Eosinophilic granulomatosis with polyangiitis: Difference between revisions

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{{Infobox medical condition (new)  
{{Infobox medical condition (new)
| name           = Eosinophilic granulomatosis with polyangiitis (EGPA)  
| name = Eosinophilic Granulomatosis with Polyangiitis (EGPA)
| image           = Churg-Strauss syndrome - high mag.jpg  
| image = Churg-Strauss syndrome - high mag.jpg
| caption         = [[Micrograph]] showing an eosinophilic [[vasculitis]] consistent with eosinophilic granulomatosis with polyangiitis. [[H&E stain]]. One of the [[American College of Rheumatology]] criteria for EGPA is extravascular eosinophil infiltration on biopsy.<ref name="ACR1990">{{cite journal | vauthors = Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY | display-authors = 6 | title = The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis) | journal = Arthritis and Rheumatism | volume = 33 | issue = 8 | pages = 1094–100 | date = August 1990 | pmid = 2202307 | doi = 10.1002/art.1780330806 }}</ref>
| caption = [[Micrograph]] showing eosinophilic [[vasculitis]] consistent with eosinophilic granulomatosis with polyangiitis. [[H&E stain]].
|
| pronounce =
| pronounce       =
| field = [[Rheumatology]], [[Immunology]]
| field           =
| synonyms = '''Churg–Strauss Syndrome''', '''Allergic Angiitis and Granulomatosis'''
| synonyms       = '''Churg–Strauss syndrome''', '''allergic angiitis and granulomatosis'''.<ref name="NORD (National Organization for Rare Disorders) 2015">{{cite web | title=Churg Strauss Syndrome | website=NORD (National Organization for Rare Disorders) | date=2015-02-11 | url=https://rarediseases.org/rare-diseases/churg-strauss-syndrome/ | access-date=2020-03-08}}</ref>
| symptoms = Fatigue, fever, weight loss, night sweats, [[asthma]], [[arthralgia]], [[myalgia]], [[purpura]], [[urticaria]], skin nodules, cough, nasal polyps, [[sinusitis]], [[neuropathy]], gastrointestinal distress.
| symptoms       = Fatigue, fever, weight loss, night sweats, abdominal pain, cough, [[arthralgia|joint pain]], [[myalgia|muscle pain]], [[purpura|bleeding into tissues under the skin]], [[urticaria|a rash with hives]], [[Nodule_(medicine)|small bumps]], or [[malaise|a general feeling of ill]].<ref name="NORD (National Organization for Rare Disorders) 2015"/>
| complications = [[Hypereosinophilia]], [[vasculitis]], [[cardiac disease]], [[neuropathy]], [[renal failure]], [[pulmonary involvement]].
| complications   = [[hypereosinophilia]], [[granulomatosis]], [[vasculitis]], [[serous otitis media|inner ear infections with fluid build up]], [[conjunctiva|inflammation of the moist membrane lining the surface of the eyelids]], or [[neuropathy|inflammation of peripheral nerves]].<ref name="NORD (National Organization for Rare Disorders) 2015"/>
| onset = Gradual, typically in adulthood, often in individuals with a history of asthma or allergic conditions.
| onset           =  
| duration = Chronic with periods of remission and flare-ups.
| duration       =  
| types = Systemic necrotizing [[vasculitis]] affecting small-to-medium blood vessels.
| types           =  
| causes = Autoimmune response; associated with [[antineutrophil cytoplasmic antibodies]] (ANCA).
| causes         =  
| risks = History of [[asthma]], [[allergic rhinitis]], [[eosinophilia]], exposure to allergens or environmental triggers.
| risks           = History of [[allergy]], [[asthma]] and asthma-associated lung abnormalities (i.e., pulmonary infiltrates).<ref name="NORD (National Organization for Rare Disorders) 2015"/>
| diagnosis = Blood tests for eosinophilia and ANCA, imaging studies, tissue biopsy, clinical criteria (American College of Rheumatology).
| diagnosis      = [[antineutrophil cytoplasmic antibodies]] (ANCA); cluster of asthma, [[eosinophilia]], mono- or polyneuropathy, nonfixed pulmonary infiltrates, abnormality of the [[paranasal sinuses]], and extravascular eosinophilia.<ref name="NORD (National Organization for Rare Disorders) 2015"/>
| differential = [[Granulomatosis with polyangiitis]], [[hypereosinophilic syndrome]], [[microscopic polyangiitis]], [[sarcoidosis]], [[asthma]].
| differential    =  
| prevention = No known prevention; early treatment can help manage disease progression.
| prevention     =
| treatment = [[Immunosuppression]] with corticosteroids and immunomodulatory drugs.
| treatment       = [[immunosuppression|Suppress the activity of the immune system]] to alleviate inflammation. <ref name="NORD (National Organization for Rare Disorders) 2015"/>
| medication = [[Corticosteroids]] (e.g., [[prednisone]]), [[mepolizumab]], [[cyclophosphamide]], [[azathioprine]], [[methotrexate]].
| medication     = [[Corticosteroid]] medications such as [[prednisone]] or [[methylprednisolone]], and [[mepolizumab]].<ref name="NORD (National Organization for Rare Disorders) 2015"/> [[cytotoxic|Proliferation inhibitor]] for those with the presence of kidney or neurological disease.<ref name="NORD (National Organization for Rare Disorders) 2015"/>
| prognosis = Variable; some achieve remission, while others develop progressive complications.
| prognosis       =  
| frequency = Rare; estimated prevalence of 10–15 cases per million.
| frequency       =  
| deaths = Can be fatal if untreated, particularly due to cardiac or pulmonary complications.
| deaths         =  
}}
}}


