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{{Churg-Strauss syndrome}} | {{Churg-Strauss syndrome}} | ||
{{CMG}} | {{CMG}}; {{AE}}{{CK}} | ||
==Overview== | ==Overview== | ||
[[Eosinophilic granulomatosis with polyangiitis]] previously called [[Eosinophilic granulomatosis with polyangiitis|Churg-Strauss syndrome]] involves primarily small and medium sized blood vessels resulting in vessel inflammation. The exact cause of the disease is not known. However, various environmental factors, [[Allergen|allergens]], [[genetics]], and [[:Category:Drugs|drugs]] may play a role in triggering [[disease]] process by activating different types of white blood cells. The disease is characterized by the presence of [[asthma]], [[hypereosinophilia]], [[rhinosinusitis]], [[Paresthesia|tingling and numbness of hands and feet]] ([[peripheral neuropathy]]) and multiple [[Organ (anatomy)|organ]] involvements including [[skin]], [[Gastrointestinal tract|GI tract]], and [[kidney]]. | |||
==What are the symptoms of Churg-Strauss syndrome | ==What are the symptoms of Churg-Strauss syndrome== | ||
[[Symptom|Symptoms]] of [[eosinophilic granulomatosis with polyangiitis]] typically develops through three phases, include prodromal phase, eosinophilic phase, and vasculitis phase. Although these phases cannot be clinically distinguishable.<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:''' Most common manifestations include | |||
**[[Rhinorrhea|Runny nose]] | |||
** [[Sneeze|Sneezing]] | |||
** [[Nasal polyp|Nasal polyps]] | |||
** [[Rhinosinusitis|Sinusitis]] | |||
**[[Asthma]] | |||
**[[Otalgia|Ear pain]] and discharge | |||
* '''Eosinophilic phase:''' This phase is characterized by [[Eosinophilia|eosinophilia]] and accumulation of [[Eosinophil granulocyte|eosinophils]] in various organs. Most commonly involved organs are [[lung]] and [[Gastrointestinal tract|GI tract]]. | |||
** [[Asthma]] | |||
** [[Cough]] | |||
** [[Dyspnea|Shortness of breath]] | |||
** [[Abdominal pain]] | |||
** GI bleeding | |||
** [[Fatigue]] | |||
* '''Vasculitic phase:''' Symptoms depending on organ system involvement. Most commonly involved organs are lungs, kidneys, skin, heart, and peripheral nerves. | |||
** [[Weight loss]] | |||
** Generalized weakness | |||
** [[Fatigue]] | |||
** [[Rash|Skin rash]] and nodules | |||
** [[Paresthesia|Numbness and tingling sensation of hands and feet]] | |||
** [[Abdominal pain]] | |||
** [[Diarrhea]] | |||
** [[Nausea and vomiting]] | |||
** [[Dyspnea|Shortness of breath]] | |||
** [[Hearing impairment|Hearing loss]] | |||
** [[Chest pain]] | |||
** [[Hematuria|Blood in urine]] | |||
==What causes Churg-Strauss syndrome?== | ==What causes Churg-Strauss syndrome?== | ||
There are no established causes for [[eosinophilic granulomatosis with polyangiitis]]. However, various [[Allergen|allergens]], [[Infection|infections]], [[Vaccination|vaccinations]] and [[:Category:Drugs|drugs]] may act as a triggering agents, and are responsible for developing disease. [[Genetics]] may also play a role. | |||
==Who is at highest risk?== | ==Who is at highest risk?== | ||
Any one can develop [[eosinophilic granulomatosis with polyangiitis]]. Studies shown, it occurs mostly between 40-60 years with people having chronic history of [[asthma]] and allergic manifestations. | |||
==Diagnosis== | ==Diagnosis== | ||
Physical examination of lungs, heart, nervous system, skin and upper airway tract, along with the following tests can help in finding out [[eosinophilic granulomatosis with polyangiitis]]. | |||
* [[Complete blood count|CBC with differential count]] | |||
* [[Chest X-ray]] | |||
* [[Computed tomography|CT scan]] of chest | |||
* Urine analysis | |||
* Serologic test for [[Anti-neutrophil cytoplasmic antibody|anti-neutrophil cytoplasmic antibodies]] ([[Anti-neutrophil cytoplasmic antibody|ANCA]]) | |||
* [[Biopsy]] of lung, skin, kidney and nerve | |||
==When to seek urgent medical care?== | ==When to seek urgent medical care?== | ||
You should see the doctor if you experience any of the following: | |||
