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Eosinophilic granulomatosis with polyangiitis

eosinophilic granulomatosis with polyangiitis tacrolimus, eosinophilic granulomatosis with polyangiitis clarithromycin
Eosinophilic granulomatosis with polyangiitis EGPA; also known as Churg-Strauss syndrome CSS or allergic granulomatosis23 is an extremely rare autoimmune condition that causes inflammation of small and medium-sized blood vessels vasculitis in persons with a history of airway allergic hypersensitivity atopy

It usually manifests in three stages The early 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 lungs and the digestive tract The third stage consists of vasculitis, which can eventually lead to cell death and can be life-threatening

This condition is now called "eosinophilic granulomatosis with polyangiitis" to remove all eponyms from the vasculitides To facilitate the transition, it was referred to as "eosinophilic granulomatosis with polyangiitis Churg-Strauss" for a period of time starting in 20124 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 it2 It is a type of systemic necrotizing vasculitis

Effective treatment of EGPA requires suppression of the immune system with medication This is typically glucocorticoids, followed by other agents such as cyclophosphamide or azathioprine

Contents

  • 1 Signs and symptoms
    • 11 Allergic stage
    • 12 Eosinophilic stage
    • 13 Vasculitic stage
  • 2 Diagnosis
  • 3 Risk stratification
  • 4 Treatment
  • 5 Historical background
  • 6 Famous cases
  • 7 References
  • 8 Further reading
  • 9 External links

Signs and symptomsedit

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;5 whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, eg with skin lesions and nasal polyps6 EGPA is consequently considered a highly variable condition in terms of its presentation and its course56

Allergic stageedit

The prodromal stage is characterized by allergy Almost all patients experience asthma and/or allergic rhinitis,7 with more than 90% having a history of asthma that is either a new development, or the worsening of pre-existing asthma,8 which may require systemic corticosteroid treatment5 On average, asthma develops from three to nine years before the other signs and symptoms5

The allergic rhinitis may produce symptoms such as rhinorrhea and nasal obstruction, and the formation of nasal polyps that require surgical removal, often more than once7 Sinusitis may also be present7

Eosinophilic stageedit

The second stage is characterized by an abnormally high level of eosinophils a type of white blood cell in the blood and tissues5 The symptoms of hypereosinophilia depend on which part of the body is affected, but most often it affects the lungs and digestive tract5 The signs and symptoms of hypereosinophilia may include weight loss, night sweats, asthma, cough, abdominal pain, and gastrointestinal bleeding5 Fever and malaise are often present9

The eosinophilic stage can last months or years, and its symptoms can disappear, only to return later5 Patients may experience the third stage simultaneously5

Vasculitic stageedit

The third and final stage, and hallmark of EGPA, is inflammation of the blood vessels, and the consequent reduction of blood flow to various organs and tissues5 Local and systemic symptoms become more widespread and are compounded by new symptoms from the vasculitis9

Severe complications may arise Blood clots may develop within the damaged arteries in severe cases, particularly in arteries of the abdominal region, which is followed by infarction and cell death, or slow atrophy9 Many patients experience severe abdominal complaints; these are most often due to peritonitis and/or ulcerations and perforations of the gastrointestinal tract, but occasionally due to acalculous cholecystitis or granulomatous appendicitis9

The most serious complication of the vasculitic stage is heart disease, which is the cause of nearly one-half of all deaths in patients with EGPA9 Among heart disease-related deaths, the most usual cause is inflammation of the heart muscle caused by the high level of eosinophils, although some are deaths due to inflammation of the arteries that supply blood to the heart or pericardial tamponade9 Kidney complications have been reported as being less common10

Diagnosisedit

Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue, and antineutrophil cytoplasmic antibodies ANCA against neutrophil granulocytes The American College of Rheumatology 1990 criteria for diagnosis of Churg–Strauss syndrome lists these criteria:

  • Asthma
  • Eosinophils greater than 10% of a differential white blood cell count
  • Presence of mononeuropathy or polyneuropathy
  • Unfixed pulmonary infiltrates
  • Presence of paranasal sinus abnormalities
  • Histological evidence of extravascular eosinophils

For classification purposes, a patient shall be said to have Churg–Strauss syndrome CSS 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 997%11

Risk stratificationedit

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:

  • Reduced renal function creatinine >158 mg/dl or 140 µmol/l
  • Proteinuria >1 g/24h
  • Gastrointestinal hemorrhage, infarction, or pancreatitis
  • Involvement of the central nervous system
  • Cardiomyopathy

The lack of any of these factors indicates milder case, with a five-year mortality rate of 119% 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 rate12

Treatmentedit

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

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 methotrexate13

Historical backgroundedit

Eosinophilic granulomatosis with polyangiitis was first described by pathologists Jacob Churg 1910–2005 and Lotte Strauss 1913–1985 at Mount Sinai Hospital in New York City in 1951, using the term "allergic granulomatosis" to describe it214 They reported "feverhypereosinophilia, symptoms of cardiac failure, renal damage, and peripheral neuropathy, resulting from vascular embarrassment in various systems of organs"15 in a series of 13 patients with necrotizing vasculitis previously diagnosed as "periarteritis nodosa", accompanied by hypereosinophilia and severe asthma16 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 granuloma16 As a result, they proposed that these cases were evident of a different disease entity, which they referred to as "allergic granulomatosis and angiitis"16