[[File:Vasculitis after 3 days therapy 2.jpg|thumb|Vasculitis after 3 days therapy]]
'''Eosinophilic Granulomatosis with Polyangiitis''' (EGPA), formerly known as '''Churg–Strauss Syndrome''', is a rare [[autoimmune disease]] characterized by [[vasculitis]] affecting small and medium-sized blood vessels. It primarily occurs in individuals with a history of [[asthma]] or other allergic conditions, and is distinguished by elevated levels of [[eosinophils]] in the blood and tissues, leading to widespread inflammation.


'''Eosinophilic granulomatosis with polyangiitis''' ('''EGPA'''), formerly known as  '''allergic granulomatosis,'''<ref name="Churg" />{{sfn|Adu|Emery|Madaio|2012|p=125}} is an extremely rare [[autoimmune disease|autoimmune]] condition that causes [[inflammation]] of small and medium-sized blood vessels ([[vasculitis]]) in persons with a history of airway allergic hypersensitivity ([[atopy]]).<ref name="WebMD 2019">{{cite web | title=What Is Churg-Strauss Syndrome? | website=WebMD | date=2019-01-30 | url=https://www.webmd.com/lung/churg-strauss-syndrome | access-date=2020-03-08}}</ref>
== Pathophysiology ==
EGPA is considered an [[antineutrophil cytoplasmic antibody]] (ANCA)-associated [[vasculitis]], though not all cases are ANCA-positive. The disease progresses in three overlapping stages:
* Allergic stage: Characterized by long-standing or newly developed [[asthma]], [[allergic rhinitis]], and [[nasal polyps]].
* Eosinophilic stage: Marked by high levels of [[eosinophilia]] (greater than 10% of total white blood cells), leading to [[pulmonary infiltrates]], [[gastrointestinal damage]], and [[cardiac involvement]].
* Vasculitic stage: The most severe phase, where systemic [[vasculitis]] affects multiple organs, causing [[neuropathy]], [[glomerulonephritis]], [[myocarditis]], and [[skin lesions]].


It usually manifests in three stages. The early ([[prodrome|prodromal]]) stage is marked by airway inflammation; almost all patients experience [[asthma]] and/or [[allergic rhinitis]]. The second stage is characterized by abnormally high numbers of eosinophils ([[hypereosinophilia]]), which causes tissue damage, most commonly to the [[lung]]s and the [[Human gastrointestinal tract|digestive tract]].<ref name="WebMD 2019"/> The third stage consists of vasculitis, which can eventually lead to [[necrosis|cell death]] and can be life-threatening.<ref name="WebMD 2019"/>
== Signs and Symptoms ==
 
EGPA manifests with a wide range of symptoms, which vary depending on the stage and organ involvement. Common symptoms include:
<youtube>
* Respiratory symptoms: Severe [[asthma]], sinusitis, nasal polyps, chronic cough, lung infiltrates.
title='''{{PAGENAME}}'''
* Skin manifestations: [[Purpura]], [[urticaria]], nodules, [[livedo reticularis]], or necrotic lesions.
movie_url=http://www.youtube.com/v/wuEysh3MmYY
* Neurological involvement: Peripheral [[neuropathy]] such as [[mononeuritis multiplex]], characterized by muscle weakness, numbness, and pain.
&rel=1
* Gastrointestinal issues: Abdominal pain, diarrhea, gastrointestinal bleeding, eosinophilic colitis.
embed_source_url=http://www.youtube.com/v/wuEysh3MmYY
* Cardiovascular complications: [[Myocarditis]], pericarditis, arrhythmias, [[heart failure]].
&rel=1
* Renal involvement: Rare but can include [[glomerulonephritis]].
wrap = yes
* General symptoms: Weight loss, fever, fatigue, night sweats, joint and muscle pain.
width=750
height=600
</youtube>
 
