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


==Overview==
==Overview==
[[Eosinophilic granulomatosis with polyangiitis]] develops through three phases, include [[Prodrome|prodromal]] phase, eosinophilic phase, vasculitic phase. Most complications result from the vasculitic phase. Most common complications include [[cardiomyopathy]], [[myocardial infarction]], [[Pericarditis|perimyocarditis]], rapidly progressive [[Renal insufficiency|renal failure]], [[Gastrointestinal bleeding|GI bleeding]], [[neuropathy]], and [[status asthmaticus]]. Prognosis of [[eosinophilic granulomatosis with polyangiitis]] is poor if left untreated. [[Prognosis]] is most likely dependent on stage at which the [[disease]] was diagnosed and organ involvement. The five-factor score assessment (FFS)  is a good predictor of survival rate. It can be used to choose the appropriate treatment.


==Natural History==
==Natural History, Complications, and Prognosis==
Classically, three phases of Eosinophilic granulomatosis with polyangiitis are seen. They are distributed as follows:<ref name="pmid25500434">{{cite journal| author=Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G et al.| title=Churg-Strauss syndrome. | journal=Autoimmun Rev | year= 2015 | volume= 14 | issue= 4 | pages= 341-8 | pmid=25500434 | doi=10.1016/j.autrev.2014.12.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25500434  }}</ref>


====Stage 1: The Prodromal Phase====
===Natural History===
This stage occurs in the teenage years through the 20’s. The first stage often involves the sinuses and the new onset of allergies or the worsening of pre-existing allergies (e.g. atopic diseases, [[allergic rhinitis]]).
*Classically, three phases of  [[eosinophilic granulomatosis with polyangiitis]] are seen. They are distributed as follows:<ref name="pmid25500434">{{cite journal |vauthors=Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M |title=Churg-Strauss syndrome |journal=Autoimmun Rev |volume=14 |issue=4 |pages=341–8 |date=April 2015 |pmid=25500434 |doi=10.1016/j.autrev.2014.12.004 |url=}}</ref>
**'''Prodromal phase:''' This stage occurs in the teenage years through the 20’s.
**'''Eosinophilic Phase:''' Peripheral blood [[eosinophilia]] is present. The second stage involves the onset of acute [[asthma]].
***People can live for many years in the first two stages before progressing to stage three.
**'''The Vasculitic Phase:''' The third phase occurs in the 40’s-50’s and involves multiple organ systems in a life-threatening systemic [[vasculitis]] of small and medium-sized vessels.
*If [[eosinophilic granulomatosis with polyangiitis]] left untreated, death occurs most commonly from the following complications:
**[[Congestive heart failure|Cardiac failure]]
**[[Myocardial infarction in the elderly|Myocardial infarction]]
**Rapidly progressive [[Renal insufficiency|renal failure]]
**[[Intracranial hemorrhage|Cerebral hemorrhage]]
**Gastrointestinal bleeding/perforation
**[[Status asthmaticus]]


