Churg-Strauss syndrome pathophysiology: Difference between revisions

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{{Churg-Strauss syndrome}}
{{Churg-Strauss syndrome}}
{{CMG}}{{APM}}{{AE}}{{KW}}
{{CMG}}{{APM}}{{AE}}{{CK}}


==Overview==
==Overview==
[[Eosinophilic granulomatosis with polyangiitis]] is a medium and small vessel [[vasculitis]], leading to [[necrosis]] and [[vasculitis]]. The pathogenesis of [[eosinophilic granulomatosis with polyangiitis]] is not fully understood. It may occur as a  result of a complex interaction involving [[Genetics|genetic]] and environmental factors that lead to an [[Inflammation|inflammatory response]] <nowiki/>involving [[Eosinophil granulocyte|eosinophils]], [[B cell|lymphocytes]] and [[Macrophage|giant cells]]. [[Autoimmunity]] has an evident role in the presence of [[Anti-neutrophil cytoplasmic antibody|ANCA]], [[hypergammaglobulinemia]], elevated levels of [[immunoglobulin E]], and [[Rheumatoid factor|rheumatic factor]] in the pathogenesis. [[HLA-DRB4]] is correlated with increased risk of development of vascular manifestations of the [[Eosinophilic granulomatosis with polyangiitis|churg-strauss syndrome]]. On microscopic  pathology, [[Eosinophil granulocyte|eosinophlic]] infiltration, [[Granulomas|necrotizing granulomas]] [[vasculitis]] and [[necrosis]] of small and medium-sized [[Artery|arteries]] can be seen.
==Pathophysiology==
==Pathophysiology==
'''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 Diseases===
==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>
*[[Eosinophilic granulomatosis with polyangiitis]]  is probably caused by the result of a complex interaction involving  [[Genetics|genetic]] and environmental factors that lead to an [[Inflammation|inflammatory response]] involving [[Eosinophil granulocyte|eosinophils]], [[T cell|T lymphocytes]], and [[B cell|B lymphocytes]].
*The disease most commonly involves  medium and small vessel resulting in [[necrosis]] and [[vasculitis]].  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]] mediations, 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>
*[[Eosinophilic granulomatosis with polyangiitis]] may develop in steroid-dependent [[asthma]] patients after they started treatment with [[Leukotriene antagonist|leukotriene receptor antagonists]] / leukotriene modifying agents (eg, [[zafirlukast]], [[montelukast]]) with reduced oral steroid dosage. This [[Complication (medicine)|complication]] is more likely caused by steroid withdrawal by unmasking underlying [[disease]].<ref name="pmid9466639">{{cite journal |vauthors=Wechsler ME, Garpestad E, Flier SR, Kocher O, Weiland DA, Polito AJ, Klinek MM, Bigby TD, Wong GA, Helmers RA, Drazen JM |title=Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast |journal=JAMA |volume=279 |issue=6 |pages=455–7 |date=February 1998 |pmid=9466639 |doi= |url=}}</ref><ref name="pmid10950903">{{cite journal |vauthors=Tuggey JM, Hosker HS |title=Churg-Strauss syndrome associated with montelukast therapy |journal=Thorax |volume=55 |issue=9 |pages=805–6 |date=September 2000 |pmid=10950903 |pmc=1745850 |doi= |url=}}</ref>
*It has been reported in patients whose oral steroid [[withdrawal]] is facilitated by use of [[Glucocorticosteroid|inhaled glucocorticosteroids]], [[omalizumab]] (human Ig E antagonist, and [[cocaine]]) likely by the unmasking of the underlying disease.
 
