Churg-Strauss syndrome pathophysiology
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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. Eosinophilic granulomatosis with polyangiitis [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.
Genetics
The allele HLA-DRB1*7 and HLA-DRB4 is correlated with Eosinophilic granulomatosis with polyangiitis.[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]
Associated Conditions
The following conditions are associated with Eosinophilic granulomatosis with polyangiitis:
- Wells syndrome[4]
- AA amyloidosis[5]
- 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%)
Gross Pathology
On gross pathology, the following changes are typically seen:[6]
- Nodular swelling that appears along the small arteries of various organs, such as the heart, liver, and the renal
- Infarcts, hemorrhage and scarring of affected organs
- Pulmonary artery occlusion
- Patchy consolidations commonly affixed in the lower portions of the lung
- Fibrosis, ventricular hypertrophy of both ventricles, patchy myocardial scars, and endocardial fibrosis of the heart
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.
- ↑ 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.
- ↑ CHURG J, STRAUSS L (1951). "Allergic granulomatosis, allergic angiitis, and periarteritis nodosa". Am J Pathol. 27 (2): 277–301. PMC 1937314. PMID 14819261.