====Stage 2: The Eosinophilic Phase====
===Complications===
Peripheral blood [[eosinophilia]] is now present. There is eosinophilic infiltration of lung and gastrointestinal (GI) tract. The second stage involves the onset of acute [[asthma]].  Normally, the person would not have had asthma previously.
*[[Complication (medicine)|Complications]] of [[eosinophilic granulomatosis with polyangiitis]] depend on the specific organ involved in the disease process.
**[[Heart|'''Heart''']]:<ref name="pmid21138993">{{cite journal |vauthors=Aakerøy L, Amundsen BH, Skomsvoll JF, Haugen BO, Soma J |title=A 50-year-old man with eosinophilia and cardiomyopathy: need for endomyocardial biopsy? |journal=Eur J Echocardiogr |volume=12 |issue=3 |pages=257–9 |date=March 2011 |pmid=21138993 |doi=10.1093/ejechocard/jeq167 |url=}}</ref>
***[[Pericarditis]]
***[[Myocarditis]]
***[[Pericardial effusion]]
***[[Myocardial infarction]]
***[[Cardiac tamponade]]
**[[Lung|'''Lung''']]:<ref name="pmid9645420">{{cite journal |vauthors=Lai RS, Lin SL, Lai NS, Lee PC |title=Churg-Strauss syndrome presenting with pulmonary capillaritis and diffuse alveolar hemorrhage |journal=Scand. J. Rheumatol. |volume=27 |issue=3 |pages=230–2 |date=1998 |pmid=9645420 |doi= |url=}}</ref>
***[[Pleural effusion]]
***[[Pulmonary hypertension]]
***Alveolar hemorrhage
***[[Status asthmaticus]]
**[[Kidney|'''Kidney''']]:<ref name="pmid2113641">{{cite journal |vauthors=Clutterbuck EJ, Evans DJ, Pusey CD |title=Renal involvement in Churg-Strauss syndrome |journal=Nephrol. Dial. Transplant. |volume=5 |issue=3 |pages=161–7 |date=1990 |pmid=2113641 |doi= |url=}}</ref>
***[[Glomerulonephritis]]
***[[Renal insufficiency|Renal failure]]
**[[Gastrointestinal tract|'''GI tract''']]:<ref name="pmid9497232">{{cite journal |vauthors=Kaneki T, Kawashima A, Hayano T, Honda T, Kubo K, Koizumi T, Sekiguchi M, Ichikawa H, Matsuzawa K, Katsuyama T |title=Churg-Strauss syndrome (allergic granulomatous angitis) presenting with ileus caused by ischemic ileal ulcer |journal=J. Gastroenterol. |volume=33 |issue=1 |pages=112–6 |date=February 1998 |pmid=9497232 |doi= |url=}}</ref>
***[[Gastrointestinal bleeding]]
***[[Abdominal pain|Acute abdominal pain]] or intestinal angina
**'''Neurologic''':<ref name="pmid20050889">{{cite journal |vauthors=Wolf J, Bergner R, Mutallib S, Buggle F, Grau AJ |title=Neurologic complications of Churg-Strauss syndrome--a prospective monocentric study |journal=Eur. J. Neurol. |volume=17 |issue=4 |pages=582–8 |date=April 2010 |pmid=20050889 |doi=10.1111/j.1468-1331.2009.02902.x |url=}}</ref>
***[[peripheral neuropathy]]
***[[Intracranial hemorrhage|Cerebral hemorrhage]]
**[[Skin|'''Skin''']]:<ref name="pmid19569284">{{cite journal |vauthors=Lestre S, Serrão V, João A, Pinheiro S, Lobo L |title=[Churg-Strauss syndrome presenting with cutaneous vasculitis] |language=Portuguese |journal=Acta Reumatol Port |volume=34 |issue=2A |pages=281–7 |date=2009 |pmid=19569284 |doi= |url=}}</ref>
***[[Purpura]]
***[[Petechia|Petichiae]]
***Noduluar lesions on extensor surfaces


People can live for many years in the first two stages before progressing to stage three.
===Prognosis===
 
*Prognosis of [[eosinophilic granulomatosis with polyangiitis]] is poor if left untreated.
====Stage 3: The Vasculitic Phase====
*Treatment with [[glucocorticoids]] has been shown to improve [[prognosis]].<ref name="pmid18181034">{{cite journal |vauthors=Guillevin L |title=Advances in the treatments of systemic vasculitides |journal=Clin Rev Allergy Immunol |volume=35 |issue=1-2 |pages=72–8 |date=October 2008 |pmid=18181034 |doi=10.1007/s12016-007-8068-4 |url=}}</ref>
The third phase occurs in the 40’s-50’s and involves multiple organ systems in a life-threatening systemic [[vasculitis]] of small and medium sized vessels.  Stage three is by far the most life threatening and painful.  Often the person will develop severe nerve pain in their legs, arms and hands.  Purple marks will appear on the skin and often sores will appear in the mouth or nose. The disease will affect the heart and lungs or it will affect the kidneys and liver.
*Prognosis is most likely dependent on stage at which the [[disease]] was diagnosed and organ involvement.
 
*The following are favorable [[Prognosis|prognostic factors]]:<ref name="pmid24574837">{{cite journal |vauthors=Kim MY, Sohn KH, Song WJ, Park HW, Cho SH, Min KU, Kang HR |title=Clinical features and prognostic factors of Churg-Strauss syndrome |journal=Korean J. Intern. Med. |volume=29 |issue=1 |pages=85–95 |date=January 2014 |pmid=24574837 |pmc=3932399 |doi=10.3904/kjim.2014.29.1.85 |url=}}</ref>
==Complications==
**Early [[diagnosis]] of the [[disease]]
Complications of Churg-Strauss syndrome depend on the specific organ involved in the disease process.
**Less [[Skin|cutaneous]] involvement
* [[Pericarditis]]
**Elevated [[C-reactive protein]]
* [[Myocarditis]]
*The following are poor [[Prognosis|prognostic factors]]:
* [[MI]]
**Five-Factor Score Assessment (FFS) :<ref name="pmid8569467">{{cite journal |vauthors=Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Casassus P |title=Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients |journal=Medicine (Baltimore) |volume=75 |issue=1 |pages=17–28 |date=January 1996 |pmid=8569467 |doi= |url=}}</ref>
* [[Heart failure]]
***[[Heart|Cardiac]] involvement([[cardiomyopathy]])
* [[Renal failure]]
***Severe GI manifestations([[Ulcer|ulcers]], [[Perforation|perforations]], bleeding)
* [[Cerebral hemorrhage]]
***[[Proteinuria]] > 1g/d
* [[Gastrointestinal bleeding]]
***[[Kidney|Renal]] involvement(Creatinine > 1.4 mg/dl)
* [[Status asthmaticus]]
***[[CNS]] involvement
 
