Rheumatology Association Research Articles

E15 Immunopositive and immunonegative juvenile idiopathic arthritis: what makes the difference?

Abstract Background Juvenile idiopathic arthritis (JIA) is a complex pediatric rheumatic disease of polymorphic expression. The immunological assessment is commonly performed during this disease. Objectives The objective of this work was to evaluate the difference between immunopositive and immunonegative forms of JIA. Methods We conducted a retrospective study including adults with long-standing JIA according to International League of Associations of Rheumatology (ILAR) criteria over a 28-year period (1994 – 2022). We retrieved the immunological results of all patients. The disease was considered immunopositive in case of rheumatoid factor (RF) and/or anti-citrullinated peptide antibody (ACPA) positivity. Results We included 29 patients (12 men and 17 women) with a mean age of 35.69 ± 11.72 [18–61] years. The mean age of disease onset was 11.10 ± 4.25 [2–16] years. The average diagnostic delay was 52.96 ± 95.97 [0–336] months. The average disease duration was 24.48 ± 12.76 [1–47] years. The mean body mass index (BMI) was 21.20 ± 4.88 [14.17–27.55] kg/m2, 8 patients had a normal BMI. Growth delay was noted in 3 cases. The polyarticular form was the most frequent, observed in 55.2% of cases. At least one extra-articular manifestation was noted in 16 patients: ophthalmologic, skin, cardiac, pulmonary, and renal involvement were found in 6,4,4,3 and 2 cases, respectively. Osteoporosis was noted in 7 cases. Mean C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were 42.74 ± 63.37 [2–218] mg/l and 69.67 ± 36.23 [36–108] mm/h. Bone erosions, hip involvement and atlantoaxial dislocation were noted in 18, 14, and 4 cases, respectively. Rheumatoid factor, ACPA and anti-nuclear antibodies were observed in 12, 7 and 5 cases respectively. The disease was considered immunopositive in 13 cases. Our comparative study found that women had more immunopositive forms (84.6% vs 15.4%; P = 0.010). In addition, osteoporosis was less frequent in immunopositive JIA (11.1% vs 88.9%; P = 0.003). Growth delay was only observed in immunopositive JIA (P = 0.012). Furthermore, the age at disease onset was greater in the immunopositive group (12.85 ± 2.94 vs 9.69 ± 4.7 years; P = 0.045). However, no significant differences were identified when comparing the rest of the JIA characteristics. Conclusion Evaluation of the immunological profile is required in JIA. Our study found associations of RF and/or ACPA positivity with age at disease onset, female gender, osteoporosis, and growth delay in JIA.

A Review of Community-Oriented Program for Control of Rheumatic Diseases Studies from India

Abstract Background: Community-Oriented Program for Control of Rheumatic Diseases (COPCORD) is a global community-based program, emphasizing prevalence data. In India, it has been headed by the World Health Organization (WHO), the International League of Associations for Rheumatology (ILAR), and the Bone and Joint Decade (BJD), India. The Indian Council of Medical Research (ICMR) also utilized the COPCORD methodology. This study aimed to review Indian COPCORD data. Methods: PubMed, COPCORD website, conference proceedings, and citations were searched from inception to 2022. Search terms included WHO-ILAR, COPCORD, and India. Studies conducted in India and available in English were included. Abstracts from journal supplements or conference proceedings were included if they studied the Indian population. Results: Bhigwan, Pune, Bikaner, Lucknow, Calicut, and Trivandrum studies were published as full-length articles. Soft-tissue rheumatism showed a higher prevalence than most diseases everywhere. The appraisal checklist found a few lacunae in the description of the study setting and statistical analysis clarity. Bhigwan had a high prevalence of “ill-defined diagnosis” (6.25%). Osteoarthritis (OA) knee had a prevalence of 10.6% in Lucknow urban. RA prevalence was 2.54% in Bikaner. Pune exhibited the highest OA prevalence (6.25%). Age and gender were important risk factors in the Calicut study. Trivandrum found that functional impairment impacted workability and finances. Malappuram reported higher rheumatic musculoskeletal disease (RMSD) in women (70%). Conclusion: COPCORD studies were the first to highlight the burden of RMSD in India. Nonclinical aspects such as architecture and living conditions have been described. Further research regarding functional ability, work disability, and psychosocial factors is recommended. Future scope to look at economic aspects and environmental risk factors remains.