* Worsening [[asthma]] | |||
* Severe breathing difficulties | |||
* Worsening [[Rhinosinusitis|sinusitis]], [[facial pain]] and [[rhinitis]] | |||
* Severe [[abdominal pain]] | |||
* [[Hematuria|Blood in urine]] | |||
* severe [[Urticaria|urticarial rash]] | |||
The above [[Symptom|symptoms]] may also present in association with other [[Disease|diseases]], but they have to be evaluated properly by medical professional. | |||
==Treatment Options== | ==Treatment Options== | ||
* Your doctor may suggest treatment with glucocorticoids (eg, predisone) and immunosuppressants that slow down the immune response. | |||
* Medicines used to treat [[eosinophilic granulomatosis with polyangiitis]] include: | |||
** [[Prednisone]] | |||
** [[Cyclophosphamide]] | |||
** [[Azathioprine]] | |||
** [[Methotrexate]] | |||
** [[Rituximab]] | |||
** Interferon alpha | |||
* These medicines may cause serious side effects. You should carefully discuss your treatment plan with your doctor. Other medicines may be prescribed, including: | |||
** [[Bisphosphonate]]: Prevent bone loss caused by [[prednisone]] | |||
** [[Folic Acid|Folic acid]]: If you are taking [[methotrexate]] | |||
** [[Sulfamethoxazole-Trimethoprim|Trimethoprim/sulfamethoxazole]]: Lung infections | |||
* Some patients may be considered for [[Surgery|surgical therapy]] to relieve symptoms of persistent [[Rhinosinusitis|sinusitis]] and [[Hearing impairment|hearing loss]]. | |||
==Where to find medical care for Churg-Strauss syndrome== | ==Where to find medical care for Churg-Strauss syndrome== | ||
[http://maps.google.com/maps?hl=en&tab=wl&q=wegener%27s%20granulomatosis Directions to Hospitals Treating eosinophilic granulomatosis with polyangiitis] | |||
==Prevention== | ==Prevention== | ||
Currently, there are no established preventive measures for [[eosinophilic granulomatosis with polyangiitis]]. | |||
==What to expect (Outlook/Prognosis)?== | ==What to expect (Outlook/Prognosis)?== | ||
* Without treatment, people with [[eosinophilic granulomatosis with polyangiitis]] can die within a few months. | |||
* With treatment, the outlook for most patients is good. | |||
* Studies have shown that most people who receive and [[cyclophosphamide]] are doing much better. However, the [[disease]] relapses are common. | |||
==Possible Complications== | ==Possible Complications== | ||
Without treatment, [[eosinophilic granulomatosis with polyangiitis]] can result in the following complications. Complications depend on organ system involvement. | |||
* [[Heart|'''Heart''']]: | |||
** [[Pericarditis]] | |||
** [[Myocarditis]] | |||
** [[Pericardial effusion]] | |||
** [[Myocardial infarction]] | |||
** [[Congestive heart failure]] | |||
* [[Lung|'''Lung''']]: | |||
** [[Pleural effusion]] | |||
** [[Pulmonary hypertension]] | |||
** [[Status asthmaticus]] | |||
* [[Kidney|'''Kidney''']]: | |||
** [[Glomerulonephritis]] | |||
** [[Renal insufficiency|Renal failure]] | |||
* [[Gastrointestinal tract|'''GI tract''']]: | |||
** [[Gastrointestinal bleeding]] | |||
** [[Abdominal pain|Acute abdominal pain]] or intestinal angina | |||
* '''Neurologic''': | |||
** [[peripheral neuropathy]] | |||
** [[Intracranial hemorrhage|Cerebral hemorrhage]] | |||
* [[Skin|'''Skin''']]: | |||
** [[Purpura]] | |||
** [[Petechia|Petichiae]] | |||
** Noduluar lesions on extensor surfaces | |||
==Sources== | ==Sources== | ||
==References== | ==References== | ||
Latest revision as of 20:23, 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 previously called Churg-Strauss syndrome involves primarily small and medium sized blood vessels resulting in vessel inflammation. The exact cause of the disease is not known. However, various environmental factors, allergens, genetics, and drugs may play a role in triggering disease process by activating different types of white blood cells. The disease is characterized by the presence of asthma, hypereosinophilia, rhinosinusitis, tingling and numbness of hands and feet (peripheral neuropathy) and multiple organ involvements including skin, GI tract, and kidney.
