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
- HLA-DRB1*7, and HLA-DRB4 are associated with the development of eosinophilic granulomatosis with polyangiitis. HLA-DRB4 is correlated with increasing risk of development of vascular manifestations of the churg-strauss syndrome.[1][2]
- Single-nucleotide polymorphisms in the Interleukin-10 gene (IL10.2 haplotype) have been associated with eosinophilic granulomatosis with polyangitis when compared to wegener's granulomatosis.[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
- Infiltration of eosinophils with necrosis[7]
- Necrotizing vasculitis involving small and medium-sized arteries and venules.
- Necrotizing granulomas[8]
- Interstitial and perivascular necrotizing granulomas
- Granulomas composed of an eosinophilic center and surrounded by macrophages and giant cells peripherally.
- Eosinophilic lymphadenopathy
References
- ↑ Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, Garini G, Manganelli P, Bottero P, Tumiati B, Sinico RA, Savi M, Buzio C, Neri TM (September 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.
- ↑ Bottero P, Motta F, Bonini M, Vecchio F, Ierna F, Cuppari I, Sinico RA (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.
- ↑ Wieczorek S, Hellmich B, Arning L, Moosig F, Lamprecht P, Gross WL, Epplen JT (June 2008). "Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener's granulomatosis". Arthritis Rheum. 58 (6): 1839–48. doi:10.1002/art.23496. PMID 18512809.
- ↑ 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.
- ↑ Churg A (December 2001). "Recent advances in the diagnosis of Churg-Strauss syndrome". Mod. Pathol. 14 (12): 1284–93. doi:10.1038/modpathol.3880475. PMID 11743052.
- ↑ Katzenstein AL (November 2000). "Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review". Am. J. Clin. Pathol. 114 (5): 767–72. doi:10.1309/F3FW-J8EB-X913-G1RJ. PMID 11068552.