Eosinophilic granulomatosis with polyangiitis

Not to be confused with granulomatosis with polyangiitis.
Eosinophilic granulomatosis with polyangiitis (EGPA)
Other namesChurg–Strauss syndrome, allergic angiitis and granulomatosis.[1]
Micrograph showing an eosinophilic vasculitis consistent with eosinophilic granulomatosis with polyangiitis. H&E stain. One of the American College of Rheumatology criteria for EGPA is extravascular eosinophil infiltration on biopsy.[2]
SpecialtyImmunology, rheumatology Edit this on Wikidata
SymptomsFatigue, fever, weight loss, night sweats, abdominal pain, cough, joint pain, muscle pain, bleeding into tissues under the skin, a rash with hives, small bumps, or a general feeling of ill.[1]
Complicationshypereosinophilia, granulomatosis, vasculitis, inner ear infections with fluid build up, inflammation of the moist membrane lining the surface of the eyelids, or inflammation of peripheral nerves.[1]
Risk factorsHistory of allergy, asthma and asthma-associated lung abnormalities (i.e., pulmonary infiltrates).[1]
Diagnostic methodantineutrophil cytoplasmic antibodies (ANCA); cluster of asthma, eosinophilia, mono- or polyneuropathy, nonfixed pulmonary infiltrates, abnormality of the paranasal sinuses, and extravascular eosinophilia.[1]
TreatmentSuppress the activity of the immune system to alleviate inflammation.[1]
MedicationCorticosteroid medications such as prednisone or methylprednisolone, and mepolizumab.[1] Proliferation inhibitor for those with the presence of kidney or neurological disease.[1]

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as allergic granulomatosis,[3][4] is an extremely rare autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy).[5]

It usually manifests in three stages. The early (prodromal) stage is marked by airway inflammation; almost all patients experience asthma and/or allergic rhinitis. The second stage is characterized by abnormally high numbers of eosinophils (hypereosinophilia), which causes tissue damage, most commonly to the lungs and the digestive tract.[5] The third stage consists of vasculitis, which can eventually lead to cell death and can be life-threatening.[5]

This condition is now called "eosinophilic granulomatosis with polyangiitis" to remove all eponyms from the vasculitides. To facilitate the transition, it was referred to as "eosinophilic granulomatosis with polyangiitis (Churg–Strauss)" for a period of time starting in 2012.[6] Prior to this it was known as Churg–Strauss syndrome, named after Jacob Churg and Lotte Strauss, who first published about the syndrome in 1951 using the term allergic granulomatosis to describe it.[3] It is a type of systemic necrotizing vasculitis.

Effective treatment of EGPA requires suppression of the immune system with medication. This is typically glucocorticoids, followed by other agents such as cyclophosphamide or azathioprine.[citation needed]

  1. ^ a b c d e f g h "Churg Strauss Syndrome". NORD (National Organization for Rare Disorders). 11 February 2015. Retrieved 8 March 2020.
  2. ^ Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. (August 1990). "The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis)". Arthritis and Rheumatism. 33 (8): 1094–100. doi:10.1002/art.1780330806. PMID 2202307.
  3. ^ a b Cite error: The named reference Churg was invoked but never defined (see the help page).
  4. ^ Adu, Emery & Madaio 2012, p. 125.
  5. ^ a b c "What Is Churg-Strauss Syndrome?". WebMD. 30 January 2019. Retrieved 8 March 2020.
  6. ^ Montesi SB, Nance JW, Harris RS, Mark EJ (June 2016). "CASE RECORDS of the MASSACHUSETTS GENERAL HOSPITAL. Case 17-2016. A 60-Year-Old Woman with Increasing Dyspnea". The New England Journal of Medicine. 374 (23): 2269–79. doi:10.1056/NEJMcpc1516452. PMID 27276565.