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Overlap Syndrome Consisting of Polymyositis/Dermatomyositis and ANCA-Associated Vasculitis According to the 2022 ACR/EULAR Criteria for Vasculitis: A Korean Single-Centre Study.

ABSTRACT: The present study applied the 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology classification criteria (the 2022 ACR/EULAR criteria) for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to ANCA-positive patients with polymyositis (PM)/dermatomyositis (DM). Also, this study investigated how many patients could be diagnosed with overlap syndrome consisting of PM/DM and AAV. Twelve ANCA-positive patients with PM/DM were included and analysed in this study. The 2022 ACR/EULAR classification criteria for microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic GPA (EGPA) are scoring systems, and when a total score is over five in cases of MPA and GPA and over six in cases of EGPA, AAV can be classified. The median age of 12 ANCA-positive patients (six with PM and six with DM) was 54.0 years and one patient (8.3%) was male. Of the 12 ANCA-positive patients with PM/DM, 11 had myeloperoxidase (MPO)-ANCA (or perinuclear [P]-ANCA) and the remaining one had proteinase 3 (PR3)-ANCA (or cytoplasmic [C]-ANCA). Nine (75.5%) and one (8.3%) ANCA-positive patients with PM/DM were diagnosed with overlap syndrome consisting of PM/DM and MPA and overlap syndrome consisting of PM/DM and GPA, respectively. The main contributors to the classification of MPA and GPA were interstitial lung disease and renal vasculitis, along with ANCA positivity, respectively. In conclusion, this study was the first to demonstrate that 83.3% of them could be diagnosed with overlap syndrome consisting of PM/DM and AAV according to the 2022 ACR/EULAR criteria for AAV.

SUBMITTER: Ha JW

PROVIDER: S-EPMC10647414 | biostudies-literature | 2023 Oct

REPOSITORIES: biostudies-literature

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Overlap Syndrome Consisting of Polymyositis/Dermatomyositis and ANCA-Associated Vasculitis According to the 2022 ACR/EULAR Criteria for Vasculitis: A Korean Single-Centre Study.

Ha Jang Woo JW Park Yong-Beom YB Lee Sang-Won SW

Journal of clinical medicine 20231025 21

The present study applied the 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology classification criteria (the 2022 ACR/EULAR criteria) for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to ANCA-positive patients with polymyositis (PM)/dermatomyositis (DM). Also, this study investigated how many patients could be diagnosed with overlap syndrome consisting of PM/DM and AAV. Twelve ANCA-positive patients with PM/DM were included and ...[more]

PMID: 37959218

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Project description:ObjectiveTo evaluate the ability to discriminate giant cell arteritis (GCA) from Takayasu arteritis (TAK) according to the modified 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) GCA classification criteria.MethodsPatients enrolled in the Japanese nationwide retrospective registry were evaluated using the criteria with partial modification; wall thickening of descending thoracic-abdominal aorta were mainly diagnosed by contrast-enhanced computed tomography (CT) or magnetic resonance imaging instead of evaluating with positron emission tomography (PET)-CT. The discriminability of the criteria was evaluated using C-statistic (> 0.7: good ability).ResultsNewly diagnosed patients with GCA (n = 139) and TAK (n = 129) were assessed, and 23.3% of TAK were aged 50 years or older at onset. The sensitivity of the modified 2022 ACR/EULAR GCA classification criteria with a score ≥ 6 was 82.0%, 68.5%, and 32.1% in all GCA, GCA with large-vessel involvement, and GCA without cranial arteritis, respectively. The specificity of the modified criteria was 96.1% for the 129 TAK as controls. Five patients with late-onset TAK met the modified criteria, and four had cranial signs and symptoms, two had bilateral axillary artery involvement, and four had descending thoracic-abdominal aorta involvement. The discriminability of the criteria was good (C-statistic: 0.986, 95% confidence interval [CI]: 0.976-0.996) and remained good after excluding age (C-statistic: 0.927, 95% CI: 0.894-0.961). The discriminability of a set of large-vessel lesions (bilateral axillary artery and descending thoracic-abdominal aorta) and inflammatory markers was markedly decreased with poor C-statistic value (C-statistic: 0.598, 95% CI: 0.530-0.667). Discriminability was improved after adding polymyalgia rheumatica (PMR) (C-statistic: 0.757, 95% CI: 0.700-0.813) or age (C-statistic: 0.913, 95%CI: 0.874-0.951) to the set of large-vessel lesions. In GCA patients with a score ≤ 5, 52% had bilateral subclavian and/or axillary artery involvement.ConclusionThe modified 2022 ACR/EULAR GCA classification criteria well performed in classifying GCA and TAK without PET-CT in routine clinical practice. A set of items included in the modified GCA classification criteria had good discriminative ability for GCA and TAK, even when age was excluded. However, age restriction or PMR was required to distinguish GCA without cranial lesions from TAK.

Project description:Background/Objectives: ACR/EULAR has recently developed new classification criteria for antiphospholipid syndrome (APS). The present study aims to analyze the impact of these new 2023 ACR/EULAR classification criteria in a cohort of pregnant women with primary APS. Methods: Retrospective cohort study of 93 consecutive pregnant women attending the Autoimmune Diseases Pregnancy Clinic, a multidisciplinary unit of a tertiary care teaching hospital, between 2005 and 2023. All of them fulfilled the Sydney classification criteria for APS. Women diagnosed with rheumatic autoimmune diseases other than APS were excluded. Results: Twenty-four out of ninety-three patients (25.8%) met the 2023 ACR/EULAR criteria for APS. Patients who met the new classification criteria were very similar to those who did not, except for being younger (p < 0.001), and had a lower number of clinical pregnancies (p = 0.004). The obstetric domain was clearly underrepresented in women who fulfilled the 2023 ACR/EULAR criteria (p < 0.001). Patients meeting the new classification criteria were primarily characterized by preterm births before 34 weeks due to severe placentation disorders (p = 0.004). Women with early and late fetal loss were significantly underrepresented (p < 0.0001 and 0.03, respectively). Nearly half of these patients had thrombocytopenia (p < 0.001). Serologically, these patients showed a higher frequency of persistent lupus anticoagulant (p = 0.02) and a lower frequency of IgM isotype antiphospholipid antibodies (p = 0.05). Conclusions: Almost three-quarters of the patients included in the study did not meet the 2023 ACR/EULAR criteria. Most patients who could not be classified according to these new classification criteria were those with early and/or late fetal deaths, as well as patients carrying only IgM aCL/AB2GPI antibodies. The high specificity of the 2023 ACR/EULAR criteria, restricted to severe placentation disorders, may leave the majority of patients with obstetric APS out of the new classification criteria.

Dataset Information

Overlap Syndrome Consisting of Polymyositis/Dermatomyositis and ANCA-Associated Vasculitis According to the 2022 ACR/EULAR Criteria for Vasculitis: A Korean Single-Centre Study.

Publications

Overlap Syndrome Consisting of Polymyositis/Dermatomyositis and ANCA-Associated Vasculitis According to the 2022 ACR/EULAR Criteria for Vasculitis: A Korean Single-Centre Study.

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