Famous casesedit

The memoir Patient, by musician Ben Watt, deals with his experience with Churg–Strauss syndrome in 1992, and his recovery17 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 m 15 ft of necrotized small intestine about 75%, leaving him on a permanently restricted diet17

Umaru Musa Yar'Adua, the president of Nigeria from 2007–2010, reportedly suffered from EGPA and died in office of complications of the disease18

DJ and author Charlie Gillett was diagnosed with EGPA in 2006; he died four years later19

Japanese ski jumper Taku Takeuchi, who won the bronze medal in the team competition, suffers from the disease and competed at the Sochi Olympics less than a month after being released from hospital treatment20

New Zealand reporter and television presenter Toni Street was diagnosed with the condition in 20152122 Street has suffered with health problems for several years, including removal of her gallbladder four months prior23

Referencesedit

  1. ^ Arthritis Rheum 1990:33:1094
  2. ^ a b c Jacob Churg; Lotte Strauss March–April 1951 "Allergic granulomatosis, allergic angiitis, and periarteritis nodosa" The American Journal of Pathology 27 2: 277–301 PMC 1937314  PMID 14819261 
  3. ^ Adu, Emery & Madaio 2012, p 125
  4. ^ Montesi, Sydney B; Nance, John W; Harris, R Scott; Mark, Eugene J 2016-06-09 "Case 17-2016 — A 60-Year-Old Woman with Increasing Dyspnea" New England Journal of Medicine 374 23: 2269–2279 ISSN 0028-4793 PMID 27276565 doi:101056/NEJMcpc1516452 
  5. ^ a b c d e f g h i j "Churg-Strauss syndrome - Symptoms" Mayo Clinic Retrieved 30 June 2013 
  6. ^ a b Della Rossa A, Baldini C, Tavoni A, et al November 2002 "Churg-Strauss syndrome: clinical and serological features of 19 patients from a single Italian centre" Rheumatology Oxford 41 11: 1286–94 PMID 12422002 doi:101093/rheumatology/41111286 
  7. ^ a b c Churg & Thurlbeck 1995, p 425
  8. ^ Rich, Robert R; Fleisher, Thomas A; Shearer, William T; Schroeder, Harry; Frew, Anthony J; Weyand, Cornelia M 2012 Clinical Immunology: Principles and Practice Elsevier Health Sciences p 701 ISBN 9780723437109 
  9. ^ a b c d e f Churg & Thurlbeck 1995, p 426
  10. ^ Rich et al 2012, p 701
  11. ^ Masi AT, Hunder GG, Lie JT, et al August 1990 "The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome allergic granulomatosis and angiitis" Arthritis & Rheumatism 33 8: 1094–100 PMID 2202307 doi:101002/art1780330806 
  12. ^ Guillevin L, Lhote F, Gayraud M, et al 1996 "Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome A prospective study in 342 patients" Medicine Baltimore 75 1: 17–28 PMID 8569467 doi:101097/00005792-199601000-00003 
  13. ^ Bosch X, Guilabert A, Espinosa G, Mirapeix E 2007 "Treatment of antineutrophil cytoplasmic antibody-associated vasculitis: a systematic review" JAMA 298 6: 655–69 PMID 17684188 doi:101001/jama2986655 
  14. ^ synd/2733 at Who Named It
  15. ^ Rich et al 2012, p 700
  16. ^ a b c Hellmich B, Ehlers S, Csernok E, Gross WL 2003 "Update on the pathogenesis of Churg-Strauss syndrome" Clinical and Experimental Rheumatology 21 6 Suppl 32: S69–77 PMID 14740430 
  17. ^ a b Whiting, Sam 10 April 1997 "Everything But the Final Song / Ben Watt lives to tell how he almost didn't" SFGate Retrieved 30 June 2013 
  18. ^ "WikiLeaks: Yar’Adua Died Of Lung Cancer And Churg Strauss Syndrome, US Cables Confirm" Sahara Reporters 2 September 2011 Retrieved 30 June 2013 
  19. ^ "Charlie Gillett - Obituary" The Daily Telegraph 18 Mar 2010 Retrieved 30 June 2013 
  20. ^ http://wwwthenationalae/sport/olympics/japans-taku-takeuchi-overcame-illness-to-win-olympic-medal-i-thought-i-might-even-die
  21. ^ "New Zealand responds to Toni Street's illness with love and support" Stuffconz Retrieved 5 October 2015 
  22. ^ "Toni Street reveals 'dark moments' as she battles deadly disease" NZ Herald Retrieved 5 October 2015 
  23. ^ "Toni Street's mystery illness revealed" NZ Herald Retrieved 5 October 2015 

Further readingedit

  • Adu, Dwomoa; Emery, Paul; Madaio, Michael 2012 Rheumatology and the Kidney 2, illustrated ed Oxford University Press ISBN 9780199579655 
  • Churg, Andrew; Thurlbeck, William M 1995 Pathology of the Lung 2, illustrated ed Thieme ISBN 9780865775343 
  • Rich, Robert R; Fleisher, Thomas A; Shearer, William T; Schroeder, Harry; Frew, Anthony J; Weyand, Cornelia M 2012 Clinical Immunology: Principles and Practice Elsevier Health Sciences ISBN 9780723437109 

External linksedit

  • Churg-Strauss syndrome – MedLink Neurology Clinical Summary

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