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{{DiseaseDisorder infobox |
{{Churg-Strauss syndrome}}
  Name          = Churg-Strauss syndrome |
{{CMG}}{{APM}}{{AE}}{{CK}}
  ICD10          = {{ICD10|M|30|1|m|30}} |
  ICD9          = {{ICD9|446.4}} |
  ICDO          = |
  Image          = |
  Caption        = |
  OMIM          = |
  OMIM_mult      = |
  MedlinePlus    = |
  DiseasesDB    = 2685 |
  MeshID        = D015267 |
}}
{{SI}}


{{CMG}}; {{AE}} {{CZ}} {{RT}}
{{SK}} Allergic granulomatosis; eosinophilic granulomatosis with polyangiitis, Churg-Strauss syndrome


==Overview==
==[[Churg-Strauss syndrome overview|Overview]]==
'''Churg-Strauss syndrome''' (also known as ''allergic [[granulomatosis]]'') 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.  It is a rare disease that is non-heritable, non-transmissable and often mis-diagnosed. Churg-Strauss syndrome was once considered a type of [[Polyarteritis nodosa|Polyarteritis nodosa (PAN)]] due to their similar morphologies.


==Historical Perspective==
==[[Churg-Strauss syndrome historical perspective|Historical Perspective]]==
It is named for Dr Jacob Churg and Dr Lotte Strauss, who described the condition in 1951.<ref>{{WhoNamedIt|synd|2733}}</ref><ref>{{cite journal |author=Churg J, Strauss L |title=Allergic granulomatosis, allergic angiitis, and periarteritis nodosa |journal=Am. J. Pathol. |volume=27 |issue=2 |pages=277-301 |year=1951 |pmid=14819261 |doi=}}</ref>


==[[Churg-Strauss syndrome classification|Classification]]==


==Pathophysiology==
==[[Churg-Strauss syndrome pathophysiology|Pathophysiology]]==


===Associated Diseases===
==[[Churg-Strauss syndrome causes|Causes]]==
* 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%)
*:* Constrictive disease
*:* 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 rare (contrast Wegener’s)
* GI Disease
*:* Eosinophilic gastroenteritis with abdominal pain (59%)
*:* Diarrhea (33%)
*:* GI bleeding (18%)


===Microscopic Pathology===
==[[Differentiating Churg-Strauss syndrome from other diseases|Differentiating Churg-Strauss syndrome from other Diseases]]==
* 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.]]
==[[Churg-Strauss syndrome epidemiology and demographics|Epidemiology and Demographics]]==


==[[Churg-Strauss syndrome risk factors|Risk Factors]]==


==Epidemiology and Demographics==
==[[Churg-Strauss syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
The mean age at diagnosis is 50. The initial onset of symptoms may be 10-20 years earlier. There is no gender predominance.


==Natural History, Complications and Prognosis==
==Diagnosis==
===Natural History===
[[Churg-Strauss syndrome history and symptoms| History and Symptoms]] | [[Churg-Strauss syndrome physical examination | Physical Examination]] | [[Churg-Strauss syndrome laboratory findings|Laboratory Findings]] | [[Churg-Strauss syndrome chest x ray|Chest X Ray]] |  [[Churg-Strauss syndrome CT|CT]] | [[Churg-Strauss syndrome MRI|MRI]] | [[Churg-Strauss syndrome other imaging findings|Other Imaging Findings]] | [[Churg-Strauss syndrome other diagnostic studies|Other Diagnostic Studies]]
Classically, three phases of the disease are described:


====Stage 1: The Prodromal Phase====
==Treatment==
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]]).
[[Churg-Strauss syndrome medical therapy|Medical Therapy]] | [[Churg-Strauss syndrome surgery|Surgery]] | [[Churg-Strauss syndrome primary prevention|Primary Prevention]] | [[Churg-Strauss syndrome secondary prevention|Secondary Prevention]] |  [[Churg-Strauss syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Churg-Strauss syndrome future or investigational therapies|Future or Investigational Therapies]]


====Stage 2: The Eosinophilic Phase====
==Case Studies==
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.
[[Churg-Strauss syndrome case study one|Case#1]]


