERMINING THE ROLE OF HLA-B27 ON COVID-19

OBJECTIVE: Human leukocyte antigen-B27 (HLA-B27) binds antigenic peptides in the structure of some viruses and presents them to cytotoxic T lymphocytes and plays a role in the immune response against these viruses. It has also been found to be associated with auto-immunity as an inverse relationship and is associated with a number of auto-immune inflammatory diseases, especially Ankylosing Spondylitis (AS). Our aim in this study is to determine the role of HLA-B27 in Severe Acute Respiratory Syndrome (SARS)-Cov-2 (Coronavirus Disease-COVID-19). METHOD: 90 HLA-B27 positive and 96 HLA-B27 negative cases were included in the study. COVID-19 Polymerase Chain Reaction (PCR) results and Thorax Computed Tomography (CT) results, hospitalization and death records of the cases were retrospectively analyzed from the system records. Results were compared between HLA-B27 positive and negative groups. RESULTS: Of the HLA-B27 positive cases, 32.2% had COVID-19 positivity, 5.6% had COVID-19 lung involvement. 44.8% of HLA-B27 negative cases had COVID-19 positivity and 11.5% had lung involvement. There was no significant difference between the HLA-B27 positive and negative groups in terms of having COVID-19 infection, lung involvement, hospitalization and death rates. CONCLUSION: The results of our study show that HLA-B27 does not have a protective role in terms of having COVID-19 and lung involvement. Again, since no auto-immunity-related SARS was observed in any of the patients, it can be said that it did not increase the severity of the hyper-immunity-related disease and did not increase the risk of death in particular for COVID-19. These results need to be supported by more studies.

Evaluation of Human Leukocyte Antigen-B27 Expression in Patients with Spondylopathy/ Spondylitis by Flowcytometry

Introduction: The role of immune mediated injury in pathogenesis of Ankylosing Spondylitis (AS) is well established. HLA B27, a Major Histocompatibility Complex (MHC) class I molecule is one of the major genetic risk factors associated with the disease. Various techniques are used for testing HLA-B27 which includes Polymerase Chain Reaction (PCR) based tests, Enzyme Linked Immunosorbent Assay (ELISA) and flowcytometry. Flowcytometry has gained popularity due to ease of procedure, shortened turnaround time and costeffectiveness. Aim: The aim of the present study was to assess the sensitivity and specificity of flowcytometry for HLA-B27 detection, taking PCR assay as the gold standard along with its association with demographic, clinicopathological and radiological parameters. Materials and Methods: This was a prospective study conducted in Department at Pathology of Shri Guru Ram Rai Institute of Medical and Health Sciences, Dehradun, India, for a period of 18 months from January 2020 to June 2021. The study included 51 patients for which HLA-B27 typing was done cases by flowcytometry and Sequence Specific Allele (SSA) PCR/ Real time PCR on peripheral blood samples. The association of HLA-B27 with clinical features {Inflammatory Back Pain (IBP), arthritis, psoriasis, uveitis, dactylitis, Inflammatory Bowel Disease (IBD), cervicitis, urethritis, diarrhoea) along with MRI findings (sacroiliitis)}, laboratory findings {C-reactive protein and Erythrocyte Sedimentation Rate (ESR)} was evaluated. The performance analysis parameters of flowcytometry were evaluated both by excluding and including the cases in grey zone taking PCR as gold standard. Statistical testing was conducted with SPSS 20.0. Chi-square test or Fisher’s-exact test were used and a p-value of less than 0.05 was taken as significant. Results: A significant association of HLA-B27 was seen only with IBP (p-value= 0.001) and sacroiliitis (p-value= 0.03). Of the 22 (43.1%) patients positive for HLA-B27 by PCR, 18(81.8%) patients were positive while the remaining 4(18.1%) were in grey zone by flowcytometry. Of the 29(56.9%) patients testing negative by PCR, 27(93.1%) patients were negative, one (3.4%) was in grey zone and one (3.4%- false positive) tested positive for HLA-B27 by flowcytometry. Sensitivity and specificity of flowcytometry for detection of HLA-B27 was found to be 100% and 96.4% respectively when grey zone cases were excluded. Conclusion: The study brings to light that flowcytometry is a fairly specific and sensitive method for HLA-B27 detection with a high Negative Predictive Value (NPV) (100%) and Positive Predictive Value (PPV) (94.7%). In the COVID-19 era, it reiterates the importance of flowcytometry for HLA-B27 especially when PCR is overburdened.