*In 2011, two more criteria are added to to the FFS, include age of the patient >65 years,  ear, nose, and throat involvement. They removed CNS involvement from earlier criteria.<ref name="pmid21200183">{{cite journal |vauthors=Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P |title=The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort |journal=Medicine (Baltimore) |volume=90 |issue=1 |pages=19–27 |date=January 2011 |pmid=21200183 |doi=10.1097/MD.0b013e318205a4c6 |url=}}</ref>
==Prognosis==
**Revised FFS assessment criteria:
In the past the prognosis of Eosinophilic granulomatosis with polyangiitis was fatal when untreated. Patients who were not treated had an estimated 5 year survival rate of 20 to 30%. Today, the prognosis of Eosinophilic granulomatosis with polyangiitis is heavily dependent on starting treatment with a glucocorticoid.  
***[[Cardiomyopathy]]
 
***Severe GI manifestations(ulcers, perforations, bleeding)
The following are favorable prognostic factors:<ref name="pmid24574837">{{cite journal| author=Kim MY, Sohn KH, Song WJ, Park HW, Cho SH, Min KU et al.| title=Clinical features and prognostic factors of Churg-Strauss syndrome. | journal=Korean J Intern Med | year= 2014 | volume= 29 | issue= 1 | pages= 85-95 | pmid=24574837 | doi=10.3904/kjim.2014.29.1.85 | pmc=3932399 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24574837  }}</ref>
***[[Proteinuria]] > 1g/d
* Early diagnosis of the disease
***Renal involvement(Creatinine > 1.6 mg/dl)
* Decreased cutaneous involvement
***Age > 65 yrs
* Elevated C-reactive protein
*The FFS is a good predictor of survival rate. It can be used to choose the appropriate treatment. Renal and gastrointestinal involvement are the most common serious prognostic factors.<ref name="pmid8569467">{{cite journal |vauthors=Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Casassus P |title=Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients |journal=Medicine (Baltimore) |volume=75 |issue=1 |pages=17–28 |date=January 1996 |pmid=8569467 |doi= |url=}}</ref><ref name="pmid22887848">{{cite journal |vauthors=Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, Matthis C, Metzler C, Nölle B, Richardt G, Gross WL |title=A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients |journal=Ann. Rheum. Dis. |volume=72 |issue=6 |pages=1011–7 |date=June 2013 |pmid=22887848 |doi=10.1136/annrheumdis-2012-201531 |url=}}</ref>
 
The following are poor prognostic factors:
 
* These factors are assessed using the Five-Factor Score
* {| class="wikitable" !Five-Factor Score Assessment |- |Proteinuria > than 1 g/d |- |Creatinine > than 140 mm/l |- |Cardiomyopathy |- |Severe gastrointestinal manifestations  |- |CNS involvement |}
According to the Five-Factor Score, a score of 0, 1, or greater than 2 has the following mortality rate:
{| class="wikitable"
{| class="wikitable"
! colspan="2" |Five-Factor Score
!style="background:#4479BA; color: #FFFFFF;" align="center" + |Five factor score
|-
!style="background:#4479BA; color: #FFFFFF;" align="center" + |5 year mortality rate
|Score
|Mortality Rate
|-
|-
|0
|0
|12%
|11.9%
|-
|-
|1
|1
|26%
|25.9%
|-
|-
|>2
|>2
|46%
|45.95%
|}
|}
<ref>{{cite journal |author=Guillevin L, Lhote F, Gayraud M, ''et al'' |title=Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients |journal=Medicine (Baltimore) |volume=75 |issue=1 |pages=17–28 |year=1996 |pmid=8569467 |doi=}}</ref> It has also been concluded that age greater than 65 is also a risk factor for mortality.<ref name="pmid21200183">{{cite journal| author=Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P et al.| title=The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. | journal=Medicine (Baltimore) | year= 2011 | volume= 90 | issue= 1 | pages= 19-27 | pmid=21200183 | doi=10.1097/MD.0b013e318205a4c6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21200183  }}</ref>