This condition is now called "eosinophilic granulomatosis with polyangiitis" to remove all eponyms from the [[vasculitis|vasculitides]]. To facilitate the transition, it was referred to as "eosinophilic granulomatosis with polyangiitis (Churg–Strauss)" for a period of time starting in 2012.<ref>{{cite journal | vauthors = Montesi SB, Nance JW, Harris RS, Mark EJ | title = CASE RECORDS of the MASSACHUSETTS GENERAL HOSPITAL. Case 17-2016. A 60-Year-Old Woman with Increasing Dyspnea | journal = The New England Journal of Medicine | volume = 374 | issue = 23 | pages = 2269–79 | date = June 2016 | pmid = 27276565 | doi = 10.1056/NEJMcpc1516452 }}</ref> Prior to this it was known as "Churg–Strauss syndrome", named after Drs. [[Jacob Churg]] and [[Lotte Strauss]] who, in 1951, first published about the syndrome using the term "allergic granulomatosis" to describe it.<ref name="Churg" /> It is a type of [[systemic necrotizing vasculitis]].
 
Effective treatment of EGPA requires [[immunosuppression|suppression of the immune system]] with medication. This is typically [[glucocorticoid]]s, followed by other agents such as [[cyclophosphamide]] or [[azathioprine]].
 
== Signs and symptoms ==
Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order;<ref name=mayo-s>{{cite web|title=Churg-Strauss syndrome - Symptoms|url=http://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/home/ovc-20232243|publisher=[[Mayo Clinic]]|access-date=30 June 2013}}</ref> whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and [[heart disease]], some patients are only mildly affected, e.g. with [[cutaneous condition|skin lesions]] and [[nasal polyps]].<ref name=oxford4111>{{cite journal | vauthors = Della Rossa A, Baldini C, Tavoni A, Tognetti A, Neglia D, Sambuceti G, Puccini R, Colangelo C, Bombardieri S | display-authors = 6 | title = Churg-Strauss syndrome: clinical and serological features of 19 patients from a single Italian centre | journal = Rheumatology | volume = 41 | issue = 11 | pages = 1286–94 | date = November 2002 | pmid = 12422002 | doi = 10.1093/rheumatology/41.11.1286 | url = http://rheumatology.oxfordjournals.org/content/41/11/1286.long | doi-access = free }}</ref> EGPA is consequently considered a highly variable condition in terms of its presentation and its course.<ref name=mayo-s/><ref name=oxford4111/>
 
== Allergic stage ==
The prodromal stage is characterized by allergy. Almost all patients experience asthma and/or allergic rhinitis,{{sfn|Churg|Thurlbeck|1995|p=425}} with more than 90% having a history of asthma that is either a new development, or the worsening of pre-existing asthma,<ref name=clim701>{{cite book|last=Rich|first=Robert R. | name-list-format = vanc |title=Clinical Immunology: Principles and Practice|year=2012|publisher=[[Elsevier|Elsevier Health Sciences]]|isbn=9780723437109|page=701|author2=Fleisher, Thomas A. |author3=Shearer, William T. |author4=Schroeder, Harry |author5=Frew, Anthony J. |author6= Weyand, Cornelia M. }}</ref> which may require systemic [[corticosteroid]] treatment.<ref name=mayo-s/> On average, asthma develops from three to nine years before the other signs and symptoms.<ref name=mayo-s/>
 
The allergic rhinitis may produce symptoms such as [[rhinorrhea]] and [[nasal obstruction]], and the formation of [[nasal polyp]]s that require surgical removal, often more than once.{{sfn|Churg|Thurlbeck|1995|p=425}} [[Sinusitis]] may also be present.{{sfn|Churg|Thurlbeck|1995|p=425}}
 
== Eosinophilic stage ==
The second stage is characterized by [[hypereosinophilia|an abnormally high level of eosinophils]] (a type of [[white blood cell]]) in the blood and tissues.<ref name=mayo-s/>  The symptoms of hypereosinophilia depend on which part of the body is affected, but most often it affects the lungs and digestive tract.<ref name=mayo-s/> The signs and symptoms of hypereosinophilia may include weight loss, night sweats, asthma, cough, abdominal pain, and gastrointestinal bleeding.<ref name=mayo-s/> [[Fever]] and [[malaise]] are often present.{{sfn|Churg|Thurlbeck|1995|p=426}}
 
The eosinophilic stage can last months or years, and its symptoms can disappear, only to return later.<ref name=mayo-s/> Patients may experience the third stage simultaneously.<ref name=mayo-s/>
 
== Vasculitic stage ==
The third and final stage, and hallmark of EGPA, is [[vasculitis|inflammation of the blood vessels]], and the consequent reduction of blood flow to various organs and tissues.<ref name=mayo-s/> Local and systemic symptoms become more widespread and are compounded by new symptoms from the vasculitis.{{sfn|Churg|Thurlbeck|1995|p=426}}
 