Clinical Manifestations of Behçet's Disease: A Retrospective Cross-Sectional Study.

Behçet's Disease (BD) is a systemic vasculitis, highly prevalent in Eastern Asia to Mediterranean countries. Iran is among the countries with the highest prevalence of BD, and previous studies in different countries have shown a broad range of clinical manifestations of the disease. The present study is conducted to evaluate the prevalence of the clinical manifestations of BD in patients referring to rheumatology clinics of two distinct referral hospitals in Tehran and Zanjan, Iran. In this retrospective, cross-sectional study, the medical records of patients with BD were reviewed, and age at onset, sex, the delay between the onset of symptoms and diagnosis, clinical manifestations, HLA B27, HLA B51, HLA B5, haematuria, proteinuria, leukocyturia, Erythrocyte Sedimentation Rate (ESR), and pathergy phenomenon were included in the study. The collected data were analysed by χ2 test using SPSS 23. A total of 188 patients (Male/female ratio = 1.47) were included in the study with mean ± SD age at onset of 27.98 ± 10.47 years and a mean ± SD of delay between the onset of symptom and diagnosis of 5.70 ± 7.16 years. The most common clinical manifestation was mucosal involvement (85.1%), followed by the ocular lesion (55.3%) and skin manifestations (44.7%). The Pathergy phenomenon was observed in 98 patients (52.1%). Moreover, 45.2% had positive HLA B5, followed by HLA B51 (35.1%) and HLA B27 (12.2%). This study demonstrated that male/female ratio and mean age at onset were comparable to the results of previous studies in Iran. Significant associations between HLAB5 and clinical manifestations underline the pivotal role of genetic factors in BD.

Association of Chlamydia trachomatis and HLA B27 in ankylosing spondylitis in an unmarried sexually active young adult

Association of Chlamydia trachomatis and HLA B27 in ankylosing spondylitis in an unmarried sexually active young adult

HLA-B27 positive anterior uveitis : Clinical aspects, diagnostics, interdisciplinary management and treatment

Acute anterior uveitis (AAU) associated with human leukocyte antigen (HLA) B27 is the most common form of noninfectious intraocular inflammation and is considered to be aseparate clinical entity. Young adults between the ages of20 and40 years are predominantly affected. The HLA-B27 positive AAU typically presents as aunilateral, fulminant disruption of the blood-aqueous humor barrier, which is accompanied by pronounced cellular infiltration and fibrinous exudation. Other characteristics are reduced intraocular pressure and ahigh tendency to relapse, which can also involve the partner eye. Patients with HLA-B27 positive AAU share ahigh risk for other genetically associated diseases, especially spondylarthritis, chronic inflammatory bowel diseases and psoriasis. As up to 40% of those affected have asystemic disease that has not yet been diagnosed, the ophthalmologist is of major importance for early detection.

Эффективность и безопасность различных режимов отмены ингибиторов фактора некроза опухоли альфа при длительной ремиссии ювенильного идиопатического артрита без системных проявлений: результаты проспективного простого рандомизированного исследования