Developing a framework for the implementation of recommendations for lifestyle factors for people with RMDs across Europe: assessment of current materials and implementation needs

Lifestyle factors (such as diet, physical activity or smoking habits, among others) are known to influence the progression of rheumatic and musculoskeletal diseases (RMDs). Despite contemporary improvements in RMD care,...

Application of EULAR and European Society of Cardiology recommendations with regard to blood pressure and lipid management in antiphospholipid syndrome

BackgroundTo examine blood pressure (BP) and lipid treatment eligibility in antiphospholipid syndrome (APS) according to European Alliance of Associations for Rheumatology (EULAR) and European Society of Cardiology (ESC) recommendations.MethodsSystematic Coronary...

Choosing Wisely: The Canadian Rheumatology Association Pediatric Committee's List of Items Physicians and Patients Should Question.

To develop a list of tests or treatments frequently used in pediatric rheumatology practice which may be unnecessary based on existing evidence. A Choosing Wisely (CW) Working Group composed of 16 pediatric rheumatologists, one allied health practitioner, one parent, and one patient used the Delphi method to generate, rank, and refine a list of tests and treatments that may be unnecessary or harmful. The items with the highest content agreement and perceived impact were presented in a survey to all Canadian Rheumatology Association (CRA) physicians who practice pediatric rheumatology. Respondents were asked to rate their agreement, impact, and rank the items. Five items with the highest composite scores and two additional items selected by the CW Working Group were put forward for literature review. The initial Delphi procedure generated 80 items. After three rounds, the list was narrowed to 13 items. The survey was completed by 41/81 (51%) CRA pediatric members across Canada. Respondent characteristics were similar to the CRA pediatric membership for gender, geographical location, and career stage. The highest composite score items were anti-nuclear antibody testing, drug toxicity monitoring, human leukocyte antigen-B27 testing, rheumatoid factor/anti-cyclic citrullinated peptide testing, and Lyme serology testing. Two additional items (numerous or repeated intra-articular corticosteroid injections, and autoinflammatory diseases genetic testing) were also selected. Literature review was performed for these seven highest priority items. We have identified areas for quality improvement in the evaluation and treatment of rheumatic diseases in Canadian children.

Omicron variant infection in inflammatory rheumatological conditions – outcomes from a COVID-19 naive population in Aotearoa New Zealand

Due to geographic isolation and border controls Aotearoa New Zealand (AoNZ) attained high levels of population coronavirus disease-19 (COVID-19) vaccination before widespread transmission of COVID-19. We describe outcomes of SARS-CoV-2 infection (Omicron variant) in people with inflammatory rheumatic diseases in this unique setting. This observational study included people with inflammatory rheumatic disease and SARS-CoV-2 infection in AoNZ between 1 February and 30 April 2022. Data were collected via the Global Rheumatology Alliance Registry including demographic and rheumatic disease characteristics, and COVID-19 vaccination status and outcomes. Multivariable logistic regression was used to explore associations of demographic and clinical factors with COVID-19 hospitalisation and death. Of the 1599 cases included, 96% were from three hospitals that systematically identified people with inflammatory rheumatic disease and COVID-19. At time of COVID-19, 1513 cases (94.6%) had received at least two COVID-19 vaccinations. Hospitalisation occurred for 104 (6.5%) cases and 10 (0.6%) patients died. Lower frequency of hospitalisation was seen in cases who had received at least two vaccinations (5.9%), compared to the unvaccinated (20.6%) or those with a single vaccine dose (10.7%). In multivariable adjusted models, people with gout or connective tissue diseases (CTD) had increased risk of the combined outcome of hospitalisation/death, compared to people with inflammatory arthritis. Glucocorticoid and rituximab use were associated with increased rates of hospitalisation/death. All patients who died had three or more co-morbidities or were over 60 years old. In this cohort with inflammatory rheumatic diseases and high vaccination rates, severe outcomes from SARS-CoV-2 Omicron variant were relatively infrequent. The outcome of Omicron variant infection among vaccinated but SARS-CoV-2 infection-naive people with inflammatory rheumatic disease without other known risk factors were favourable. Financial support from the American College of Rheumatology (ACR) and European Alliance of Associations for Rheumatology (EULAR) included management of COVID-19 Global Rheumatology Alliance funds.

