Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis

Emmi, Giacomo; Bettiol, Alessandra; Gelain, Elena; Bajema, Ingeborg M.; Berti, Alvise; Burns, Stella; Cid, María C.; Tervaert, Jan Willem Cohen; Cottin, Vincent; Durante, Eugenia; Holle, Julia; Mahr, Alfred; Pero, Marcos Martinez Del; Marvisi, Chiara; Mills, John A.; Moiseev, Sergey; Moosig, Frank; Mukhtyar, Chetan; Neumann, Thomas; Olivotto, Iacopo; Salvarani, Carlo; Seeliger, Benjamin; Sinico, Renato Alberto; Taillé, Camille; Terrier, Benjamin; Venhoff, Nils; Βertsias, George; Guillevin, Loı̈c; Jayne, David; Vaglio, Augusto

doi:10.1038/s41584-023-00958-w

Cited by 50 publications

(93 citation statements)

References 108 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Common features of GPA include sinonasal disease, lower respiratory tract involvement with granulomatous inflammation or pulmonary hemorrhage, and glomerulonephritis [18]. Patients with MPA may present with some GPA symptoms, although without granulomatous inflammation and usually with more severe renal disease [19,20].…”

Section: Gpa and Mpamentioning

confidence: 99%

“…EGPA is characterized by asthma, eosinophilia, and vasculitis in many cases. Although categorized as a form of AAV, it has fewer similarities with GPA and MPA concerning its genetic, pathogenetic, and clinical features and is typically considered a separate entity [20]. EGPA is primarily characterized as a type-2 related entity, with clinical manifestations of late-onset asthma in nearly all patients (95-100%) [50].…”

Section: Egpamentioning

confidence: 99%

“…In EGPA, approximately 50% of patients test positive for MPO-ANCAs, and the presence of these autoantibodies is associated with kidney involvement, but inversely correlated with heart involvement [20]. ANCA positivity in EGPA correlates more with vasculitis manifestations, including alveolar hemorrhage, whereas ANCA negativity correlates with eosinophilic lung infiltrates [102].…”

Section: Egpa Pathogenesismentioning

confidence: 99%

Section: Treatment Of Pulmonary Manifestations In Aavmentioning

confidence: 99%

“…The treatment approach for EGPA involves high doses of corticosteroids to rapidly reduce inflammation and alleviate symptoms [20]. Alongside corticosteroids, immunosuppressive medications, such as cyclophosphamide or rituximab, may be employed to help control autoimmune reactions in severe disease [20]. For remission maintenance of non-severe disease or for induction in resistant cases of non-severe EGPA, mepolizumab, which targets IL-5, may be considered [20].…”

Section: Treatment Of Pulmonary Manifestations In Aavmentioning

confidence: 99%

See 4 more Smart Citations

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

Fouka,

Drakopanagiotakis,

Steiropoulos

2024

IJMS

View full text Add to dashboard Cite

Pulmonary manifestations of vasculitis are associated with significant morbidity and mortality in affected individuals. They result from a complex interplay between immune dysregulation, which leads to vascular inflammation and tissue damage. This review explored the underlying pathogenesis of pulmonary involvement in vasculitis, encompassing various forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and anti-GBM disease. Mechanisms involving ANCA and anti-GBM autoantibodies, neutrophil activation, and neutrophil extracellular trap (NETs) formation are discussed, along with the role of the complement system in inducing pulmonary injury. Furthermore, the impact of genetic predisposition and environmental factors on disease susceptibility and severity was considered, and the current treatment options were presented. Understanding the mechanisms involved in the pathogenesis of pulmonary vasculitis is crucial for developing targeted therapies and improving clinical outcomes in affected individuals.

show abstract

Section: Gpa and Mpamentioning

confidence: 99%

Section: Egpamentioning

confidence: 99%

Section: Egpa Pathogenesismentioning

confidence: 99%

Section: Treatment Of Pulmonary Manifestations In Aavmentioning

confidence: 99%

Section: Treatment Of Pulmonary Manifestations In Aavmentioning

confidence: 99%

See 3 more Smart Citations

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

Fouka,

Drakopanagiotakis,

Steiropoulos

2024

IJMS

View full text Add to dashboard Cite

show abstract

Bronchial asthma and allergic rhinitis—The skin sample reveals a severe systemic disease

Wölbing,

Dugas-Breit,

Hartschuh

et al. 2024

Dermatologie

View full text Add to dashboard Cite

ZusammenfassungVorgestellt wird eine 30-jährige Patientin, die seit Jahren an initial unspezifischen Symptomen, wie rezidivierenden, nicht allergischen und nicht infektiösen Sinusitiden, „late onset“ Asthma bronchiale und ausgeprägter Lymphadenopathie litt. Erst nach Auftreten erster Hauterscheinungen konnte durch die Entnahme einer Probebiopsie die Diagnose einer eosinophilen granulomatösen Polyangiitis (EGPA) gestellt werden. Die EGPA ist eine schwerwiegende Systemerkrankung, die unbehandelt multiple Organschäden verursachen und sogar einen tödlichen Verlauf nehmen kann. Mit der adäquaten Behandlung verläuft die Erkrankung in mehr als 90 % der Fälle milde und kann oft sogar vollständig in Remission gehen. Durch die richtige Diagnosestellung konnte die Patientin adäquat und erfolgreich behandelt und das Risiko für Spätmanifestationen und Folgeschäden mit potenziell tödlichem Verlauf vermindert werden.

show abstract

Cardiovascular Disease in Anti-neutrophil Cytoplasm Antibody-Associated Vasculitis

Sayer,

Chapman,

Thomas

et al. 2023

Curr Rheumatol Rep

View full text Add to dashboard Cite

Purpose of Review Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a rare, multisystem, autoimmune disease characterised by microvascular inflammation. Over the past 20 years, advances in immunological management have improved short-term patient outcomes. Longer-term patient outcomes remain poor with cardiovascular disease now the leading cause of death in AAV. Here, we examine the potential pathways that contribute to the increased risk of cardiovascular disease in AAV and the current evidence to manage this risk. Recent Findings The incidence of cardiovascular disease in AAV exceeds that expected by traditional risk factors alone, suggesting a contribution from disease-specific factors. Similarly, it is unclear how different immunosuppressive therapies contribute to and modify cardiovascular risk, and there is a paucity of data examining the efficacy of traditional cardioprotective medications in AAV. Summary There is a lack of evidence-based cardiovascular risk assessment tools and cardioprotective therapies in patients with AAV which should be addressed to improve long-term outcomes.

show abstract

Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis

Cited by 50 publications

References 108 publications

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

Bronchial asthma and allergic rhinitis—The skin sample reveals a severe systemic disease

Cardiovascular Disease in Anti-neutrophil Cytoplasm Antibody-Associated Vasculitis

Contact Info

Product

Resources

About