Difference between revisions of "Eosinophilic granulomatosis with polyangiitis"

From Libre Pathology
Jump to navigation Jump to search
(split out)
 
(similar name to what was prev Wegener's)
 
(9 intermediate revisions by the same user not shown)
Line 1: Line 1:
'''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]].
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Churg-Strauss_syndrome_-_high_mag.jpg
| Width      =
| Caption    = Eosinophilic granulomatosis with polyangiitis. [[H&E stain]].
| Synonyms  = Churg-Strauss syndrome, Churg-Strauss disease
| Micro      = small vessel [[vasculitis]] with abundant eosinophils and [[granuloma]]s
| Subtypes  =
| LMDDx      = eosinophilic vasculitis associated with a [[connective tissue disease]]
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Staging    =
| Site      = [[blood vessels]]
| Assdx      = [[asthma]]
| Syndromes  =
| Clinicalhx =
| Signs      = fever
| Symptoms  =
| Prevalence = rare
| Bloodwork  = ANCA +ve/-ve
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
| Tx        =
}}
'''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 is also known as ''allergic granulomatous angiitis''.<ref name=emedicine333492>[http://emedicine.medscape.com/article/333492-overview http://emedicine.medscape.com/article/333492-overview]</ref>
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 9: 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 ''[[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

  • CSS - high mag. (WC)

  • CSS - very high mag. (WC)

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.
Retrieved from "https://librepathology.org/w/index.php?title=Eosinophilic_granulomatosis_with_polyangiitis&oldid=46373"