EULAR 2023 Abstract Highlights

The following selected highlights spotlight several interesting and timely abstracts presented at the European Alliance of Associations for Rheumatology (EULAR) Congress 2023, covering topics such as recurring joint inflammation in juvenile idiopathic arthritis, systemic lupus erythematosus, and the importance of a healthy lifestyle in patients with osteoarthritis.

European Alliance of Associations for Rheumatology (EULAR) Congress 2023: Take-Home Messages from an Excellent Congress

This excellent congress took place between 31st May–3rd June 2023 in the beautiful city of Milan, Italy, featuring the stunning church of Duomo di Milano in the centre of the city. The congress covered all topics of Rheumatology. During the opening plenary session, Annamaria Iagnocco, European Alliance of Associations for Rheumatology (EULAR) President, presented EULAR’s new strategy and its key priorities.

Response to Biologic Drugs in Patients With Rheumatoid Arthritis and Antidrug Antibodies

There are conflicting data on the association of antidrug antibodies with response to biologic disease-modifying antirheumatic drugs (bDMARDs) in rheumatoid arthritis (RA). To analyze the association of antidrug antibodies with response to treatment for RA. This cohort study analyzed data from the ABI-RA (Anti-Biopharmaceutical Immunization: Prediction and Analysis of Clinical Relevance to Minimize the Risk of Immunization in Rheumatoid Arthritis Patients) multicentric, open, prospective study of patients with RA from 27 recruiting centers in 4 European countries (France, Italy, the Netherlands, and the UK). Eligible patients were 18 years or older, had RA diagnosis, and were initiating a new bDMARD. Recruitment spanned from March 3, 2014, to June 21, 2016. The study was completed in June 2018, and data were analyzed in June 2022. Patients were treated with a new bDMARD: adalimumab, infliximab (grouped as anti-tumor necrosis factor [TNF] monoclonal antibodies [mAbs]), etanercept, tocilizumab, and rituximab according to the choice of the treating physician. The primary outcome was the association of antidrug antibody positivity with EULAR (European Alliance of Associations for Rheumatology; formerly, European League Against Rheumatism) response to treatment at month 12 assessed through univariate logistic regression. The secondary end points were the EULAR response at month 6 and at visits from month 6 to months 15 to 18 using generalized estimating equation models. Detection of antidrug antibody serum levels was performed at months 1, 3, 6, 12, and 15 to 18 using electrochemiluminescence (Meso Scale Discovery) and drug concentration for anti-TNF mAbs, and etanercept in the serum was measured using enzyme-linked immunosorbent assay. Of the 254 patients recruited, 230 (mean [SD] age, 54.3 [13.7] years; 177 females [77.0%]) were analyzed. At month 12, antidrug antibody positivity was 38.2% in patients who were treated with anti-TNF mAbs, 6.1% with etanercept, 50.0% with rituximab, and 20.0% with tocilizumab. There was an inverse association between antidrug antibody positivity (odds ratio [OR], 0.19; 95% CI, 0.09-0.38; P < .001) directed against all biologic drugs and EULAR response at month 12. Analyzing all the visits starting at month 6 using generalized estimating equation models confirmed the inverse association between antidrug antibody positivity and EULAR response (OR, 0.35; 95% CI, 0.18-0.65; P < .001). A similar association was found for tocilizumab alone (OR, 0.18; 95% CI, 0.04-0.83; P = .03). In the multivariable analysis, antidrug antibodies, body mass index, and rheumatoid factor were independently inversely associated with response to treatment. There was a significantly higher drug concentration of anti-TNF mAbs in patients with antidrug antibody-negative vs antidrug antibody-positive status (mean difference, -9.6 [95% CI, -12.4 to -6.9] mg/L; P < 001). Drug concentrations of etanercept (mean difference, 0.70 [95% CI, 0.2-1.2] mg/L; P = .005) and adalimumab (mean difference, 1.8 [95% CI, 0.4-3.2] mg/L; P = .01) were lower in nonresponders vs responders. Methotrexate comedication at baseline was inversely associated with antidrug antibodies (OR, 0.50; 95% CI, 0.25-1.00; P = .05). Results of this prospective cohort study suggest an association between antidrug antibodies and nonresponse to bDMARDs in patients with RA. Monitoring antidrug antibodies could be considered in the treatment of these patients, particularly nonresponders to biologic RA drugs.

