Churg-Strauss syndrome pathophysiology: Difference between revisions

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==Pathogenesis==
==Pathogenesis==
*The pathogenesis of [[Eosinophilic granulomatosis with polyangiitis]] is not fully understood.<ref name="pmid14740430">{{cite journal |vauthors=Hellmich B, Ehlers S, Csernok E, Gross WL |title=Update on the pathogenesis of Churg-Strauss syndrome |journal=Clin. Exp. Rheumatol. |volume=21 |issue=6 Suppl 32 |pages=S69–77 |date=2003 |pmid=14740430 |doi= |url=}}</ref>
*The pathogenesis of [[Eosinophilic granulomatosis with polyangiitis]] is not fully understood.<ref name="pmid14740430">{{cite journal |vauthors=Hellmich B, Ehlers S, Csernok E, Gross WL |title=Update on the pathogenesis of Churg-Strauss syndrome |journal=Clin. Exp. Rheumatol. |volume=21 |issue=6 Suppl 32 |pages=S69–77 |date=2003 |pmid=14740430 |doi= |url=}}</ref>
*Eosinophilic granulomatosis with polyangiitis  is probably caused by the result of a complex interaction involving  genetic and environmental factors that lead to an inflammatory response involving eosinophils, T, and B lymphocytes.
*[[Eosinophilic granulomatosis with polyangiitis]] is a medium and small vessel [[vasculitis]], leading to [[necrosis]].  It involves mainly the blood vessels of the [[lungs]], [[Gastrointestinal tract|gastrointestinal system]], and [[Peripheral nervous system|peripheral nerves]], but also affects the [[heart]], [[skin]], and [[Kidney|kidneys]].
*[[Eosinophilic granulomatosis with polyangiitis]] is a medium and small vessel [[vasculitis]], leading to [[necrosis]].  It involves mainly the blood vessels of the [[lungs]], [[Gastrointestinal tract|gastrointestinal system]], and [[Peripheral nervous system|peripheral nerves]], but also affects the [[heart]], [[skin]], and [[Kidney|kidneys]].
*There is insufficient data to support the role of [[Cell-mediated immunity|cell-mediated]] and [[immune complex]] mechanisms, although [[autoimmunity]] has an evident role with the presence of [[Anti-neutrophil cytoplasmic antibody|ANCA]], [[hypergammaglobulinemia]], elevated levels of [[immunoglobulin E]], and [[Rheumatoid factor|rheumatic factor]].<ref name="pmid18586318">{{cite journal |vauthors=Tsurikisawa N, Saito H, Tsuburai T, Oshikata C, Ono E, Mitomi H, Akiyama K |title=Differences in regulatory T cells between Churg-Strauss syndrome and chronic eosinophilic pneumonia with asthma |journal=J. Allergy Clin. Immunol. |volume=122 |issue=3 |pages=610–6 |date=September 2008 |pmid=18586318 |doi=10.1016/j.jaci.2008.05.040 |url=}}</ref>
*There is insufficient data to support the role of [[Cell-mediated immunity|cell-mediated]] and [[immune complex]] mechanisms, although [[autoimmunity]] has an evident role with the presence of [[Anti-neutrophil cytoplasmic antibody|ANCA]], [[hypergammaglobulinemia]], elevated levels of [[immunoglobulin E]], and [[Rheumatoid factor|rheumatic factor]].<ref name="pmid18586318">{{cite journal |vauthors=Tsurikisawa N, Saito H, Tsuburai T, Oshikata C, Ono E, Mitomi H, Akiyama K |title=Differences in regulatory T cells between Churg-Strauss syndrome and chronic eosinophilic pneumonia with asthma |journal=J. Allergy Clin. Immunol. |volume=122 |issue=3 |pages=610–6 |date=September 2008 |pmid=18586318 |doi=10.1016/j.jaci.2008.05.040 |url=}}</ref>

Revision as of 18:30, 29 March 2018

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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: Chandrakala Yannam, MD [3]

Overview

Eosinophilic granulomatosis with polyangiitis is a medium and small vessel vasculitis, leading to necrosis. The pathogenesis of Eosinophilic granulomatosis with polyangiitis is not fully understood. Autoimmunity has an evident role with the presence of ANCA, hypergammaglobulinemia, elevated levels of immunoglobulin E, and rheumatic factor in the pathogenesis. HLA-DRB4 is correlated with increasing risk of development of vascular manifestations of the churg-strauss syndrome. On microscopic pathology, eosinophic infiltration, necrotizing granulomas vasculitis and necrosis of small and medium-sized arteries can be seen.

Pathophysiology

Pathogenesis

Genetics

Associated Conditions

The following conditions are associated with Eosinophilic granulomatosis with polyangiitis:[8][9][10][11]

Gross Pathology

On gross pathology, the following changes are seen in patients with eosinophilic granulomatosis with polyangiitis include:[12]

Microscopic Pathology

On microscopic examination, following findings can be seen in eosinophilic granulomatosis with polyangiitis, include:[13][14]

Lung biopsy showing eosinophilic vasculitis consistent with Churg-Strauss syndrome - By By Nephron - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=15244866


References

  1. Hellmich B, Ehlers S, Csernok E, Gross WL (2003). "Update on the pathogenesis of Churg-Strauss syndrome". Clin. Exp. Rheumatol. 21 (6 Suppl 32): S69–77. PMID 14740430.
  2. Tsurikisawa N, Saito H, Tsuburai T, Oshikata C, Ono E, Mitomi H, Akiyama K (September 2008). "Differences in regulatory T cells between Churg-Strauss syndrome and chronic eosinophilic pneumonia with asthma". J. Allergy Clin. Immunol. 122 (3): 610–6. doi:10.1016/j.jaci.2008.05.040. PMID 18586318.
  3. Wechsler ME, Garpestad E, Flier SR, Kocher O, Weiland DA, Polito AJ, Klinek MM, Bigby TD, Wong GA, Helmers RA, Drazen JM (February 1998). "Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast". JAMA. 279 (6): 455–7. PMID 9466639.
  4. Tuggey JM, Hosker HS (September 2000). "Churg-Strauss syndrome associated with montelukast therapy". Thorax. 55 (9): 805–6. PMC 1745850. PMID 10950903.
  5. 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.
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. Neumann T, Manger B, Schmid M, Kroegel C, Hansch A, Kaiser WA, Reinhardt D, Wolf G, Hein G, Mall G, Schett G, Zwerina J (July 2009). "Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis". Medicine (Baltimore). 88 (4): 236–43. doi:10.1097/MD.0b013e3181af35a5. PMID 19593229.
  11. Chumbley LC, Harrison EG, DeRemee RA (August 1977). "Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases". Mayo Clin. Proc. 52 (8): 477–84. PMID 18640.
  12. CHURG J, STRAUSS L (1951). "Allergic granulomatosis, allergic angiitis, and periarteritis nodosa". Am J Pathol. 27 (2): 277–301. PMC 1937314. PMID 14819261.
  13. 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.
  14. 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.

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