2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis

Grayson, Peter C.; Ponte, Cristina; Suppiah, Ravi; Robson, Joanna; Craven, Anthea; Judge, Andrew; Khalid, Sara; Hutchings, Andrew; Luqmani, Raashid; Watts, Richard A.; Merkel, Peter A.

doi:10.1136/annrheumdis-2021-221794

Cited by 251 publications

(192 citation statements)

References 11 publications

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“…Diagnosis of EGPA was supported by a cumulative score of ≥ six points. When these criteria were tested in the validation dataset, the sensitivity was 85% (95% CI: 77-91%) and the specificity was 99% (95% CI: 98-100%) [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

A Surgical Presentation of Churg-Strauss Syndrome

Vasandani¹,

Isaac²,

Bajwa³

et al. 2022

Cureus

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Eosinophilic granulomatosis with polyangiitis (EGPA) or Churg-Strauss syndrome (CSS) is a rare, autoimmune vasculitis usually affecting small and medium-sized blood vessels in its later phases. It is a diffuse, systemic, multisystem disease that is reported to present with gastrointestinal manifestations but very rarely as an acute abdomen secondary to eosinophilic peritonitis. A 28-year-old relatively healthy male with a pre-existing diagnosis of inactive pulmonary sarcoidosis presented to the emergency department with an acute abdomen. After an exploratory laparotomy, multi-specialty involvement, and extensive investigations to exclude other differentials, a diagnosis of EGPA was made. The patient was treated with systemic glucocorticoids initially, followed by a tapering course of steroids and anti-interleukin 5 monoclonal antibodies as maintenance upon remission. EGPA can manifest in a myriad of ways including an acute abdomen, and medical treatment is useful in managing this presentation. Surgeons should be aware of the atypical causes of acute abdomen and should routinely broaden their differential diagnosis to include medical pathologies.

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Section: Introductionmentioning

confidence: 99%

A Surgical Presentation of Churg-Strauss Syndrome

Vasandani¹,

Isaac²,

Bajwa³

et al. 2022

Cureus

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“…Recently, the newly revised classification criteria of AAV have incorporated the MPO-ANCA and PR3-ANCA into the system for the first time, highlighting the significance of specific ANCA ( 28 – 30 ). Positive cANCA or PR3-ANCA in GPA and pANCA or MPO-ANCA in MPA are weighted largely in the new criteria, because these antibodies are very commonly seen in GPA or MPA.…”

Section: Discussionmentioning

confidence: 99%

Clinical Significance of MPO-ANCA in Eosinophilic Granulomatosis With Polyangiitis: Experience From a Longitudinal Chinese Cohort

et al. 2022

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ObjectivesThe aim of this study is to investigate the clinical significance of myeloperoxidase (MPO)–antineutrophil cytoplasmic antibody (ANCA) on eosinophilic granulomatosis with polyangiitis (EGPA) from a longitudinal Chinese cohort.MethodsA total of 120 patients with EGPA were consecutively enrolled and followed up. Two patients with PR3 ANCA was excluded and our analysis focused on the 118 patients with EGPA. On the basis of MPO-ANCA status, baseline clinical manifestations, treatment, and outcomes were analyzed. Logistic regression analysis was performed to analyze the independently associated factors for renal involvement.ResultsANCA positivity was observed in 24.2% of patients with EGPA. Patients with MPO-ANCA accounted for 20.8%. Patients with positive MPO-ANCA had higher levels of erythrocyte sedimentation rate (ESR), C-reactive protein, Birmingham Vasculitis Activity Score (BVAS), higher ratios of fever, myalgia, renal involvement, and biopsy-proven vasculitis. Heart manifestations and asthma were more common in patients with negative ANCA. Baseline MPO-ANCA titers positively correlated with ESR, eosinophil count, and BVAS and were higher in patients with methylprednisolone pulse. Among patients with renal involvement, patients with positive MPO-ANCA had higher proportions of female, fever, biopsy-proven vasculitis, and faster ESR; patients with negative ANCA developed more skin and cardiac involvement. MPO-ANCA positivity, male, and ear involvement were the independent factors associated with renal involvement. Intravenous cyclophosphamide and immunoglobulins were prescribed more frequently in patients with positive MPO-ANCA.ConclusionIn this cohort, patients with positive MPO-ANCA and negative ANCA displayed distinct clinical features, suggesting that MPO-ANCA might be a valuable biomarker for EGPA stratification. Baseline MPO-ANCA level correlated positively with disease activity of EGPA. MPO-ANCA was a significant independent factor associated with renal involvement.

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“…On top of it, the recent classification included patients with MPA, which was not the case with the previous one. Another advantage is the shifting toward weighted criteria which enables a more discriminative classification (75)(76)(77).…”

Section: Classification Criteriamentioning

confidence: 99%

Diagnostic and Therapeutic Approach in ANCA-Associated Glomerulonephritis: A Review on Management Strategies

2022

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Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a destructive small vessel vasculitis affecting multiple organs. Renal involvement often leads to end-stage renal disease and increases mortality. Prompt diagnosis and initiation of adequate immunosuppressive therapy are critical for the best patient and kidney outcomes. However, considerable heterogeneity in symptoms and severity across the patients frequently hinder the diagnosis and management. The objective of this review is to emphasize the heterogeneity of the ANCA-associated vasculitis, facilitate the recognition and give guidance to the therapeutical possibilities. We present epidemiologic and risk factors, pathogenesis, and provide comprehensive clinical features of the disease. This article also focuses on the currently available therapeutic options and emerging cellular and molecular targets for the management of systemic and especially renal disease. We conducted extensive literature research published on PubMed and Google Scholar. We systematically reviewed, analyzed, and assembled databases, covering a broad spectrum of aspects of the disease. We compared and summarized the recommendations of two recent guidelines on ANCA-associated vasculitis. The incidence of ANCA-associated vasculitis, hence glomerulonephritis shows a steady increase. Familiarity with the presenting symptoms and laboratory abnormalities are necessary for rapid diagnosis. Early initiation of treatment is the key aspect for favorable patient and renal outcomes. A better understanding of the pathogenesis constantly leads to more targeted and therefore more efficient and less toxic treatment.

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2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis

Cited by 251 publications

References 11 publications

A Surgical Presentation of Churg-Strauss Syndrome

A Surgical Presentation of Churg-Strauss Syndrome

Clinical Significance of MPO-ANCA in Eosinophilic Granulomatosis With Polyangiitis: Experience From a Longitudinal Chinese Cohort

Diagnostic and Therapeutic Approach in ANCA-Associated Glomerulonephritis: A Review on Management Strategies

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