==Genetics==
*[[Human leukocyte antigen|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 [[Eosinophilic granulomatosis with polyangiitis|churg-strauss syndrome]].<ref name="pmid17763415">{{cite journal |vauthors=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 |title=HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome |journal=Arthritis Rheum. |volume=56 |issue=9 |pages=3159–66 |date=September 2007 |pmid=17763415 |doi=10.1002/art.22834 |url=}}</ref><ref name="pmid24734195">{{cite journal |vauthors=Bottero P, Motta F, Bonini M, Vecchio F, Ierna F, Cuppari I, Sinico RA |title=Can HLA-DRB4 Help to Identify Asthmatic Patients at Risk of Churg-Strauss Syndrome? |journal=ISRN Rheumatol |volume=2014 |issue= |pages=843804 |date=2014 |pmid=24734195 |pmc=3963189 |doi=10.1155/2014/843804 |url=}}</ref>
*Single-[[Polymorphisms|nucleotide polymorphisms]] in the [[Interleukin 10|Interleukin-10]] gene (IL10.2 haplotype) have been associated in the [[pathogenesis]] of [[Eosinophilic granulomatosis with polyangiitis|eosinophilic granulomatosis with polyangiitis]].<ref name="pmid18512809">{{cite journal |vauthors=Wieczorek S, Hellmich B, Arning L, Moosig F, Lamprecht P, Gross WL, Epplen JT |title=Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener's granulomatosis |journal=Arthritis Rheum. |volume=58 |issue=6 |pages=1839–48 |date=June 2008 |pmid=18512809 |doi=10.1002/art.23496 |url=}}</ref>
 
==Associated Conditions==
The following conditions are associated with [[Eosinophilic granulomatosis with polyangiitis]]:<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><ref name="pmid22891088">{{cite journal| author=Maamar M, Tazi-Mezalek Z, Harmouche H, El Hamany Z, Adnaoui M, Aouni M| title=Churg-Strauss syndrome associated with AA amyloidosis: a case report. | journal=Pan Afr Med J | year= 2012 | volume= 12 | issue=  | pages= 30 | pmid=22891088 | doi= | pmc=3415051 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22891088  }} </ref><ref name="pmid19593229">{{cite journal |vauthors=Neumann T, Manger B, Schmid M, Kroegel C, Hansch A, Kaiser WA, Reinhardt D, Wolf G, Hein G, Mall G, Schett G, Zwerina J |title=Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis |journal=Medicine (Baltimore) |volume=88 |issue=4 |pages=236–43 |date=July 2009 |pmid=19593229 |doi=10.1097/MD.0b013e3181af35a5 |url=}}</ref><ref name="pmid18640">{{cite journal |vauthors=Chumbley LC, Harrison EG, DeRemee RA |title=Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases |journal=Mayo Clin. Proc. |volume=52 |issue=8 |pages=477–84 |date=August 1977 |pmid=18640 |doi= |url=}}</ref>
* Well's syndrome
* AA [[amyloidosis]]
* [[Asthma]]
* [[Asthma]]
*:* >95%
* [[Nasal polyp|Nasal polyposis]] and [[Rhinosinusitis|sinusitis]]
*:* Usually precedes the vasculitic phase by 8-10 years (but can be coincident)
* [[Skin]]
* Nasal and Sinus disease
** Subcutaneous tender [[Nodule (medicine)|nodules]] on the extensor surfaces of the [[arm]] and [[Leg|legs]].
* Skin Disease
** Palpable [[purpura]]
*:* 66%
** Hemorrhagic [[Petechia|petechiae]]
*:* Subcutaneous nodules on the extensor surfaces of the arm
* Cardiovascular:
* Cardiovascular disease
** [[Pericarditis]]
*:* [[Pericarditis]] (32%)
** [[Myocarditis]]
*:* [[Pericardial constriction]]
** Sub endocardial fibrosis
*:* [[Heart failure]] (47%)
** [[Pericardial constriction]]
*:* [[Myocardial infarction]] (MI)
** [[Heart failure]]  
* Neurologic Disease
** [[Myocardial infarction]]
*:* 75% [[mononeuritis multiplex]]
* Neurologic:
*:* If untreated may progress to [[polyneuropathy]]
** [[Mononeuritis multiplex]]
* Kidney Disease
** [[Polyneuropathy]]
*:* 85% with [[focal segmental glomerulonephritis]] ([[FSGN]]) on biopsy
* Renal:
*:*:* Unclear total incidence of renal disease.
** [[Focal segmental glomerulosclerosis|Focal segmental glomerulonephritis]]
*:* 70% [[P-anca]] positive
** [[Renal insufficiency|Renal failure]]
*:*:* But [[renal failure]] is rare (in contrast to [[Wegener’s]])
* GI Disease:
* GI Disease
** [[Eosinophilic gastroenteritis]] with [[abdominal pain]]  
*:* [[Eosinophilic gastroenteritis]] with [[abdominal pain]] (59%)
** [[Diarrhea]]  
*:* [[Diarrhea]] (33%)
** [[GI bleeding]]
*:* [[GI bleeding]] (18%)
 