Including the tumor necrosis factor TNF inhibitors (TNFi) in the management guidelines for children with juvenile non-systemic juvenile idiopathic arthritis (JIA) has significantly increased the effectiveness of antirheumatic treatment. However, the guidelines of TNFa withdrawal when disease remission is achieved remains unclear. Objective. To study the efficacy and safety of different TNFi withdrawal regimens in patients with juvenile idiopathic arthritis without systemic manifestations due to the achievement of long-term disease remission. Patients and methods: The prospective simple randomized trial included 76 patients with JIA without non-systemic juvenile idiopathic arthritis, with a disease remission duration of ≥24 months, developed under the conditions of treatment with TNFi. Of these, 38.2% are male, the average age is 11.6 years; 67 (88.2%) patients received etanercept (0.8 mg / kg / week. subcutaneously 1 time per week), the rest – adalimumab (24 mg/m2 subcutaneously 1 time in 2 weeks). Depending on the withdrawal regimen, patients were divided into three groups: in the I group, one-time withdrawa was presented, in the II group, the interval between administrations was of two times and the III group was reduced by a dose of two times. The main indicator of the study: preservation of the stage of inactive disease / remission (according to the criteria for remission of C. Wallace) for 6, 12 and 18 months after the withdrawal of TNFi. Results. At the time of withdrawal of TNFi, the average duration of the disease was 7.3 years, the duration of therapy with TNFa was 5 years, remission – was 4.4 years. For 6, 12 and 18 months after the withdrawal of TNFi, the stage of remission persisted in 44 (57.9%), 21 (27.6%) and 18 (23.7%) patients. Exacerbation of disease was registered in 58/76 (76%) patients. Biologic therapy was resumed in 56/58 children, in all cases the stage of inactive disease was reached. Among patients with exacerbation of JIA, 23/56 (41%) continued to receive disease-modifying antirheumatic drugs. Predictors of successful withdrawal of TNFa in patients with non-systemic JIA 6 and 12 months after discontinuation of the biological therapy were the female sex, the absence of HLA B27, antinuclear factor and long-term methotrexate therapy; predictors of exacerbation - increasing in the serum concentration of calprotectin (S-100 protein), the presence of subclinical synovitis according to ultrasound and MRI of the joints and uveitis. Conclusion. After the withdrawal of TNFi, the remission stage for 6 and 12 months persisted more than in half and one third of patients. The withdrawal regimens did not affect the duration of the remission of disease. The resumption of biological therapy in the exacerbation stage of JIA made it possible to reach the remission stage in most patients. Predictors of successful withdrawal of TNFi in patients with non-systemic JIA 6 and 12 months after discontinuation of the biological therapy were the female sex, the absence of HLA B27, antinuclear factor and long-term methotrexate therapy; predictors of exacerbation – increasing in the serum concentration of calprotectin (S-100 protein), the presence of subclinical synovitis according to ultrasound and MRI of the joints and uveitis. The presence of exacerbation predictors makes it impractical the withdrawal of biological therapy due to the high risk of developing an exacerbation of disease. Key words: (TNF)-alpha inhibitors, juvenile idiopathic arthritis, long-term remission, withdrawal predictors, high-sensitivity C-reactive protein (hsCRP), serum calprotectin (S-100 protein)

Clinical profile of anterior uveitis in a tertiary eye care center

Background: Anterior uveitis is the inflammation of the anterior uveal tissue that is iris and pars plicata of the ciliary body. Due to varied etiology, detailed history and systematic investigations are needed for accurate diagnosis and management. Aims and Objectives: The aims of this study were to describe the clinical features, causes, and management of patients with acute anterior uveitis. Materials and Methods: A prospective study of clinical features, complication, and prognosis was done on anterior uveitis patients for a period of 10 months. Detailed history, complete ocular examination, systemic evaluation, and relevant investigations were done in 64 patients; appropriate systemic and ocular management were done. Results: Of 64 patients, 65.6% were male and 34.3% female. Mean age of presentation was 42.6 years. About 71.8% unilateral and 28.1% bilateral eye involvement were seen. Visual acuity was between 6/18 and 3/60 at the time of presentation. About 21.8% had tuberculosis, 9.3% had trauma, 4.6% had UTI, 3.1% had ankylosing spondylitis (HLA B27 associated) and 3.1% had rheumatoid arthritis, 3.1% had sarcoidosis, and 1.5% had toxoplasmosis. About 53.1% were idiopathic. Complications were noted in 50% of cases, 25% had secondary glaucoma, 21.8% had posterior subcapsular cataract, and 3.1% has cystoid macular edema. Appropriate medical management was started in patients. Conclusion: Idiopathic uveitis was higher in our study. Trauma was the most common non-infectious entity. Tuberculosis was most common infectious cause in our study, all patients responded well with medication.