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.
== Diagnosis ==
The diagnosis of Churg-Strauss syndrome can be difficult to make clinically for the following reasons:
* Individual manifestations of the syndrome can occur in isolation
* 40% of patients present with pulmonary opacities, [[asthma]], and [[eosinophilia]] prior to systemic vasculitis, and granulomatosis, vasculitis, and [[eosinophilia]] are features of other diseases as well
* Lung involvement is not universal
* Chronic eosinophilic [[pneumonia]] may be the presenting feature
===American College of Rheumatology Diagnostic Criteria===
The presence of 4 of the 6 criteria below is 85% sensitive and 99.7% specific:
# [[Asthma]] (history of wheezing or diffuse high-pitched wheezes on expiration)
# [[Eosinophilia]] (>10%)
# [[Neuropathy]] (mono, mono-multiplex, or poly)
# Migratory or transient pulmonary opacities on chest x-ray
# Paranasal sinus abnormality
# Biopsy containing a blood vessel with [[eosinophilia]] (eos) in extravascular areas
===History and Symptoms===
Symptoms can include:
* [[Fever]]
* [[Weight loss]]
* [[Malaise]]
* [[Asthenia]]
===Laboratory Findings===
====Electrolyte and Biomarker Studies====
Diagnostic markers include [[eosinophil granulocyte]]s and [[granuloma]]s in affected tissue and [[Anti-neutrophil cytoplasmic antibody|Anti-neutrophil cytoplasmic antibodies (ANCA)]] against [[neutrophil granulocyte]]s.  Differentiation from [[Wegener's granulomatosis]] can be difficult, though the increasing use of ANCA assays has made the distinction more routine.  Wegener's is closely associated with c-ANCA, unlike Churg-Strauss which shows elevations of p-ANCA.
The findings on other laboratory studies is nonspecific. [[Eosinophilia]] (5K-9K/uL) may be present. [[Anemia]], elevated [[erythrocyte sedimentation rate]] ([[ESR]]), [[leukocytosis]], elevated [[immunoglobin E]] ([[IgE]]), [[hypergammaglobulinemia]], and a low-positive [[rheumatoid factor]] ([[RF]]) may be present.
===Imaging===
====X-ray====
[[Image:Churg strauss cxr.jpg|left|thumb|CXR in a patient with Churg-Strauss syndrome showing opacification, hilar adenopathy]]
* Interlobular septal thickening with or without associated peripheral [[Ground glass opacification on CT|ground glass opacities]] or consolodation
* Transient opacities
* Symmetrical opacities in axillary and peripheral distribution
* Hilar with hilar adenopathy
* Diffuse interstitial or miliary opacities
* Pulmonary hemorrhage
* Nodular disease (without cavitation)
* [[Pleural effusion]]s (30%) – exudative and eosinophilic
<ref>Fraser RS, Müller NL, Colman N, Paré PD. Diagnosis of diseases of the chest. Fourth edition. WB Saunders Company. Philadelphia, 1999.</ref> <ref>Worthy AS, Müller NL, Hansel DM, Flower CDR. Churg-Strauss Syndrome: The spectrum of pulmonary CT findings in 17 patients. AJR 1998;170:297-300.</ref> <ref>Johkoh T, Müller NL, Akira M, Ichikado K et al. Eosinophilic lung diseases: Diagnostic accuracy of thin-section CT in 111 patients. Radiology 2000;216:773-780.</ref>
==Risk stratification==
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),
(3) gastrointestinal hemorrhage, infarction or [[pancreatitis]],
(4) involvement of the [[central nervous system]] or
(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>
== Treatment ==
===Acute Pharmacotherapy===
Treatment for Churg-Strauss syndrome includes glucocorticoids such as [[prednisone]] at a dose of 0.5-1.5 mg/kg per day x 6-12 weeks and other immunosupressive drugs such as [[azathioprine]] and [[cyclophosphamide]]. In many cases the disease can be put into a type of chemical remission through drug therapy, but the disease is chronic and life long.
A [[systematic review]] conducted in 2007 indicated that all patients should be treated with high-dose steroids, but that in patients with an FFS of 1 or higher cyclophosphamide pulse therapy should be commenced, with 12 pulses leading to less relapses than 6. Remission can be maintained with a less toxic drug, such as azathioprine or [[methotrexate]].<ref>{{cite journal |author=Bosch X, Guilabert A, Espinosa G, Mirapeix E |title=Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review |journal=JAMA |volume=298 |issue=6 |pages=655–69 |year=2007 |pmid=17684188 |doi=10.1001/jama.298.6.655}}</ref>
The [[erythrocyte sedimentation rate]] ([[ESR]]) and [[eosinophil]] count can be followed to gauge the response to therapy. Late relapses are uncommon and refractory disease may require [[Cyclophosphamide]], [[Azathioprine]], intravenous immunoglobulin (IVIG) and [[plasmapheresis]].
==References==
{{reflist|2}}


==Suggested Reading==
==Suggested Reading==

Latest revision as of 20:35, 10 April 2018

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Synonyms and keywords: Allergic granulomatosis; eosinophilic granulomatosis with polyangiitis, Churg-Strauss syndrome

Overview

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Pathophysiology

Causes

Differentiating Churg-Strauss syndrome from other Diseases

Epidemiology and Demographics

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Case#1


Suggested Reading

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