238. efficacy of Conventional Immunosuppressants in Steroid-Dependent or Refractory Eosinophilic Granulomatosis With Polyangiitis: Study on a New Canadian Patient Cohort

Doubelt, Irena; Pulenzas, Natalie; Carette, Simon; Pagnoux, Christian

doi:10.1093/rheumatology/kez062.012

Cited by 8 publications

(10 citation statements)

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“…Within the EGPA subset at 2 and 5 years, relapse-free survival did not differ between groups (46). Although there is no evidence that adding immunosuppressants to initial induction is superior to GC alone, conventional immunosuppressants are often justified if vasculitic disease manifestations, such as mononeuritis multiplex, progress (47). Until further data is available, any of the conventional immunosuppressants (AZA, MTX, LEF, MMF, or even CYC in some cases) should be promptly added in patients with progressive vasculitic manifestations of EGPA for whom the FFS remains 0.…”

Section: Patients With Non-severe Egpa Without Major Organ Involvemen...mentioning

confidence: 98%

CanVasc Consensus Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitis: 2020 Update

Mendel¹,

Ennis²,

Go³

et al. 2020

J Rheumatol

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Objective In 2015, the Canadian Vasculitis Research Network (CanVasc) created recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) in Canada. The current update aimed to revise existing recommendations and create additional recommendations, as needed, based on a review of new available evidence. Methods A needs assessment survey of CanVasc members informed questions for an updated systematic literature review (publications spanning May 2014-September 2019) using Medline, Embase, and Cochrane. New and revised recommendations were developed and categorized according to the level of evidence and strength of each recommendation. The CanVasc working group used a two-step modified Delphi procedure to reach >80% consensus on the inclusion, wording and grading of each new and revised recommendation. Results Eleven new and 16 revised recommendations were created, and 12 original (2015) recommendations were retained. New and revised recommendations are discussed in detail within this document. Five original recommendations were removed, of which 4 were incorporated into the explanatory text. The supplementary appendix for practical use was revised to reflect the updated recommendations. Conclusion The 2020 updated recommendations provide rheumatologists, nephrologists, and other specialists caring for patients with AAV in Canada with new management guidance, based on current evidence and consensus from Canadian experts.

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Section: Patients With Non-severe Egpa Without Major Organ Involvemen...mentioning

confidence: 98%

CanVasc Consensus Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitis: 2020 Update

Mendel¹,

Ennis²,

Go³

et al. 2020

J Rheumatol

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“…[41][42][43] For severe complications, GC, traditional immunosuppressants, and IVIG are still the most effective treatment. 44 There were inevitable limitations to this study. First, as this was a retrospective study, we could not make a causal inference for the risk factors of cardiac involvement and the IVIG's effectiveness.…”

Section: Discussionmentioning

confidence: 94%

“…41–43 For severe complications, GC, traditional immunosuppressants, and IVIG are still the most effective treatment. 44…”

Section: Discussionmentioning

confidence: 99%

Cardiac manifestations of eosinophilic granulomatosis with polyangiitis from a single-center cohort in China: clinical features and associated factors

Liu

Guo

Zhang

et al. 2021

Therapeutic Advances in Chronic Disease

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Background: Cardiac manifestations are common and life-threatening in eosinophilic granulomatosis with polyangiitis (EGPA), which remains poorly studied in China. We aim to investigate its clinical features, associated factors, treatment, and outcomes. Methods: We reviewed the clinical records of 110 EGPA patients and examined the independent factors associated with cardiac manifestations using multivariate logistic regression. Receiver operating characteristic curves determined the cut-off values, and survival was calculated via Kaplan–Meier curves. Results: Cardiac involvement was present in 36.4% (40/110) of EGPA patients, which mainly manifested as pericardial effusion (16.4%, 18/110), myocardial involvement (13.6%, 15/110), and heart failure (8.2%, 9/110). The mean age was 42.1 ± 14.23 years with no female/male predominance. Compared with the cardiac-unaffected group, the cardiac-affected group showed a lower rate of biopsy-proved vasculitis (0% versus 20%, p = 0.002). The eosinophil count [odds ratio (OR) = 1.142, 95% confidence interval (CI) 1.029–1.267] was independently associated with cardiac manifestations in EGPA, with a cut-off value of 3.66 × 109/L [area under the curve (AUC) = 0.692, p = 0.001]. Regarding treatment, the cardiac-affected group displayed a higher ratio of glucocorticoid pulse combined with intravenous cyclophosphamide (CYC-IV) (40% versus 21.4%, p = 0.037), and intravenous immunoglobulin combined with glucocorticoid plus CYC-IV (17.5% versus 4.3%, p = 0.035) than the control group. Outcomes ( p = 0.131) and survival ( p = 0.1972) were not significantly different between the groups. Conclusion: In this single-center Chinese EGPA cohort, cardiac manifestations are observed in 36.4% of patients, which primarily presents as myocardial involvement, pericardial effusion, and heart failure, independently associated with eosinophil count. Glucocorticoid combined with cyclophosphamide is the treatment cornerstone for EGPA patients with cardiac manifestations.

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“…Despite paucity of data, MTX, MMF and AZA are still often employed clinically in addition to glucocorticoid in nonsevere disease in both induction and remission phases in the spirit of steroid-sparing, and these traditional DMARDs and glucocorticoid are favoured over glucocorticoid monotherapy in the recent 2021 ACR/VF guidelines [2 ▪▪ ]. Two recent small retrospective studies of traditional DMARD use for remission induction in EGPA have shown favourable results [28 ▪ ,29 ▪ ], including a study of MMF in 15 newly diagnosed EGPA patients in which two-third achieved remission at 6 months with median prednisone dose 7.5 mg/day [29 ▪ ]. Larger, controlled trials are certainly needed.…”

Section: Traditional Disease-modifying Antirheumatic Drugsmentioning

confidence: 99%

Therapeutic advances in eosinophilic granulomatosis with polyangiitis

Ford

Aleatany

Gewurz‐Singer

2022

Current Opinion in Rheumatology

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Purpose of reviewIn recent years, therapeutic advances in eosinophilic granulomatosis with polyangiitis (EGPA) have changed our treatment paradigm. This review will summarize and discuss updates in management of EGPA, with a particular focus on biologic therapies. Recent findingsThe anti-interleukin (IL)-5 agent mepolizumab (the first FDA-approved drug specifically for EGPA) is effective in induction and maintenance of remission particularly in patients with predominantly asthma and allergic manifestations, though efficacy in ANCA-positive, vasculitic disease is unclear; additional anti-IL-5 agents are under study. Rituximab is currently recommended for remission induction in severe disease, particularly in ANCA-positive patients with vasculitic manifestations, though the supportive evidence is mostly observational. Evidence supporting use of traditional DMARDs and other biologic agents such as omalizumab remains limited and observational.

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238. efficacy of Conventional Immunosuppressants in Steroid-Dependent or Refractory Eosinophilic Granulomatosis With Polyangiitis: Study on a New Canadian Patient Cohort

Cited by 8 publications

References 0 publications

CanVasc Consensus Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitis: 2020 Update

CanVasc Consensus Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitis: 2020 Update

Cardiac manifestations of eosinophilic granulomatosis with polyangiitis from a single-center cohort in China: clinical features and associated factors

Therapeutic advances in eosinophilic granulomatosis with polyangiitis

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