Churg–Strauss syndrome

Churg-Strauss syndrome
Classification and external resources
Micrograph showing an eosinophilic vasculitis consistent with Churg-Strauss syndrome. H&E stain.
ICD-10	M30.1
ICD-9	446.4
DiseasesDB	2685
eMedicine	med/2926 derm/78 neuro/501
MeSH	D015267

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.

Disease stages

This disease has three distinct stages.

• 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%).^[4]

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.^[5]

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."^[6] Researchers have searched for links between drugs such as montelukast (Singulair)^[7] 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] A case study in 2006 was inconclusive but suggested the need for further research.^[9]

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.^[10]

The DJ and author Charlie Gillett was diagnosed with Churg-Strauss in 2006; he died four years later.

References

1. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0. 
2. ^ synd/2733 at Who Named It?
3. ^ Churg J, Strauss L (1951). "Allergic granulomatosis, allergic angiitis, and periarteritis nodosa". Am. J. Pathol. 27 (2): 277–301. PMC 1937314. PMID 14819261. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1937314. 
4. ^ 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. 
5. ^ 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. 
6. ^ Lasse G. Gøransson; Roald Omdal (2000). "A severe systemic inflammatory reaction following therapy with montelukast (Singulair)". Nephrol Dial Transplant (Oxford Journals): pp. 1054–1055. http://ndt.oxfordjournals.org/cgi/content/full/15/7/1054. Retrieved 2009-10-06. 
7. ^ Businesswire (Oct 22, 2009). "Merck Announces Third-Quarter 2009 Financial Results". Reuters. http://www.reuters.com/article/pressRelease/idUS125664+22-Oct-2009+BW20091022. Retrieved 2009-11-05. "Continued Growth of Key Products, JANUVIA, JANUMET, ISENTRESS and SINGULAIR, Drives Strong Results -- Merck Updates Full-Year 2009" 
8. ^ 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. http://chestjournal.chestpubs.org/content/117/3/708.full. Retrieved 8 September 2009. 
9. ^ Füsun Topçu; Levent Akyıldız; Tekin Yıldız; Kenan øltumur; Faruk Oktay; Nihal Kılınç (2007 B(1)). "Montelukast and Churg-Strauss Syndrome". Turkish Respiratory Journal (Lung Pathology): pp. 27–29. http://docs.google.com/gview?a=v&q=cache:i5Mj2ZF0W58J:www.turkishrespiratoryjournal.com/pdf/pdf_TRJ_488.pdf+(%22Singulair%22+OR+%22montelukast%22)+Churg-Strauss&hl=en&gl=us&sig=AFQjCNE3_EOsKBGdtHrtfgxMb3faffyHpw. Retrieved 2009-10-06. 
10. ^ Saharareporters (May 5, 2010). "Umaru Yar’adua Dies; Jonathan To Be Sworn In". http://www.saharareporters.com/news/5996-yaradua-is-dead.html. Retrieved May 5, 2010. 

External links

• Churg-Strauss syndrome - MedLink Neurology Clinical Summary
• 13631492 at GPnotebook