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Disease]]
[[Category:Pulmonology]]
[[Category:Autoimmune diseases]]
[[Category:Rheumatology]]


{{WH}}
{{WH}}
{{WS}}
{{WS}}
[[Category: (name of the system)]]

Latest revision as of 17:54, 12 April 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [2]

Overview

Eosinophilic granulomatosis with polyangiitis develops through three phases, include prodromal phase, eosinophilic phase, vasculitic phase. Most complications result from the vasculitic phase. Most common complications include cardiomyopathy, myocardial infarction, perimyocarditis, rapidly progressive renal failure, GI bleeding, neuropathy, and status asthmaticus. Prognosis of eosinophilic granulomatosis with polyangiitis is poor if left untreated. Prognosis is most likely dependent on stage at which the disease was diagnosed and organ involvement. The five-factor score assessment (FFS) is a good predictor of survival rate. It can be used to choose the appropriate treatment.

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

Five factor score 5 year mortality rate
0 11.9%
1 25.9%
>2 45.95%

References

  1. ↑ Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M (April 2015). "Churg-Strauss syndrome". Autoimmun Rev. 14 (4): 341–8. doi:10.1016/j.autrev.2014.12.004. PMID 25500434.
  2. ↑ Aakerøy L, Amundsen BH, Skomsvoll JF, Haugen BO, Soma J (March 2011). "A 50-year-old man with eosinophilia and cardiomyopathy: need for endomyocardial biopsy?". Eur J Echocardiogr. 12 (3): 257–9. doi:10.1093/ejechocard/jeq167. PMID 21138993.
  3. ↑ Lai RS, Lin SL, Lai NS, Lee PC (1998). "Churg-Strauss syndrome presenting with pulmonary capillaritis and diffuse alveolar hemorrhage". Scand. J. Rheumatol. 27 (3): 230–2. PMID 9645420.
  4. ↑ Clutterbuck EJ, Evans DJ, Pusey CD (1990). "Renal involvement in Churg-Strauss syndrome". Nephrol. Dial. Transplant. 5 (3): 161–7. PMID 2113641.
  5. ↑ Kaneki T, Kawashima A, Hayano T, Honda T, Kubo K, Koizumi T, Sekiguchi M, Ichikawa H, Matsuzawa K, Katsuyama T (February 1998). "Churg-Strauss syndrome (allergic granulomatous angitis) presenting with ileus caused by ischemic ileal ulcer". J. Gastroenterol. 33 (1): 112–6. PMID 9497232.
  6. ↑ Wolf J, Bergner R, Mutallib S, Buggle F, Grau AJ (April 2010). "Neurologic complications of Churg-Strauss syndrome--a prospective monocentric study". Eur. J. Neurol. 17 (4): 582–8. doi:10.1111/j.1468-1331.2009.02902.x. PMID 20050889.
  7. ↑ Lestre S, Serrão V, João A, Pinheiro S, Lobo L (2009). "[Churg-Strauss syndrome presenting with cutaneous vasculitis]". Acta Reumatol Port (in Portuguese). 34 (2A): 281–7. PMID 19569284.
  8. ↑ Guillevin L (October 2008). "Advances in the treatments of systemic vasculitides". Clin Rev Allergy Immunol. 35 (1–2): 72–8. doi:10.1007/s12016-007-8068-4. PMID 18181034.
  9. ↑ Kim MY, Sohn KH, Song WJ, Park HW, Cho SH, Min KU, Kang HR (January 2014). "Clinical features and prognostic factors of Churg-Strauss syndrome". Korean J. Intern. Med. 29 (1): 85–95. doi:10.3904/kjim.2014.29.1.85. PMC 3932399. PMID 24574837.
  10. ↑ 10.0 10.1 Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Casassus P (January 1996). "Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients". Medicine (Baltimore). 75 (1): 17–28. PMID 8569467.
  11. ↑ Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P (January 2011). "The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort". Medicine (Baltimore). 90 (1): 19–27. doi:10.1097/MD.0b013e318205a4c6. PMID 21200183.
  12. ↑ Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, Matthis C, Metzler C, Nölle B, Richardt G, Gross WL (June 2013). "A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients". Ann. Rheum. Dis. 72 (6): 1011–7. doi:10.1136/annrheumdis-2012-201531. PMID 22887848.

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