Severe complications may arise. [[Thrombosis|Blood clots]] may develop within the damaged arteries in severe cases, particularly in arteries of the abdominal region, which is followed by [[infarction]] and [[necrosis|cell death]], or slow [[atrophy]].{{sfn|Churg|Thurlbeck|1995|p=426}} Many patients experience severe abdominal complaints; these are most often due to [[peritonitis]] and/or ulcerations and [[Gastrointestinal perforation|perforations]] of the gastrointestinal tract, but occasionally due to [[Cholecystitis|acalculous cholecystitis]] or granulomatous [[appendicitis]].{{sfn|Churg|Thurlbeck|1995|p=426}}
 
The most serious complication of the vasculitic stage is [[cardiovascular disease|heart disease]], which is the cause of nearly one-half of all deaths in patients with EGPA.{{sfn|Churg|Thurlbeck|1995|p=426}} Among heart disease-related deaths, the most usual cause is [[myocarditis|inflammation of the heart muscle]] caused by the high level of eosinophils, although some are deaths due to inflammation of the [[coronary artery|arteries that supply blood to the heart]] or [[Cardiac tamponade|pericardial tamponade]].{{sfn|Churg|Thurlbeck|1995|p=426}} [[Renal|Kidney]] complications have been reported as being less common.{{sfn|Rich|Fleisher|Shearer|Schroeder|2012|p=701}}


== Diagnosis ==
== Diagnosis ==
Diagnostic markers include [[eosinophil granulocyte]]s and [[granuloma]]s in affected tissue, and [[anti-neutrophil cytoplasmic antibody|antineutrophil cytoplasmic antibodies]] (ANCA) against [[neutrophil granulocyte]]s. The [[American College of Rheumatology]] 1990 criteria for diagnosis of Churg–Strauss syndrome lists these criteria:{{update inline|date=April 2020}}
EGPA is diagnosed based on a combination of clinical symptoms, laboratory findings, and histopathological examination. Diagnostic criteria include:
*Asthma
* Blood tests:
*[[Eosinophilia]], i.e. eosinophil blood count greater than 500/microliter, or [[hypereosinophilia]], i.e. eosinophil blood count greater than 1,500/microliter
* Elevated eosinophil count (>1,500/microliter).
*Presence of [[mononeuropathy]] or [[polyneuropathy]]
* Presence of ANCA antibodies (positive in ~40% of cases).
*Unfixed pulmonary infiltrates
* Elevated [[C-reactive protein]] (CRP) and [[erythrocyte sedimentation rate]] (ESR).
*Presence of [[paranasal sinuses|paranasal sinus]] abnormalities
* Increased [[IgE]] levels.
*[[Histology|Histological]] evidence of extravascular eosinophils
* Imaging:
 
* Chest X-ray or CT scan showing lung infiltrates or nodules.
For classification purposes, a patient shall be said to have EGPA if at least four of these six criteria are positive. The presence of any four or more of the six criteria yields a sensitivity of 85% and a specificity of 99.7%.<ref name="ACR1990"/>{{update inline|date=April 2020}}
* MRI or nerve conduction studies to assess neuropathy.
 
* [[Biopsy]]:
===Risk stratification===
* Tissue biopsy from affected organs (e.g., skin, lung, nerve) demonstrating vasculitis, eosinophilic infiltration, and granuloma formation.
The French Vasculitis Study Group has developed a five-point system ("five-factor score") that predicts the risk of death in Churg–Strauss syndrome using clinical presentations. These factors are:
* American College of Rheumatology (ACR) Classification Criteria:
*Reduced [[renal function]] (creatinine >1.58&nbsp;mg/dl or 140 µmol/l)
* Asthma.
*[[Proteinuria]] (>1 g/24h)
* Eosinophilia (>10% of total white blood cells).
*[[Gastrointestinal hemorrhage]], infarction, or [[pancreatitis]]
* Neuropathy (mono- or polyneuropathy).
*Involvement of the central nervous system
* Pulmonary infiltrates.
*Cardiomyopathy
* Sinus abnormalities.
 
* Extravascular eosinophil accumulation on biopsy.
The lack of any of these factors indicates milder case, with a five-year mortality rate of 11.9%. The presence of one factor indicates severe disease, with a five-year mortality rate of 26%, and two or more indicate very severe disease: 46% five-year mortality rate.<ref>{{cite journal | vauthors = Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Casassus P | display-authors = 6 | title = Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients | journal = Medicine | volume = 75 | issue = 1 | pages = 17–28 | date = January 1996 | pmid = 8569467 | doi = 10.1097/00005792-199601000-00003 }}</ref>
* A patient is classified as having EGPA if four or more of these criteria are met.


== Treatment ==
== Treatment ==
Treatment for eosinophilic granulomatosis with polyangiitis includes glucocorticoids (such as [[prednisolone]]) and other immunosuppressive 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 lifelong.
Management of EGPA depends on the severity of organ involvement. The primary goal is to reduce inflammation and prevent long-term damage.