Rheumatology congresses post COVID-19: we cannot regress.

Rheumatology congresses post COVID-19: we cannot regress.

Trajectories of self-reported pain-related health outcomes and longitudinal effects on medication use in rheumatoid arthritis: a prospective cohort analysis using the Australian Rheumatology Association Database (ARAD)

ObjectiveTo determine distinct trajectories of self-reported pain-related health status in rheumatoid arthritis (RA), their relationship with sociodemographic factors and medication use.Methods988 Australian Rheumatology Association Database participants with RA (71% female,...

Recommendations for diagnosis and treatment of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with complicated pathogenesis and diverse clinical manifestations. The current recommendations of the Chinese Rheumatology Association are based on a comprehensive investigation of evidence based medicine, domestic and international guidelines for SLE, and experts' proposals, and aim to provide a more scientific and authoritative reference for the diagnosis and management of SLE. The recommendations focus on four aspects; clinical manifestations, laboratory evaluation, diagnosis and disease assessment, and disease treatment and monitoring. The goal of the recommendations is to standardize the diagnosis and treatment of SLE in China so as to improve the prognosis of SLE patients.

Difficult-to-Treat Disease in Rheumatology

THE CHALLENGE of difficult-to-treat disease in rheumatology was explored during an insightful clinical science session at the European Alliance of Associations for Rheumatology (EULAR) 2023 Congress, which took place in Milan, Italy, between the 31st of May–3rd of June. The session, entitled ‘Everything is difficult to treat?’, explored key elements that contribute to the challenge in treating rheumatoid arthritis (RA), axial spondyloarthritis (axSpA), systemic lupus erythematosus (SLE), and systemic sclerosis (SSc).

Precision Medicine in Systemic Lupus Erythematosus

A session on precision medicine in systemic lupus erythematosus (SLE) took place at the European Alliance of Associations for Rheumatology (EULAR) 2023 meeting in Milan, Italy, merging one of the most contemporary topics in modern healthcare with an important subject in the rheumatology specialty. Co-chaired by Dimitrios Boumpas, University of Athens, Greece, and José Pego-Reigosa, University Hospital Complex of Vigo, Spain, this series of presentations was among the most highly attended and featured cutting-edge insights from front-running experts on lupus.