What are the symptoms of Churg-Strauss syndrome
Symptoms of eosinophilic granulomatosis with polyangiitis typically develops through three phases, include prodromal phase, eosinophilic phase, and vasculitis phase. Although these phases cannot be clinically distinguishable.[1]
- Prodromal phase: Most common manifestations include
- Runny nose
- Sneezing
- Nasal polyps
- Sinusitis
- Asthma
- Ear pain and discharge
- Eosinophilic phase: This phase is characterized by eosinophilia and accumulation of eosinophils in various organs. Most commonly involved organs are lung and GI tract.
- Asthma
- Cough
- Shortness of breath
- Abdominal pain
- GI bleeding
- Fatigue
- Vasculitic phase: Symptoms depending on organ system involvement. Most commonly involved organs are lungs, kidneys, skin, heart, and peripheral nerves.
- Weight loss
- Generalized weakness
- Fatigue
- Skin rash and nodules
- Numbness and tingling sensation of hands and feet
- Abdominal pain
- Diarrhea
- Nausea and vomiting
- Shortness of breath
- Hearing loss
- Chest pain
- Blood in urine
What causes Churg-Strauss syndrome?
There are no established causes for eosinophilic granulomatosis with polyangiitis. However, various allergens, infections, vaccinations and drugs may act as a triggering agents, and are responsible for developing disease. Genetics may also play a role.
Who is at highest risk?
Any one can develop eosinophilic granulomatosis with polyangiitis. Studies shown, it occurs mostly between 40-60 years with people having chronic history of asthma and allergic manifestations.
Diagnosis
Physical examination of lungs, heart, nervous system, skin and upper airway tract, along with the following tests can help in finding out eosinophilic granulomatosis with polyangiitis.
- CBC with differential count
- Chest X-ray
- CT scan of chest
- Urine analysis
- Serologic test for anti-neutrophil cytoplasmic antibodies (ANCA)
- Biopsy of lung, skin, kidney and nerve
When to seek urgent medical care?
You should see the doctor if you experience any of the following:
- Worsening asthma
- Severe breathing difficulties
- Worsening sinusitis, facial pain and rhinitis
- Severe abdominal pain
- Blood in urine
- severe urticarial rash
The above symptoms may also present in association with other diseases, but they have to be evaluated properly by medical professional.
Treatment Options
- Your doctor may suggest treatment with glucocorticoids (eg, predisone) and immunosuppressants that slow down the immune response.
- Medicines used to treat eosinophilic granulomatosis with polyangiitis include:
- Prednisone
- Cyclophosphamide
- Azathioprine
- Methotrexate
- Rituximab
- Interferon alpha
- These medicines may cause serious side effects. You should carefully discuss your treatment plan with your doctor. Other medicines may be prescribed, including:
- Bisphosphonate: Prevent bone loss caused by prednisone
- Folic acid: If you are taking methotrexate
- Trimethoprim/sulfamethoxazole: Lung infections
- Some patients may be considered for surgical therapy to relieve symptoms of persistent sinusitis and hearing loss.
Where to find medical care for Churg-Strauss syndrome
Directions to Hospitals Treating eosinophilic granulomatosis with polyangiitis
Prevention
Currently, there are no established preventive measures for eosinophilic granulomatosis with polyangiitis.
What to expect (Outlook/Prognosis)?
- Without treatment, people with eosinophilic granulomatosis with polyangiitis can die within a few months.
- With treatment, the outlook for most patients is good.
- Studies have shown that most people who receive and cyclophosphamide are doing much better. However, the disease relapses are common.
Possible Complications
Without treatment, eosinophilic granulomatosis with polyangiitis can result in the following complications. Complications depend on organ system involvement.
- Heart:
- Lung:
- Kidney:
- GI tract:
- Gastrointestinal bleeding
- Acute abdominal pain or intestinal angina
- Neurologic:
- Skin:
Sources
References
- ↑ 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.