Churg-Strauss syndrome pathophysiology: Difference between revisions
No edit summary |
|||
| Line 13: | Line 13: | ||
'''Churg–Strauss syndrome'''<ref name="Bolognia">{{cite book |author=Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. |title=Dermatology: 2-Volume Set |publisher=Mosby |location=St. Louis |year=2007 |pages= |isbn=1-4160-2999-0 |oclc= |doi= |accessdate=}}</ref> is a medium and small vessel [[autoimmune disease|autoimmune]] [[vasculitis]], leading to [[necrosis]]. It involves mainly the blood vessels of the [[lungs]] (it begins as a severe type of [[asthma]]), gastrointestinal system, and peripheral nerves, but also affects the heart, skin, and kidneys. | '''Churg–Strauss syndrome'''<ref name="Bolognia">{{cite book |author=Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. |title=Dermatology: 2-Volume Set |publisher=Mosby |location=St. Louis |year=2007 |pages= |isbn=1-4160-2999-0 |oclc= |doi= |accessdate=}}</ref> is a medium and small vessel [[autoimmune disease|autoimmune]] [[vasculitis]], leading to [[necrosis]]. It involves mainly the blood vessels of the [[lungs]] (it begins as a severe type of [[asthma]]), gastrointestinal system, and peripheral nerves, but also affects the heart, skin, and kidneys. | ||
==Associated Conditions== | |||
The following conditions are associated with Eosinophilic granulomatosis with polyangiitis: | The following conditions are associated with Eosinophilic granulomatosis with polyangiitis: | ||
* Wells syndrome<ref name="pmid22148019">{{cite journal| author=Lee SH, Roh MR, Jee H, Chung KY, Jung JY| title=Wells' syndrome associated with churg-strauss syndrome. | journal=Ann Dermatol | year= 2011 | volume= 23 | issue= 4 | pages= 497-500 | pmid=22148019 | doi=10.5021/ad.2011.23.4.497 | pmc=3229945 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22148019 }} </ref> | * Wells syndrome<ref name="pmid22148019">{{cite journal| author=Lee SH, Roh MR, Jee H, Chung KY, Jung JY| title=Wells' syndrome associated with churg-strauss syndrome. | journal=Ann Dermatol | year= 2011 | volume= 23 | issue= 4 | pages= 497-500 | pmid=22148019 | doi=10.5021/ad.2011.23.4.497 | pmc=3229945 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22148019 }} </ref> | ||
Revision as of 15:36, 18 November 2016
|
Churg-Strauss syndrome Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Churg-Strauss syndrome pathophysiology On the Web |
|
American Roentgen Ray Society Images of Churg-Strauss syndrome pathophysiology |
|
Risk calculators and risk factors for Churg-Strauss syndrome pathophysiology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]
Overview
Pathogenesis
The pathogenesis of Eosinophilic granulomatosis with polyangiitis is not fully understood. However, it is hypothesized
Genetics
There is correlation between Eosinophilic granulomatosis with polyangiitis with allele HLA-DRB4 and HLA-DRB1*7.[1] [2] Presence of elevated HLA-DRB4 gene in patients with asthma (with or without eosinophilia), could be used to identify a possible risk of developing Eosinophilc granulomatosis with polyangiitis. However, further follow up studies are required. [3]
Pathophysiology
Churg–Strauss syndrome[4] is a medium and small vessel autoimmune vasculitis, leading to necrosis. It involves mainly the blood vessels of the lungs (it begins as a severe type of asthma), gastrointestinal system, and peripheral nerves, but also affects the heart, skin, and kidneys.
Associated Conditions
The following conditions are associated with Eosinophilic granulomatosis with polyangiitis:
- Wells syndrome[5]
- AA amyloidosis[6]
- Asthma
- >95%
- Usually precedes the vasculitic phase by 8-10 years (but can be coincident)
- Nasal and Sinus disease
- Skin Disease
- 66%
- Subcutaneous nodules on the extensor surfaces of the arm
- Cardiovascular disease
- Pericarditis (32%)
- Pericardial constriction
- Heart failure (47%)
- Myocardial infarction (MI)
- Neurologic Disease
- 75% mononeuritis multiplex
- If untreated may progress to polyneuropathy
- Kidney Disease
- 85% with focal segmental glomerulonephritis (FSGN) on biopsy
- Unclear total incidence of renal disease.
- 70% P-anca positive
- But renal failure is rare (in contrast to Wegener’s)
- 85% with focal segmental glomerulonephritis (FSGN) on biopsy
- GI Disease
- Eosinophilic gastroenteritis with abdominal pain (59%)
- Diarrhea (33%)
- GI bleeding (18%)
Microscopic Pathology
- Eosinophilic infiltrates with necrosis (sometimes quite extensive)
- Giant cell vasculitis with eosinophils
- Interstitial and perivascular necrotizing granulomas
- Eosinophilic lymphadenopathy
References
- ↑ Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A; et al. (2007). "HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome". Arthritis Rheum. 56 (9): 3159–66. doi:10.1002/art.22834. PMID 17763415.
- ↑ Wieczorek S, Hellmich B, Gross WL, Epplen JT (2008). "Associations of Churg-Strauss syndrome with the HLA-DRB1 locus, and relationship to the genetics of antineutrophil cytoplasmic antibody-associated vasculitides: comment on the article by Vaglio et al". Arthritis Rheum. 58 (1): 329–30. doi:10.1002/art.23209. PMID 18163478.
- ↑ Bottero P, Motta F, Bonini M, Vecchio F, Ierna F, Cuppari I; et al. (2014). "Can HLA-DRB4 Help to Identify Asthmatic Patients at Risk of Churg-Strauss Syndrome?". ISRN Rheumatol. 2014 ( ): 843804. doi:10.1155/2014/843804. PMC 3963189. PMID 24734195.
- ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
- ↑ Lee SH, Roh MR, Jee H, Chung KY, Jung JY (2011). "Wells' syndrome associated with churg-strauss syndrome". Ann Dermatol. 23 (4): 497–500. doi:10.5021/ad.2011.23.4.497. PMC 3229945. PMID 22148019.
- ↑ Maamar M, Tazi-Mezalek Z, Harmouche H, El Hamany Z, Adnaoui M, Aouni M (2012). "Churg-Strauss syndrome associated with AA amyloidosis: a case report". Pan Afr Med J. 12: 30. PMC 3415051. PMID 22891088.