A [[systematic review]] conducted in 2007 indicated all patients should be treated with high-dose steroids, but in patients with a five-factor score of one or higher, cyclophosphamide pulse therapy should be commenced, with 12 pulses leading to fewer relapses than six. Remission can be maintained with a less toxic drug, such as azathioprine or [[methotrexate]].<ref>{{cite journal | vauthors = 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 | date = August 2007 | pmid = 17684188 | doi = 10.1001/jama.298.6.655 | doi-access = free }}</ref>
===First-Line Treatment===
* [[Corticosteroids]] ([[Prednisone]], [[Methylprednisolone]]):
* High-dose corticosteroids are the cornerstone of treatment, particularly during acute exacerbations.
* Gradual tapering is required to avoid flare-ups.


On December 12, 2017, the [[FDA]] approved [[mepolizumab]], the first drug therapy specifically indicated for the treatment of eosinophilic granulomatosis with polyangiitis.<ref name="FDA mepolizumab">{{cite web|title=Press Announcements - FDA approves first drug for Eosinophilic Granulomatosis with Polyangiitis, a rare disease formerly known as the Churg-Strauss Syndrome|url=https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm588594.htm|website=www.fda.gov|publisher=FDA|access-date=13 December 2017|language=en}}</ref> Patients taking mepolizumab experienced a "significant improvement" in their symptoms.<ref name="FDA mepolizumab" />
===Immunosuppressive Therapy===
* Used in cases with severe organ involvement or steroid-refractory disease.
* [[Cyclophosphamide]]: Indicated for life-threatening manifestations (cardiac, neurological, or renal involvement).
* Azathioprine or Methotrexate: Used as steroid-sparing agents for long-term maintenance.


== History ==
===Biologic Therapy===
Eosinophilic granulomatosis with polyangiitis was first described by [[Pathology|pathologists]] [[Jacob Churg]] (1910–2005) and [[Lotte Strauss]] (1913–1985) at [[Mount Sinai Hospital, New York|Mount Sinai Hospital]] in [[New York City]] in 1951, using the term "allergic granulomatosis" to describe it.<ref name="Churg">{{cite journal | vauthors = Churg J, Strauss L | title = Allergic granulomatosis, allergic angiitis, and periarteritis nodosa | journal = The American Journal of Pathology | volume = 27 | issue = 2 | pages = 277–301 | date = March–April 1951 | pmid = 14819261 | pmc = 1937314 | doi =  }}</ref><ref>{{WhoNamedIt|synd|2733}}</ref> They reported "fever...[[hypereosinophilia]], symptoms of [[heart failure|cardiac failure]], renal damage, and [[peripheral neuropathy]], resulting from vascular embarrassment in various systems of organs"{{sfn|Rich|Fleisher|Shearer|Schroeder|2012|p=700}} in a series of 13 patients with necrotizing vasculitis previously diagnosed as "[[periarteritis nodosa]]", accompanied by hypereosinophilia and severe asthma.<ref name=hellmich>{{cite journal | vauthors = Hellmich B, Ehlers S, Csernok E, Gross WL | title = Update on the pathogenesis of Churg-Strauss syndrome | journal = Clinical and Experimental Rheumatology | volume = 21 | issue = 6 Suppl 32 | pages = S69-77 | year = 2003 | pmid = 14740430 | url = http://www.clinexprheumatol.org/article.asp?a=2254 }}</ref> Drs. Churg and Strauss noted three features which distinguished their patients from other patients with periarteritis nodosa but without asthma: necrotizing vasculitis, tissue [[eosinophilia]], and extravascular [[granuloma]].<ref name=hellmich/> As a result, they proposed that these cases were evident of a different disease entity, which they referred to as "allergic granulomatosis and angiitis".<ref name=hellmich/>
* [[Mepolizumab]] (IL-5 inhibitor): Approved for EGPA, particularly for cases with eosinophilia and asthma.
* [[Rituximab]] (B-cell inhibitor): Sometimes used in ANCA-positive EGPA.


== Society and culture ==
===Supportive Treatment===
The memoir ''[[Patient (memoir)|Patient]]'', by musician [[Ben Watt]], deals with his experience with EGPA in 1992, and his recovery.<ref name=whiting>{{cite news|last=Whiting|first=Sam| name-list-format = vanc |title=Everything But the Final Song / Ben Watt lives to tell how he almost didn't|url=http://www.sfgate.com/entertainment/article/Everything-But-the-Final-Song-Ben-Watt-lives-to-2845684.php|access-date=30 June 2013|newspaper=[[San Francisco Chronicle|SFGate]]|date=10 April 1997}}</ref> Watt's case was unusual in that it mainly affected his gastrointestinal tract, leaving his lungs largely unaffected; this unusual presentation contributed to a delay in proper diagnosis. His treatment required the removal of 5&nbsp;m (15&nbsp;ft) of necrotized small intestine (about 75%), leaving him on a permanently restricted diet.<ref name=whiting/>
* Bronchodilators and inhaled steroids for asthma symptoms.
* Anticoagulation if vasculitis leads to blood clotting complications.
* Pain management for neuropathy-related symptoms.


