Eosinophilic granulomatosis with polyangiitis (EGPA; also known as Churg-Strauss syndrome [CSS] or allergic granulomatosis) is a rare autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy).
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. The third stage consists of vasculitis, which can eventually lead to cell death and can be life-threatening.
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. Prior to this it was known as "Churg-Strauss syndrome", named after Drs. Jacob Churg and Lotte Strausswho, in 1951, first published about the syndrome using the term "allergic granulomatosis" to describe it. It is a type ofsystemic 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.
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Raina s, kaul r, kaur n, chauhan ns. Eosinophilic granulomatosis with polyangitis (churg-strauss syndrome): a diagnostic rarity with an atypical presentation. Egypt rheumatol rehabil [serial online] 2014 [cited 2016 dec 29];41:187-9. Available from: http://www.err.eg.net/text.asp?2014/41/4/187/147363
Https://www.hindawi.com/journals/crie/2013/415810/ russell senanayake and mamoun mukhtar, “cotrimoxazole-induced hypoglycaemia in a patient with churg-strauss syndrome,” case reports in endocrinology, vol. 2013, article id 415810, 3 pages, 2013. Doi:10.1155/2013/415810
Https://www.hindawi.com/journals/crihep/2011/412524/ keiji matsui and kosuke nishijima, “a case of montelukast-induced churg-strauss syndrome associated with liver dysfunction,” case reports in hepatology, vol. 2011, article id 412524, 4 pages, 2011. Doi:10.1155/2011/412524
Https://www.hindawi.com/journals/cripu/2014/673906/ kamal kishore pandita, khalid javid bhat, sushil razdan, and r. P. Kudyar, ““peripheral neuropathy crippling bronchial asthma”: two rare case reports of churg-strauss syndrome,” case reports in pulmonology, vol. 2014, article id 673906, 3 pages, 2014. Doi:10.1155/2014/673906
Kamata y, hashizume k, kaneko m, kurosaka d. A case of churg-strauss syndrome and central retinal artery occlusion with good visual recovery. Indian j ophthalmol [serial online] 2013 [cited 2016 dec 29];61:178-9. Available from: http://www.ijo.in/text.asp?2013/61/4/178/112164
Https://www.hindawi.com/journals/ijr/2009/504105/ r. A. M. Quax, a. J. G. Swaak, and m. G. A. Baggen, “churg-strauss syndrome following ptu treatment,”international journal of rheumatology, vol. 2009, article id 504105, 4 pages, 2009. Doi:10.1155/2009/504105