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Eosinophilic granulomatosis with polyangiitis

Last updated: December 14, 2022

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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 phrases: 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.

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 [1]

Most cases are idiopathic.

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. [2]

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

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

Laboratory studies [2][4][5][6]

Negative ANCA does not rule out EGPA.

Imaging studies [2][6]

Cardiac involvement affects treatment decisions and is the main cause of death in patients with EGPA. [4]

Biopsy

General principles [4][5][7]

  • 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. [7]

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). [4]

Pharmacotherapy [4]

Supportive care

  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. Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of churg-strauss syndrome (allergic granulomatosis and angiitis). Arthritis & Rheumatism. 2010; 33 (8): p.1094-1100. doi: 10.1002/art.1780330806 . | Open in Read by QxMD
  8. 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

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