[[Umaru Musa Yar'Adua]], the president of [[Nigeria]] from 2007 to 2010, reportedly had EGPA and died in office of complications of the disease.<ref name=sahara>{{cite news|title=WikiLeaks: Yar'Adua Died Of Lung Cancer And Churg Strauss Syndrome, US Cables Confirm|url=http://saharareporters.com/news-page/wikileaks-yar’adua-died-lung-cancer-and-churg-strauss-syndrome-us-cables-confirm|access-date=30 June 2013|newspaper=[[Sahara Reporters]]|date=2 September 2011}}</ref>
== Prognosis ==
The prognosis of EGPA varies based on disease severity and response to treatment:
* Mild cases (limited to asthma and skin involvement) often have a good prognosis with corticosteroids alone.
* Severe cases (cardiac, renal, or neurological complications) require aggressive treatment.
* Relapses are common, and lifelong monitoring is necessary.


DJ and author [[Charlie Gillett]] was diagnosed with EGPA in 2006; he died four years later.<ref name=gillett>{{cite news|title=Charlie Gillett - Obituary|url=https://www.telegraph.co.uk/news/obituaries/culture-obituaries/tv-radio-obituaries/7474947/Charlie-Gillett.html|access-date=30 June 2013|newspaper=[[The Daily Telegraph]]|date=18 Mar 2010}}</ref>
Without treatment, EGPA can lead to life-threatening complications, particularly from cardiac and pulmonary involvement. With appropriate immunosuppressive therapy, the 5-year survival rate exceeds 80%.


Japanese ski jumper [[Taku Takeuchi]], who won the bronze medal in the team competition, has the disease and competed at the Sochi Olympics less than a month after being released from hospital treatment.<ref>{{Cite web | agency = Associated Press | url=http://www.thenational.ae/sport/olympics/japans-taku-takeuchi-overcame-illness-to-win-olympic-medal-i-thought-i-might-even-die | title=Japan's Taku Takeuchi overcame illness to win Olympic medal - 'I thought I might even die' | work = The National | date = 18 February 2014 }}</ref>
== Epidemiology ==
EGPA is rare, with an estimated incidence of 1–3 cases per million per year. It primarily affects adults between ages 30 and 50, with no significant gender predominance. It is more common in individuals with long-standing asthma or allergic rhinitis.


New Zealand reporter and television presenter [[Toni Street]] was diagnosed with the condition in 2015.<ref>{{cite news|title=New Zealand responds to Toni Street's illness with love and support|url=http://www.stuff.co.nz/entertainment/tv-radio/72706867/new-zealand-responds-to-toni-streets-illness-with-love-and-support|access-date=5 October 2015|publisher=Stuff.co.nz}}</ref><ref>{{cite news|title=Toni Street reveals 'dark moments' as she battles deadly disease|url=http://www.nzherald.co.nz/entertainment/news/article.cfm?c_id=1501119&objectid=11523905|access-date=5 October 2015|publisher=NZ Herald}}</ref> Street has had health problems for several years, including removal of her gallbladder four months prior.<ref>{{cite news|title=Toni Street's mystery illness revealed|url=http://www.nzherald.co.nz/entertainment/news/article.cfm?c_id=1501119&objectid=11458564|access-date=5 October 2015|publisher=NZ Herald}}</ref>
== History ==
The condition was first described in 1951 by Jacob Churg and Lotte Strauss, who identified the combination of asthma, eosinophilia, and vasculitis. The name was changed from Churg–Strauss Syndrome to Eosinophilic Granulomatosis with Polyangiitis (EGPA) in 2012 to align with standardized nomenclature for vasculitides.


[[Willie Naulls]] died on November 22, 2018 in [[Laguna Niguel, California]], from respiratory failure due to EGPA,<ref name=goldstein>{{citation |last=Goldstein |first=Richard | name-list-format = vanc |title=Willie Naulls, Knicks All-Star and Celtics Champion, Dies at 84 | newspaper=[[The New York Times]] |date=November 25, 2018 |url=https://www.nytimes.com/2018/11/25/obituaries/willie-naulls-dies.html?action=click&module=Well&pgtype=Homepage&section=Obituaries}}</ref> which he had been battling for eight years.<ref name=bolch_11252018>{{cite news|last=Bolch|first=Ben| name-list-format = vanc |title=Former UCLA great and integration pioneer Willie Naulls dies at 84|date=November 25, 2018|newspaper=Los Angeles Times|url=https://www.latimes.com/sports/ucla/la-sp-ucla-willie-naulls-20181125-story.html|access-date=November 26, 2018}}</ref>
== Notable Cases ==
* Ben Watt, musician and author, chronicled his experience with EGPA in his memoir ''Patient''.
* Umaru Musa Yar'Adua, former President of Nigeria, reportedly had EGPA.
* Charlie Gillett, renowned DJ and music historian, passed away due to EGPA complications.
* Toni Street, New Zealand broadcaster, publicly shared her battle with EGPA.