Clues for inflammatory diseases in the differential diagnosis of a child with sacroiliitis.

The purpose of this study was to compare the demographic, clinical and laboratory characteristics of patients with enthesitis-related arthritis (ERA), familial Mediterranean fever (FMF) and inflammatory bowel disease (IBD), which are inflammatory diseases that may develop sacroiliitis. Thus, it was aimed to reveal various findings that may indicate primary disease in patients with sacroiliitis. Pediatric patients aged 6-18 years, who were being followed with a diagnosis of ERA (n= 62), FMF (n= 590), and IBD (n= 56) over the period 2013-2021 were included in the study. Sacroiliitis (n= 55) was diagnosed by magnetic resonance imaging of the sacroiliac joint, obtained from clinically suspected patients. Sacroiliitis was detected in 54.8% of ERA patients, 2.3% of FMF patients, and 12.5% of IBD patients. The mean follow-up period was 4.1± 2.8 years (10 months-8 years) for the entire study group. The most common MRI finding for sacroiliitis was bone marrow edema. Peripheral joint involvement (73.5%) and HLA B27 positivity (64.7%) was significantly higher in ERA patients, and ERA was diagnosed more frequently in patients presenting with sacroiliitis. Non-steroidal anti-inflammatory drugs (NSAIDs) were the first choice of treatment agent when sacroiliitis developed in all three patient groups. The clinical and laboratory findings of ERA, FMF and IBD can sometimes be intertwined or can even coexist. Treatment may differ depending on the disease associated with sacroiliitis, although NSAIDs may be used in the first-line treatment of all three diseases. Sacroiliitis patients with HLA B27 positivity and peripheral arthritis may need to be addressed as ERA.

Uveitis in spondyloarthropathies

Background: Uveitis is associated with several systemic disorders. It may be the initial presentation or manifestation of a systemic disease. It is the most common ocular complication and is sometimes the earliest manifestation of spondyloarthropathies. This study aimed to review the current literature on spondyloarthropathies and associated uveitis.&#x0D; Methods: A narrative review was performed using various combinations of the keywords spondyloarthropathies, seronegative spondylarthritis-related uveitis, and human leukocyte antigen-associated uveitis using PubMed/MEDLINE and Google Scholar from January 1, 2000, to September 30, 2022. We describe the disease mechanisms, genetics, and classification of spondyloarthropathies, the clinical patterns of their related ocular diseases, and the current modalities for the management of their ocular or systemic manifestations.&#x0D; Results: Seronegative spondyloarthropathies are a group of rheumatic disorders including ankylosing spondylitis, reactive arthritis, psoriatic arthritis, inflammatory bowel disease-related spondyloarthropathy, juvenile-onset spondyloarthritis, and undifferentiated spondyloarthritis. These are characterized by enthesitis in the absence of serum rheumatoid factor and have a strong association with human leukocyte antigen B27. The clinical courses and features of spondyloarthropathies are remarkably diverse. Ocular inflammation is common in spondyloarthropathies, often precedes the onset or diagnosis of systemic disease, and responds well to topical therapy. Timely diagnosis of systemic diseases may improve quality of life and help avoid ocular and skeletal complications. Recurrence of ocular inflammation is frequent; on occasion, it may be associated with etanercept administration.&#x0D; Conclusions: Eye care professionals should be able to recognize spondyloarthropathies, manage ocular disease, and collaborate with related specialties for modification of systemic treatment if associated with ocular complications. Timely referral and early management could attenuate or prevent ocular or systemic morbidities associated with spondyloarthropathies.