==Gross Pathology==
On [[gross pathology]], the following changes are  seen in patients with [[eosinophilic granulomatosis with polyangiitis]] include:<ref name="pmid14819261">{{cite journal| author=CHURG J, STRAUSS L| title=Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. | journal=Am J Pathol | year= 1951 | volume= 27 | issue= 2 | pages= 277-301 | pmid=14819261 | doi= | pmc=1937314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14819261  }} </ref>
* Nodular [[Swelling|swellings]] that appear along the course of small arteries of various organs.
** [[Heart]], [[liver]], [[kidney]], [[Gastrointestinal tract|GI tract]], and [[spleen]].
* [[Infarction|Infarcts]], hemorrhage and [[Scar|scarring]] of affected organs
* [[Pulmonary artery]] occlusion and [[thrombus]] formation
* Patchy [[Consolidation (medicine)|consolidations]] of the [[lung]]  
* [[Ventricular hypertrophy]], and  myocardial scariing.
==Microscopic Pathology==
On microscopic examination, following findings can be seen in [[eosinophilic granulomatosis with polyangiitis]], include:<ref name="pmid11743052">{{cite journal |vauthors=Churg A |title=Recent advances in the diagnosis of Churg-Strauss syndrome |journal=Mod. Pathol. |volume=14 |issue=12 |pages=1284–93 |date=December 2001 |pmid=11743052 |doi=10.1038/modpathol.3880475 |url=}}</ref><ref name="pmid11068552">{{cite journal |vauthors=Katzenstein AL |title=Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review |journal=Am. J. Clin. Pathol. |volume=114 |issue=5 |pages=767–72 |date=November 2000 |pmid=11068552 |doi=10.1309/F3FW-J8EB-X913-G1RJ |url=}}</ref>
*Infiltration of [[Eosinophil granulocyte|eosinophils]] with [[necrosis]]
*Necrotizing [[vasculitis]] involving small and medium-sized [[Artery|arteries]] and [[Venule|venules]].
*Necrotizing [[Granuloma|granulomas]]
**Interstitial and perivascular necrotizing [[Granuloma|granulomas]]
**[[Granuloma|Granulomas]] composed of an [[eosinophilic]] center and surrounded by [[Macrophage|macrophages]], [[Histiocyte|histocytes]], and multi nucleated giant cells peripherally.
[[File:Churg-Strauss syndrome.jpg|centre|thumb|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]]


===Microscopic Pathology===
* Eosinophilic infiltrates with necrosis (sometimes quite extensive)
* Giant cell vasculitis with eosinophils
* Interstitial and perivascular necrotizing granulomas
* Eosinophilic lymphadenopathy


[[Image:Churg strauss arteritis.jpg|thumb|center|Necrotizing and granulomatous arteritis in the lung of a patient with Churg-Strauss syndrome.]]
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
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Latest revision as of 17:43, 12 April 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 and vasculitis. The pathogenesis of eosinophilic granulomatosis with polyangiitis is not fully understood. It may occur as a  result of a complex interaction involving genetic and environmental factors that lead to an inflammatory response involving eosinophils, lymphocytes and giant cells. Autoimmunity has an evident role in the presence of ANCA, hypergammaglobulinemia, elevated levels of immunoglobulin E, and rheumatic factor in the pathogenesis. HLA-DRB4 is correlated with increased risk of development of vascular manifestations of the churg-strauss syndrome. On microscopic pathology, eosinophlic 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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