== References ==
== See Also ==
{{Reflist}}
* [[Vasculitis]]
 
* [[Eosinophilia]]
== Further reading ==
* [[Asthma]]
{{refbegin}}
* [[Antineutrophil cytoplasmic antibody]]
* {{cite book |last=Adu |first=Dwomoa |last2=Emery |first2=Paul |last3=Madaio |first3=Michael | name-list-format = vanc |title=Rheumatology and the Kidney|year=2012|publisher=[[Oxford University Press]]|edition=2, illustrated|isbn=9780199579655|ref=harv}}
* {{cite book |last=Churg |first=Andrew |last2=Thurlbeck |first2=William | name-list-format = vanc |title=Pathology of the LungM.|year=1995|publisher=[[Thieme Medical Publishers|Thieme]]|isbn=9780865775343|edition=2, illustrated|ref=harv|url-access=registration|url=https://archive.org/details/pathologyoflung0000unse}}
* {{cite book|last=Rich|first=Robert R. |last2=Fleisher|first2=Thomas A.|last3=Shearer|first3=William T.|last4=Schroeder|first4=Harry|last5=Frew|first5=Anthony J.|last6=Weyand|first6=Cornelia M. | name-list-format = vanc |title=Clinical Immunology: Principles and Practice|year=2012|publisher=[[Elsevier|Elsevier Health Sciences]]|isbn=9780723437109|ref=harv}}
{{refend}}
 
== External links ==
{{Medical resources
| ICD10          = {{ICD10|M|30|1|m|30}}
| ICD9            = {{ICD9|446.4}}
| MedlinePlus    =
| eMedicineSubj  = med
| eMedicineTopic  = 2926
| eMedicine_mult  = {{eMedicine2|derm|78}} {{eMedicine2|neuro|501}}
| DiseasesDB      = 2685
| MeshID          = D015267
| Orphanet        = 183
}}


{{Systemic vasculitis}}
{{Systemic vasculitis}}
 
{{Autoimmune diseases}}
{{DEFAULTSORT:Eosinophilic granulomatosis with polyangiitis}}
{{stub}}
[[Category:Autoimmune diseases]]
[[Category:Autoimmune diseases]]
[[Category:Eosinophilic cutaneous conditions]]
[[Category:Eosinophilic conditions]]
[[Category:Lung disorders]]
[[Category:Lung disorders]]
[[Category:Steroid-responsive inflammatory conditions]]
[[Category:Steroid-responsive inflammatory conditions]]
[[Category:Syndromes affecting the lung]]
[[Category:Rare diseases]]
{{stub}}

Latest revision as of 18:50, 19 March 2025

Eosinophilic Granulomatosis with Polyangiitis (EGPA)
Synonyms Churg–Strauss Syndrome, Allergic Angiitis and Granulomatosis
Pronounce
Field Rheumatology, Immunology
Symptoms Fatigue, fever, weight loss, night sweats, asthma, arthralgia, myalgia, purpura, urticaria, skin nodules, cough, nasal polyps, sinusitis, neuropathy, gastrointestinal distress.
Complications Hypereosinophilia, vasculitis, cardiac disease, neuropathy, renal failure, pulmonary involvement.
Onset Gradual, typically in adulthood, often in individuals with a history of asthma or allergic conditions.
Duration Chronic with periods of remission and flare-ups.
Types Systemic necrotizing vasculitis affecting small-to-medium blood vessels.
Causes Autoimmune response; associated with antineutrophil cytoplasmic antibodies (ANCA).
Risks History of asthma, allergic rhinitis, eosinophilia, exposure to allergens or environmental triggers.
Diagnosis Blood tests for eosinophilia and ANCA, imaging studies, tissue biopsy, clinical criteria (American College of Rheumatology).
Differential diagnosis Granulomatosis with polyangiitis, hypereosinophilic syndrome, microscopic polyangiitis, sarcoidosis, asthma.
Prevention No known prevention; early treatment can help manage disease progression.
Treatment Immunosuppression with corticosteroids and immunomodulatory drugs.
Medication Corticosteroids (e.g., prednisone), mepolizumab, cyclophosphamide, azathioprine, methotrexate.
Prognosis Variable; some achieve remission, while others develop progressive complications.
Frequency Rare; estimated prevalence of 10–15 cases per million.
Deaths Can be fatal if untreated, particularly due to cardiac or pulmonary complications.


Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg–Strauss Syndrome, is a rare autoimmune disease characterized by vasculitis affecting small and medium-sized blood vessels. It primarily occurs in individuals with a history of asthma or other allergic conditions, and is distinguished by elevated levels of eosinophils in the blood and tissues, leading to widespread inflammation.

