Eosinophilic granulomatosis with polyangiitis

Last updated: June 15, 2023

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Summarytoggle arrow icon

Eosinophilic granulomatosis with polyangiitis (EGPA) is an ANCA-associated vasculitis of small vessels characterized by necrotizing granulomatous vasculitis with eosinophilia. It most commonly involves the lungs and skin. Clinical features are often divided into three phases: a prodromal phase (i.e., severe allergic asthma attacks, allergic rhinitis/sinusitis), an eosinophilic phase (e.g., pericarditis, gastrointestinal involvement), and a vasculitic phase (e.g., cutaneous nodules, palpable purpura, mononeuritis multiplex); constitutional symptoms are often also present. Laboratory studies typically reveal peripheral blood eosinophilia, MPO-ANCAs, and increased IgE levels. High-resolution CT chest and echocardiogram are required to assess for pulmonary and cardiac involvement. Biopsy of the affected tissue is required to confirm the diagnosis; findings include necrotizing vasculitis (eosinophilic infiltration with fibrinoid necrosis). Management typically involves immunosuppressive agents (e.g., glucocorticoids). Plasmapheresis may be indicated in patients with rapidly progressive renal failure or pulmonary hemorrhage.

Definitiontoggle arrow icon

A multisystem disease characterized by necrotizing granulomatous vasculitis with eosinophilia; that most commonly involves the lungs and the skin; can also affect the renal, cardiovascular, gastrointestinal, central, and peripheral nervous systems [2]

Etiologytoggle arrow icon

Most cases are idiopathic.

Clinical featurestoggle arrow icon

Constitutional symptoms are often present in all phases. Features from all three phases may be present at the same time and do not necessarily follow a specific order. [3]

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): pANCA and polyneuropathy (foot or wrist drop), allergic rhinitis/sinusitis/asthma, vasculitis, eosinophilia, and skin nodules

Diagnosticstoggle arrow icon

EGPA is primarily a clinical diagnosis supported by imaging and histopathological studies.

Laboratory studies [3][5][6][7]

Negative ANCA does not rule out EGPA.

Imaging studies [3][7]

Cardiac involvement affects treatment decisions and is the main cause of death in patients with EGPA. [5]


Treatmenttoggle arrow icon

General principles [5][6][8]

  • Consult rheumatology and other specialties (e.g., nephrology, pulmonology) as required.
  • Goal of pharmacotherapy (e.g., glucocorticoids PLUS cyclophosphamide) is induction of remission
  • Plasmapheresis is only considered for patients with rapidly progressive renal failure or pulmonary hemorrhage. [8]

Management of EGPA is based on disease severity, taking into account the presence of organ- and/or life-threatening manifestations (e.g., alveolar hemorrhage, glomerulonephritis, limb ischemia). [5]

Pharmacotherapy [5]

Supportive care

Referencestoggle arrow icon

  1. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
  2. Greco A, Rizzo MI, De Virgilio A, et al. Churg–Strauss syndrome. Autoimmun Rev. 2015; 14 (4): p.341-348.doi: 10.1016/j.autrev.2014.12.004 . | Open in Read by QxMD
  3. Assmann G, Molinger M, Pfreundschuh M, Bohle R, Zimmer V. Gastrointestinal perforation due to vasculitis at primary diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) despite a high dose glucocorticosteroids treatment. SpringerPlus. 2014; 3 (1).doi: 10.1186/2193-1801-3-404 . | Open in Read by QxMD
  4. Chung SA, Langford CA, Maz M, et al. American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis. Arthritis Care Res. 2021; 73 (8): p.1088-1105.doi: 10.1002/acr.24634 . | Open in Read by QxMD
  5. Groh M, Pagnoux C, Baldini C, et al. Eosinophilic granulomatosis with polyangiitis (Churg–Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015; 26 (7): p.545-553.doi: 10.1016/j.ejim.2015.04.022 . | Open in Read by QxMD
  6. Vaglio A, Buzio C, Zwerina J. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art. Allergy. 2013; 68 (3): p.261-273.doi: 10.1111/all.12088 . | Open in Read by QxMD
  7. Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016; 75 (9): p.1583-1594.doi: 10.1136/annrheumdis-2016-209133 . | Open in Read by QxMD
  8. $Contributor Disclosures - Eosinophilic granulomatosis with polyangiitis. All of the relevant financial relationships listed for the following individuals have been mitigated: Alexandra Willis (copyeditor, was previously employed by OPEN Health Communications). None of the other individuals in control of the content for this article reported relevant financial relationships with ineligible companies. For details, please review our full conflict of interest (COI) policy.

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