Eosinophilic granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA)
Other names	Churg–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]
Specialty	Immunology, rheumatology
Symptoms	Fatigue, 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]
Complications	hypereosinophilia, 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 factors	History of allergy, asthma and asthma-associated lung abnormalities (i.e., pulmonary infiltrates).[1]
Diagnostic method	antineutrophil cytoplasmic antibodies (ANCA); cluster of asthma, eosinophilia, mono- or polyneuropathy, nonfixed pulmonary infiltrates, abnormality of the paranasal sinuses, and extravascular eosinophilia.[1]
Treatment	Suppress the activity of the immune system to alleviate inflammation.[1]
Medication	Corticosteroid 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]}