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{{Churg-Strauss syndrome}}
{{Churg-Strauss syndrome}}
 
{{CMG}}; {{AE}} {{CK}}  
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==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 the disease are described:
 
====Stage 1: The Prodromal Phase====
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]]).
 
====Stage 2: The Eosinophilic Phase====
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.
 
People can live for many years in the first two stages before progressing to stage three.
 
====Stage 3: 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.  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.
 
==Complications==
Complications of Churg-Strauss syndrome depend on the specific organ involved in the disease process.
* [[Pericarditis]]
* [[Myocarditis]]
* [[MI]]
* [[Heart failure]]
* [[Renal failure]]
* [[Cerebral hemorrhage]]
* [[Gastrointestinal bleeding]]
* [[Status asthmaticus]]
 
==Prognosis==
* Churg-Strauss syndrome is a serious disease that can be fatal if untreated.
* Untreated patients have a 5 yr survival rate of 20%-30%
 
Prior to the steroid treatment, the disease was uniformly fatal.  Now, 5 year survival tops 70%.  In general, poor prognostic indicators include cardiac involvement, GI disease, [[renal insufficiency]] (Cr >1.6), [[proteinuria]] (>1 g/day) and central nervous system (CNS) involvement.  A shorter duration of asthma before the onset of vasculitis also portends poorer prognosis.  The role of anti-neutrophil cytoplasmic antibodies (ANCA) in predicting outcome is unclear.
 
The French Vasculitis Study Group has developed a five-point score ("five-factor score" or FFS) that predicts the risk of death in Churg-Strauss syndrome.  These are
 
(1) reduced [[renal function]] (creatinine >1.58 mg/dL or 140 μmol/l),


(2) [[proteinuria]] (>1 g/24h),  
===Natural History===
*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]]


(3) gastrointestinal hemorrhage, infarction or [[pancreatitis]],  
===Complications===
*[[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


(4) involvement of the [[central nervous system]] or
===Prognosis===
*Prognosis of [[eosinophilic granulomatosis with polyangiitis]] is poor if left untreated.
*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>
*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>
**Early [[diagnosis]] of the [[disease]]
**Less [[Skin|cutaneous]] involvement
**Elevated [[C-reactive protein]]
*The following are poor [[Prognosis|prognostic factors]]:
**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|Cardiac]] involvement([[cardiomyopathy]])
***Severe GI manifestations([[Ulcer|ulcers]], [[Perforation|perforations]], bleeding)
***[[Proteinuria]] > 1g/d
***[[Kidney|Renal]] involvement(Creatinine > 1.4 mg/dl)
***[[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>
**Revised FFS assessment criteria:
***[[Cardiomyopathy]]
***Severe GI manifestations(ulcers, perforations, bleeding)
***[[Proteinuria]] > 1g/d
***Renal involvement(Creatinine > 1.6 mg/dl)
***Age > 65 yrs
*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>
{| class="wikitable"
!style="background:#4479BA; color: #FFFFFF;" align="center" + |Five factor score
!style="background:#4479BA; color: #FFFFFF;" align="center" + |5 year mortality rate
|-
|0
|11.9%
|-
|1
|25.9%
|-
|>2
|45.95%
|}


(5) [[cardiomyopathy]].
Presence of 1 of these indicates severe disease (5-year mortality 26%) and 2 or more very severe disease (mortality 46%), while absence of any of these 5 indicates a milder case (mortality 11.9%).<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>
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Disease]]
[[Category:Pulmonology]]
[[Category:Autoimmune diseases]]
[[Category:Rheumatology]]


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{{WS}}
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[[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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