Efficacy of inclusion of vitamin U and provitamin B5 combination in the complex therapy of primary osteoarthritis against the background of gastrogenic exocrine pancreatic insufficiency

Clinical comorbidities at osteoarthritis (OA) complicate its treatment. The number and nature of various comorbid conditions determine the severity of the condition in patients with multimorbidity. However, apart from the common risk factors such as aging and obesity, little is known about the biological plausibility of explaining the co‑occurrence of OA and comorbidities. According to the European Alliance of Associations for Rheumatology, EULAR, and the National Institute of Health and Nursing, the diagnosis and treatment of specific comorbidities, as well as understanding their nature in OA, are important for choosing the best treatment tactics.&#x0D; Objective — to investigate efficacy of inclusion of vitamin U and provitamin B5 combination in the complex therapy of patients with primary OA in comorbidity with gastrogenic exocrine pancreatic insufficiency (EPI) for the correction of clinical syndromes.&#x0D; Materials and methods. Examinations involved 61 patients with primary OA in comorbidity with gastrointestinal diseases, accompanied by EPI of gastrogenic genesis without exacerbation. The comparison group consisted of 30 practically healthy people. All patients were divided into two comparable groups: group 1 (n=30) received a course of basic therapy drugs, namely: nonsteroidal anti‑inflammatory drugs, chondroprotectors and/or chondrostimulators, antispasmodics, prokinetics, proton pump inhibitors, enzyme agents (pancreatin); group 2 (n=31), in addition to the course of basic therapy, received a course of the vitamin drug combination of vitamin U and provitamin B5, 2 tablets once a day for 6 weeks. The calculation of the WOMAC index (Western Ontario and McMaster University) was used to assess the articular status of patients with primary OA, as well as the effectiveness of the treatment measures. Gastrointestinal Symptom Rating Scale (GSRS) questionnaire was used to assess gastrointestinal symptoms.&#x0D; Results. The proposed complex treatment of patients with primary OA with gastrogenic EPI with the additional inclusion of a vitamin preparation combination of vitamin U and provitamin B5 contributed to more significant regression of WOMAC indices compared to that during protocol treatment (the total WOMAC index decreased by 16.03% against 9.10%, respectively) and indicators of the scales of the GSRS questionnaire in comparison with the subgroup that received treatment according to the protocol (in total — by 35.96% vs. 22.19%, p &lt;0.05),&#x0D; Conclusions. The study results showed the advisability of using a vitamin medication, a combination of vitamin U and provitamin B5. in the treatment of patients with primary OA with gastrogenic EPI.

Comparison Between Early-Onset and Common Gout: A Systematic Literature Review.

Gout is an inflammatory, metabolic disease associated with a high comorbidity burden including cardiovascular disease, hypertension, type 2 diabetes, hyperlipidemia, renal disease, and metabolic syndrome. Approximately 9.2 million Americans have gout, making prognosis and treatment outcome predictors highly important. About 600,000 Americans have early-onset gout (EOG), generally defined as first gout attack at ≤ 40years of age. However, data on EOG clinical features, comorbidity profile, and treatment response are sparse; this systematic literature review provides insight. PubMed and American College of Rheumatology (ACR)/European Alliance of the Associations for Rheumatology (EULAR) abstract archives were searched for early-onset gout, "early onset gout," and ("gout" AND "age of onset"). Duplicate, foreign language, single case report, older (before 2016), and irrelevant/data insufficient publications were excluded. The age of diagnosis categorized patients as having common gout (CG, generally > 40years) or EOG (generally ≤ 40years). Applicable publications were extensively reviewed/discussed among authors for inclusion/exclusion consensus. A total of 283 publications were identified, with 46 (35 articles, 10 abstracts) reviewed and 17 (12 articles,5 abstracts) ultimately included. Eleven reported clinical characteristics, with 6 EOG-CG retrospective/cross-sectional comparisons. Gout diagnosis preceded cardiometabolic comorbidity and renal comorbidities were less prevalent in EOG than CG patients. EOG patients had more severe disease (more gout flares, polyarticular disease), higher pre-therapy serum urate (SU), and worse oral urate-lowering therapy response. Genetics-focused publications reported higher incidences of dysfunctional urate transporter mutations in EOG patients. This review suggests that EOG is more recalcitrant to urate-lowering therapy, is associated with urate transporter defects, and carries heavy disease burden. Therefore, early rheumatology referral and urate-lowering in a treat-to-target fashion may benefit EOG patients. Interestingly, EOG patients had fewer cardiometabolic comorbidities at diagnosis than CG patients, presenting a potential "window of opportunity" to attenuate cardiometabolic comorbidity development with SU control. Preventing gout-related suffering and health burden is particularly important in these young EOG patients who will live with gout and its sequelae for decades.

