Eosinophilic granulomatosis with polyangiitis
| Eosinophilic granulomatosis with polyangiitis | |
|---|---|
| Specialty | Immunology, rheumatology  |
Churg–Strauss syndrome (also known as "Allergic granulomatosis"[1]) is a medium and small vessel 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-inheritable and non-transmissible. Churg–Strauss syndrome was once considered a type of polyarteritis nodosa due to their similar morphologies.
The syndrome was first described by Drs. Jacob Churg and Lotte Strauss at Mount Sinai Hospital in New York City in 1951.[2][3]
Diagnosis
Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue and anti-neutrophil cytoplasmic antibodies (ANCA) against neutrophil granulocytes. Differentiation from Wegener's granulomatosis is not difficult. Wegener's is closely associated with c-ANCA, while Churg-Strauss can be associated with elevations of p-ANCA.
The American College of Rheumatology 1990 criteria for diagnosis of Churg-Strauss Syndrome lists the following criteria: Asthma, Blood Eosinophilia >10%, Presence of mononeuropathy or polyneuropathy, non fixed pulmonary infiltrates, presence of paranasal sinus abnormality and histological evidence of extravascular eosinophils. For classification purposes, a patient shall be said to have Churg-Strauss syndrome (CSS) if at least 4 of these 6 criteria are positive. The presence of any 4 or more of the 6 criteria yields a sensitivity of 85% and a specificity of 99.7%.[4]
Disease stages
This disease has three distinct stages.[citation needed]
- The first stage often involves the sinuses and the onset of allergies not previously had or the worsening of pre-existing allergies.
- The second stage involves the onset of acute asthma. Normally, the person would not have had asthma previously.
- The third and final stage involves the various organ systems. 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 can affect the heart, lungs, kidneys, liver and digestive system.
People can live for many years in the first two stages before progressing to stage three.
Risk stratification
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%).[5]
Treatment
Treatment for Churg–Strauss syndrome 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 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 fewer relapses than 6. Remission can be maintained with a less toxic drug, such as azathioprine or methotrexate.[6]
Montelukast
A case study in 2000 noticed a "marked and severe inflammatory reaction characterized by eosinophilia and glomerulonephritis" and wondered whether this "might have been triggered by the leukotriene receptor antagonist, montelukast."[7] Researchers have searched for links between leukotriene antagonists and Churg–Strauss syndrome; in another study in 2000, researchers did not find a cause-and-effect relationship and wrote: "The occurrence of Churg-Strauss syndrome in asthmatic patients receiving leukotriene modifiers appears to be related to unmasking of an underlying vasculitic syndrome that is initially clinically recognized as moderate to severe asthma and treated with corticosteroids. Montelukast does not appear to directly cause the syndrome in these patients."[8]
Famous patients
The memoir Patient, by the musician Ben Watt, deals with Watt's mid-1990s experience with Churg–Strauss syndrome, and his recovery. 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 large sections of necrotized intestine, leaving Watt on a permanently restricted diet.
Umaru Musa Yar'Adua, the president of Nigeria from 2007–2010, reportedly suffered from Churg–Strauss syndrome and died in office of complications of the disease.[9]
The DJ and author Charlie Gillett was diagnosed with Churg-Strauss in 2006; he died four years later.
References
- ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
{{cite book}}: CS1 maint: multiple names: authors list (link) - ^ synd/2733 at Who Named It?
- ^ Churg J, Strauss L (1951). "Allergic granulomatosis, allergic angiitis, and periarteritis nodosa". Am. J. Pathol. 27 (2): 277–301. PMC 1937314. PMID 14819261.
- ^ Masi AT, Hunder GG, Lie JT; et al. (1990). "The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis)". Arthritis Rheum. 33 (8): 1094–100. PMID 2202307.
{{cite journal}}: Explicit use of et al. in:|author=(help); Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ 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. doi:10.1097/00005792-199601000-00003. PMID 8569467.
{{cite journal}}: Explicit use of et al. in:|author=(help)CS1 maint: multiple names: authors list (link) - ^ Bosch X, Guilabert A, Espinosa G, Mirapeix E (2007). "Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review". JAMA. 298 (6): 655–69. doi:10.1001/jama.298.6.655. PMID 17684188.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ Lasse G. Gøransson; Roald Omdal (2000). "A severe systemic inflammatory reaction following therapy with montelukast (Singulair)". Nephrol Dial Transplant. Oxford Journals. pp. 1054–1055. Retrieved 2009-10-06.
- ^ Michael E. Wechsler MD; David Finn MD; Dineli Gunawardena MD; Robert Westlake MD; Alan Barker MD FCCP; Sai P. Haranath MD; Romain A. Pauwels MD Johan Kips MD Jeffrey M. Drazen MD (March 2000 vol. 117 no. 3). "Churg-Strauss Syndrome in Patients Receiving Montelukast as Treatment for Asthma". American College of Chest Physicians. pp. 708–713. doi:10.1378/chest.117.3.708. Retrieved 8 September 2009.
{{cite news}}: Check date values in:|date=(help) - ^ Saharareporters (May 5, 2010). "Umaru Yar'adua Dies; Jonathan To Be Sworn In". Retrieved May 5, 2010.
External links
- Churg-Strauss syndrome - MedLink Neurology Clinical Summary
- . GPnotebook https://www.gpnotebook.co.uk/simplepage.cfm?ID=13631492.
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