Difference between revisions of "Eosinophilic granulomatosis with polyangiitis"

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(+infobox)
(similar name to what was prev Wegener's)
 
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| Width      =
| Width      =
| 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      = wosinophilic vasculitis associated with a [[connective tissue disease]]
| 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''',<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> is a type of [[vasculitis]].
'''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==
Line 39: Line 43:
*'''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==

Latest revision as of 21:50, 28 November 2016

Eosinophilic granulomatosis with polyangiitis
Diagnosis in short

Eosinophilic granulomatosis with polyangiitis. H&E stain.

Synonyms Churg-Strauss syndrome, Churg-Strauss disease

LM small vessel vasculitis with abundant eosinophils and granulomas
LM DDx eosinophilic vasculitis associated with a connective tissue disease
Site blood vessels

Associated Dx asthma
Signs fever
Prevalence rare
Blood work ANCA +ve/-ve

Eosinophilic granulomatosis with polyangiitis, abbreviated EGPA,[1] is a type of vasculitis.

It was previously known as Churg-Strauss syndrome.[2]

It should not be confused with granulomatosis with polyangiitis, previously known as Wegener's granulomatosis.

General

Defining features - memory device GAFE:

  • Granulomata.
  • Asthma.
  • Fever.
  • Eosinophilia.

Other clinical features - may be present:[3]

  • ANCA.
  • Weight loss.
  • Myalgias.
  • Arthralgias
  • Glomerulonephritis.
  • Hematuria.

Notes:

Microscopic

Features:

DDx:

Images

See also

References

  1. Xu, XL.; Song, W.; Sui, X.; Song, L.; DU, QN.; Wang, X. (Oct 2016). "Radiological and Clinical Features of Eosinophilic Granulomatosis with Polyangiitis.". Zhongguo Yi Xue Ke Xue Yuan Xue Bao 38 (5): 617-620. doi:10.3881/j.issn.1000-503X.2016.05.023. PMID 27825424.
  2. Stone, JR.; Bruneval, P.; Angelini, A.; Bartoloni, G.; Basso, C.; Batoroeva, L.; Buja, LM.; Butany, J. et al. "Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases.". Cardiovasc Pathol 24 (5): 267-78. doi:10.1016/j.carpath.2015.05.001. PMID 26051917.
  3. Cottin, V.; Bel, E.; Bottero, P.; Dalhoff, K.; Humbert, M.; Lazor, R.; Sinico, RA.; Sivasothy, P. et al. (Sep 2016). "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).". Autoimmun Rev. doi:10.1016/j.autrev.2016.09.018. PMID 27671089.
  4. URL: http://emedicine.medscape.com/article/334024-overview. Accessed on: 22 January 2011.
  5. Greco, A.; Rizzo, MI.; De Virgilio, A.; Gallo, A.; Fusconi, M.; Ruoppolo, G.; Altissimi, G.; De Vincentiis, M. (Apr 2015). "Churg-Strauss syndrome.". Autoimmun Rev 14 (4): 341-8. doi:10.1016/j.autrev.2014.12.004. PMID 25500434.
  6. Chen, KR.; Su, WP.; Pittelkow, MR.; Conn, DL.; George, T.; Leiferman, KM. (Aug 1996). "Eosinophilic vasculitis in connective tissue disease.". J Am Acad Dermatol 35 (2 Pt 1): 173-82. PMID 8708015.