#4323 A RARE CASE OF MEMBRANOUS NEPHROPATHY AS THE INITIAL PRESENTATION OF SJöGREN SYNDROME

Abstract Introduction Primary Sjögren's syndrome (pSS) is an autoimmune disorder characterized by lymphocytic infiltrates in exocrine glands, most commonly manifesting as dry eyes and mouth. Even though extra glandular involvement can occur in Sjögren, renal involvement is rare. It affects less than 10% of the patient population, with tubulointerstitial nephritis being the usual presentation. Glomerular involvement and, in particular, membranous nephropathy are not commonly encountered. We describe a rare case of membranous nephropathy as the initial presentation of Primary Sjögren's syndrome. Case history A 21-year-old female with a history of sickle cell trait presented to our emergency department with one week history of anasarca. Vital signs were unremarkable, and the physical exam was significant for a 2+ pitting edema of bilateral upper and lower extremities. Initial investigation revealed acute kidney injury (Creatinine 2.9 mg/dl from normal baseline), nephrotic range proteinuria of 6 grams on 24-hour urine collection, and hypoalbuminemia (serum albumin 1.5 mg/dl). Immunological workup showed positive ANA (speckled), strongly positive anti-SSA, borderline elevated anti-SSB, negative anti-PLA2R, negative cryoglobulins, negative anti-dsDNA, and negative anti-Smith antibody (Figure 1). Renal biopsy (Figure 2) revealed membranous nephropathy with acute tubular necrosis (ATN). PLA2R staining was negative. We attributed acute kidney injury to ATN and proteinuria to membranous nephropathy. Negative serologies and renal biopsy findings ruled out systemic Lupus. The patient was therefore diagnosed with membranous nephropathy secondary to pSS. The patient was started on Mycophenolate mofetil and steroid taper with an improvement of proteinuria and anasarca and currently follows at our clinic. Discussion Glomerular involvement is rare, and when involved, it is membranoproliferative glomerulonephritis (MPGN) associated with B-cell activation and positive cryoglobulins. Membranous nephropathy is rare with primary Sjögren syndrome. In our patient, a negative anti-PLA2R goes against primary membranous nephropathy. A negative anti-dsDNA and anti-Smith and biopsy findings essentially rule out Lupus nephritis, leaving Sjögren induced secondary membranous nephropathy as the most likely diagnosis. A literature search revealed few described cases of membranous nephropathy as the presenting feature of pSS with later development of sicca symptoms and progression of renal disease to MPGN. Interestingly, our patient had no sicca symptoms at the presentation time. Occult salivary gland involvement has been described in some of these patients; however, our patient deferred salivary gland biopsy. The management of renal disease in pSS has yet to be well described because of its rarity. Rituximab has shown the most benefit in patients with pSS who have positive cryoglobulins and associated MPGN. Steroids and mycophenolate mofetil (MMF) are other possible agents that can be tried. Given that experience, we started on steroids and MMF with an excellent response to treatment indicated by improvement in proteinuria. Conclusion This case adds to the limited available literature on membranous nephropathy as the initial manifestation of pSS. Although the European Alliance of Associations for Rheumatology (EULAR) criteria strongly emphasizes sicca symptoms and glandular involvement in pSS, the possibility of a small subset of pSS patients who present with early renal involvement with latent glandular findings needs to be recognized.

Solid validity evidence for two tools assessing competences in musculoskeletal ultrasound: a validity study.