Pathophysiology[edit]

EGPA is considered an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, though not all cases are ANCA-positive. The disease progresses in three overlapping stages:

Signs and Symptoms[edit]

EGPA manifests with a wide range of symptoms, which vary depending on the stage and organ involvement. Common symptoms include:

  • Respiratory symptoms: Severe asthma, sinusitis, nasal polyps, chronic cough, lung infiltrates.
  • Skin manifestations: Purpura, urticaria, nodules, livedo reticularis, or necrotic lesions.
  • Neurological involvement: Peripheral neuropathy such as mononeuritis multiplex, characterized by muscle weakness, numbness, and pain.
  • Gastrointestinal issues: Abdominal pain, diarrhea, gastrointestinal bleeding, eosinophilic colitis.
  • Cardiovascular complications: Myocarditis, pericarditis, arrhythmias, heart failure.
  • Renal involvement: Rare but can include glomerulonephritis.
  • General symptoms: Weight loss, fever, fatigue, night sweats, joint and muscle pain.

Diagnosis[edit]

EGPA is diagnosed based on a combination of clinical symptoms, laboratory findings, and histopathological examination. Diagnostic criteria include:

  • Blood tests:
  • Elevated eosinophil count (>1,500/microliter).
  • Presence of ANCA antibodies (positive in ~40% of cases).
  • Elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR).
  • Increased IgE levels.
  • Imaging:
  • Chest X-ray or CT scan showing lung infiltrates or nodules.
  • MRI or nerve conduction studies to assess neuropathy.
  • Biopsy:
  • Tissue biopsy from affected organs (e.g., skin, lung, nerve) demonstrating vasculitis, eosinophilic infiltration, and granuloma formation.
  • American College of Rheumatology (ACR) Classification Criteria:
  • Asthma.
  • Eosinophilia (>10% of total white blood cells).
  • Neuropathy (mono- or polyneuropathy).
  • Pulmonary infiltrates.
  • Sinus abnormalities.
  • Extravascular eosinophil accumulation on biopsy.
  • A patient is classified as having EGPA if four or more of these criteria are met.

Treatment[edit]

Management of EGPA depends on the severity of organ involvement. The primary goal is to reduce inflammation and prevent long-term damage.

First-Line Treatment[edit]

Immunosuppressive Therapy[edit]

  • Used in cases with severe organ involvement or steroid-refractory disease.
  • Cyclophosphamide: Indicated for life-threatening manifestations (cardiac, neurological, or renal involvement).
  • Azathioprine or Methotrexate: Used as steroid-sparing agents for long-term maintenance.

Biologic Therapy[edit]

  • Mepolizumab (IL-5 inhibitor): Approved for EGPA, particularly for cases with eosinophilia and asthma.
  • Rituximab (B-cell inhibitor): Sometimes used in ANCA-positive EGPA.

Supportive Treatment[edit]

  • Bronchodilators and inhaled steroids for asthma symptoms.
  • Anticoagulation if vasculitis leads to blood clotting complications.
  • Pain management for neuropathy-related symptoms.

Prognosis[edit]

The prognosis of EGPA varies based on disease severity and response to treatment:

  • Mild cases (limited to asthma and skin involvement) often have a good prognosis with corticosteroids alone.
  • Severe cases (cardiac, renal, or neurological complications) require aggressive treatment.
  • Relapses are common, and lifelong monitoring is necessary.

Without treatment, EGPA can lead to life-threatening complications, particularly from cardiac and pulmonary involvement. With appropriate immunosuppressive therapy, the 5-year survival rate exceeds 80%.

Epidemiology[edit]

EGPA is rare, with an estimated incidence of 1–3 cases per million per year. It primarily affects adults between ages 30 and 50, with no significant gender predominance. It is more common in individuals with long-standing asthma or allergic rhinitis.

History[edit]

The condition was first described in 1951 by Jacob Churg and Lotte Strauss, who identified the combination of asthma, eosinophilia, and vasculitis. The name was changed from Churg–Strauss Syndrome to Eosinophilic Granulomatosis with Polyangiitis (EGPA) in 2012 to align with standardized nomenclature for vasculitides.

Notable Cases[edit]

  • Ben Watt, musician and author, chronicled his experience with EGPA in his memoir Patient.
  • Umaru Musa Yar'Adua, former President of Nigeria, reportedly had EGPA.
  • Charlie Gillett, renowned DJ and music historian, passed away due to EGPA complications.
  • Toni Street, New Zealand broadcaster, publicly shared her battle with EGPA.

See Also[edit]


Systemic vasculitis
Large vessel
Medium vessel
Small vessel
Other


‹ The template below (Hypersensitivity and autoimmune diseases) is being considered for merging with Allergic conditions. See templates for discussion to help reach a consensus. ›
Hypersensitivity and autoimmune diseases (279.5–6)
Type I/allergy/atopy
(IgE)
Type II/ADCC
Type III
(Immune complex)
Type IV/cell-mediated
(T cells)
Unknown/
multiple


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