Eosinophilic granulomatosis with polyangiitis: Difference between revisions
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similar name to what was prev Wegener's |
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| Caption = Eosinophilic granulomatosis with polyangiitis. [[H&E stain]]. | | Caption = Eosinophilic granulomatosis with polyangiitis. [[H&E stain]]. | ||
| Synonyms = | | Synonyms = Churg-Strauss syndrome, Churg-Strauss disease | ||
| Micro = small vessel [[vasculitis]] with abundant eosinophils and [[granuloma]]s | | Micro = small vessel [[vasculitis]] with abundant eosinophils and [[granuloma]]s | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = | | LMDDx = eosinophilic vasculitis associated with a [[connective tissue disease]] | ||
| Stains = | | Stains = | ||
| IHC = | | IHC = | ||
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| Symptoms = | | Symptoms = | ||
| Prevalence = rare | | Prevalence = rare | ||
| Bloodwork = | | Bloodwork = ANCA +ve/-ve | ||
| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
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| Tx = | | Tx = | ||
}} | }} | ||
'''Eosinophilic granulomatosis with polyangiitis''', previously known as '''Churg-Strauss syndrome''' | '''Eosinophilic granulomatosis with polyangiitis''', abbreviated '''EGPA''',<ref>{{Cite journal | last1 = Xu | first1 = XL. | last2 = Song | first2 = W. | last3 = Sui | first3 = X. | last4 = Song | first4 = L. | last5 = DU | first5 = QN. | last6 = Wang | first6 = X. | title = Radiological and Clinical Features of Eosinophilic Granulomatosis with Polyangiitis. | journal = Zhongguo Yi Xue Ke Xue Yuan Xue Bao | volume = 38 | issue = 5 | pages = 617-620 | month = Oct | year = 2016 | doi = 10.3881/j.issn.1000-503X.2016.05.023 | PMID = 27825424 }}</ref> is a type of [[vasculitis]]. | ||
It was previously known as '''Churg-Strauss syndrome'''.<ref name=pmid26051917>{{Cite journal | last1 = Stone | first1 = JR. | last2 = Bruneval | first2 = P. | last3 = Angelini | first3 = A. | last4 = Bartoloni | first4 = G. | last5 = Basso | first5 = C. | last6 = Batoroeva | first6 = L. | last7 = Buja | first7 = LM. | last8 = Butany | first8 = J. | last9 = d'Amati | first9 = G. | title = Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases. | journal = Cardiovasc Pathol | volume = 24 | issue = 5 | pages = 267-78 | month = | year = | doi = 10.1016/j.carpath.2015.05.001 | PMID = 26051917 }}</ref> | |||
It should '''''not''''' be confused with ''[[granulomatosis with polyangiitis]]'', previously known as ''Wegener's granulomatosis''. | |||
==General== | ==General== | ||
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*'''F'''ever. | *'''F'''ever. | ||
*'''E'''osinophilia. | *'''E'''osinophilia. | ||
Other clinical features - may be present:<ref name=pmid27671089 >{{Cite journal | last1 = Cottin | first1 = V. | last2 = Bel | first2 = E. | last3 = Bottero | first3 = P. | last4 = Dalhoff | first4 = K. | last5 = Humbert | first5 = M. | last6 = Lazor | first6 = R. | last7 = Sinico | first7 = RA. | last8 = Sivasothy | first8 = P. | last9 = Wechsler | first9 = ME. | title = Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). | journal = Autoimmun Rev | volume = | issue = | pages = | month = Sep | year = 2016 | doi = 10.1016/j.autrev.2016.09.018 | PMID = 27671089 }}</ref> | |||
*ANCA. | |||
*Weight loss. | |||
*Myalgias. | |||
*Arthralgias | |||
*Glomerulonephritis. | |||
*Hematuria. | |||
Notes: | Notes: | ||
*Similar to [[Granulomatosis with polyangiitis]] (Wegener's granulomatosis) - classically c-ANCA +ve, and ''microscopic polyangiitis''.<ref>URL: [http://emedicine.medscape.com/article/334024-overview http://emedicine.medscape.com/article/334024-overview]. Accessed on: 22 January 2011.</ref> | *Similar to [[Granulomatosis with polyangiitis]] (Wegener's granulomatosis) - classically c-ANCA +ve, and ''microscopic polyangiitis''.<ref>URL: [http://emedicine.medscape.com/article/334024-overview http://emedicine.medscape.com/article/334024-overview]. Accessed on: 22 January 2011.</ref> | ||
*T<sub>H</sub>2 disease - like [[asthma]].<ref name=pmid25500434>{{Cite journal | last1 = Greco | first1 = A. | last2 = Rizzo | first2 = MI. | last3 = De Virgilio | first3 = A. | last4 = Gallo | first4 = A. | last5 = Fusconi | first5 = M. | last6 = Ruoppolo | first6 = G. | last7 = Altissimi | first7 = G. | last8 = De Vincentiis | first8 = M. | title = Churg-Strauss syndrome. | journal = Autoimmun Rev | volume = 14 | issue = 4 | pages = 341-8 | month = Apr | year = 2015 | doi = 10.1016/j.autrev.2014.12.004 | PMID = 25500434 }}</ref> | |||
==Microscopic== | ==Microscopic== | ||