Musculoskeletal ultrasound (MSUS) is increasingly used by rheumatologists in daily clinical practice. However, MSUS is only valuable in trained hands, and assessment of trainee competences is therefore essential before independent practice. Thus, this study aimed to establish validity evidence for the EULAR and the Objective Structured Assessment of Ultrasound Skills (OSAUS) tools used for assessing MSUS competences. Thirty physicians with different levels of MSUS experience (novices, intermediates, and experienced) performed four MSUS examinations of different joint areas on the same rheumatoid arthritis patient. All examinations were video recorded (n = 120), anonymized, and subsequently assessed in random order by two blinded raters using first the OSAUS assessment tool followed by the EULAR tool 1 month after. The inter-rater reliability between the two raters was high for both the OSAUS and EULAR tools, with a Pearson correlation coefficient (PCC) of 0.807 and 0.848, respectively. Both tools demonstrated excellent inter-case reliability, with a Cronbach's alpha of 0.970 for OSAUS and 0.964 for EULAR. Furthermore, there was a strong linear correlation between the OSAUS and the EULAR performance scores and the participants' experience levels (R2 = 0.897 and R2 = 0.868, respectively) and a significant discrimination between different MSUS experience levels (P < 0.001 for both). MSUS operator competences can be assessed reliably and valid using either the OSAUS or the EULAR assessment tool, thereby allowing a uniform competency-based MSUS education in the future. Although both tools demonstrated high inter-rater reliability, the EULAR tool was superior to OSAUS. ClinicalTrials.gov, http://clinicaltrials.gov, NCT05256355.

Hughes-Stovin syndrome: alife-threatening manifestation of Behçet's syndrome

Hughes-Stovin syndrome (HSS) is asystemic inflammatory condition of unknown origin that is considered to be part of the Behçet's syndrome (BS) spectrum. Recurrent venous thrombosis and superficial thrombophlebitis in combination with bilateral pulmonary artery aneurysms (PAA) represent the hallmark of HSS. The diagnostic evaluation includes computed tomography pulmonary angiography to detect signs of pulmonary vasculitis. The management of HSS is based on the European Alliance of Associations for Rheumatology (EULAR) recommendations for BS and mainly comprises immunosuppressive therapy with glucocorticoids and cyclophosphamide. In addition to drug therapy, PAA should be evaluated for interventional treatment. Spontaneous PAA rupture due to fragile vessel architecture can occur even in cases of remission and/or PAA regression.

Evaluation ofMinistry ofHealth, Labour andWelfare diagnostic criteria forantineutrophil cytoplasmic antibody-associated vasculitis compared to ACR/EULAR 2022 classification criteria.

This study aimed to evaluate the Ministry of Health, Labour and Welfare (MHLW) diagnostic criteria for antineutrophil cytoplasmic antibody-associated vasculitis compared to the new American College of Rheumatology/European Alliance of Associations for Rheumatology 2022 criteria. Two nationwide cohort studies were used, and participants were categorised as having eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis (GPA), or microscopic polyangiitis (MPA) according to the American College of Rheumatology/European Alliance of Associations for Rheumatology 2022 and MHLW criteria. Of the entire patient population, only 10 (2.1%) were unclassifiable according to the MHLW probable criteria, while a significant number of patients (71.3%) met at least two criteria. The MHLW probable criteria for MPA had some challenges in differentiating between MPA and eosinophilic granulomatosis with polyangiitis, and the same was true for MHLW probable criteria for GPA in differentiating MPA from GPA. Nevertheless, improved classification results were obtained when the MHLW probable criteria were applied in the order of eosinophilic granulomatosis with polyangiitis, MPA, and GPA. The application of MHLW criteria could categorise a substantial number of patients with antineutrophil cytoplasmic antibody-associated vasculitis into one of the three antineutrophil cytoplasmic antibody-associated vasculitis diseases. The classification was in accordance with the American College of Rheumatology/European Alliance of Associations for Rheumatology 2022 criteria when considering the order of application.