Churg-Strauss Syndrome 

  • Author: Mehran Farid-Moayer, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Apr 13, 2011
 

Background

Churg-Strauss syndrome (CSS), or allergic granulomatous angiitis, is a rare syndrome that affects small- to medium-sized arteries and veins. Wegener granulomatosis, Churg-Strauss syndrome, and the microscopic form of periarteritis (ie, microscopic polyangiitis) are three closely related vasculitic syndromes that affect medium- and small-sized vessels and are associated with antibodies to neutrophil cytoplasmic antigens (ANCAs).[1, 2, 3, 4]

In 1951, Churg and Strauss first described the syndrome in 13 patients who had asthma, eosinophilia, granulomatous inflammation, necrotizing systemic vasculitis, and necrotizing glomerulonephritis.[2]

The American College of Rheumatology (ACR) has proposed 6 criteria for the diagnosis of Churg-Strauss syndrome.[5] The presence of 4 or more criteria yields a sensitivity of 85% and a specificity of 99.7%. These criteria include (1) asthma (wheezing, expiratory rhonchi), (2) eosinophilia of more than 10% in peripheral blood, (3) paranasal sinusitis, (4) pulmonary infiltrates (may be transient), (5) histological proof of vasculitis with extravascular eosinophils, and (6) mononeuritis multiplex or polyneuropathy.

The 1994 Chapel Hill consensus conference on the classification of vasculitides did not modify the ACR criteria.[6]

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Pathophysiology

Churg-Strauss syndrome is a granulomatous small-vessel vasculitis. The cause of this allergic angiitis and granulomatosis is unknown.[7] No data have been reported regarding the role of immune complexes or cell-mediated mechanisms in this disease, although autoimmunity is evident with the presence of hypergammaglobulinemia, increased levels of immunoglobulin E (IgE), rheumatoid factor, and ANCA.

A Churg-Strauss syndrome–like syndrome develops as a rare complication in people with asthma who are steroid-dependent and who are treated with leukotriene receptor antagonists (eg, montelukast, zafirlukast) upon reduction in their oral steroid dose.[8] The Churg-Strauss syndrome–like complication is reported in people whose withdrawal of oral steroids is also facilitated by inhaled steroids. This complication is probably related to steroid withdrawal, which unmasks underlying Churg-Strauss syndrome,[9, 10, 11] rather than to the drugs themselves. However, in rare cases, this syndrome has developed when a leukotriene receptor antagonist has been substituted for inhaled steroids without a history of oral steroid withdrawal.

HLA-DRB4 positivity may be a genetic risk factor for the development of Churg-Strauss syndrome and may increase the likelihood of vasculitic manifestations of the disease.[12]

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Epidemiology

Frequency

United States

The incidence of Churg-Strauss syndrome in the United States is 1-3 cases per 100,000 adults per year.[13]

International

The international incidence of Churg-Strauss syndrome is approximately 2.5 cases per 100,000 adults per year.

Mortality/Morbidity

  • The principal causes of morbidity and mortality in Churg-Strauss syndrome are myocarditis and myocardial infarction secondary to coronary arteritis.[14]
  • With treatment, the 1-year survival rate is 90% and the 5-year survival rate is 62%.[1, 15]

Sex

Churg-Strauss syndrome is slightly more common in males than in females.

Age

The age at onset varies from 15-70 years, with a mean age of approximately 38 years. Churg-Strauss syndrome in pediatric patients is well described, but mostly as case reports. The mean age at diagnosis is around 50 years.

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Contributor Information and Disclosures
Author

Mehran Farid-Moayer, MD  Adjunct Clinical Faculty, Department of Psychiatry, Sleep Disorders Clinic, Stanford Medical Center

Mehran Farid-Moayer, MD is a member of the following medical societies: American Academy of Sleep Medicine, American Medical Association, and American Thoracic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Spencer T Lowe, MD  Rheumatologist, Private Practice, Peninsula Medical Group, Burlingame, CA

Spencer T Lowe, MD is a member of the following medical societies: American College of Rheumatology, California Medical Association, and International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Lawrence H Brent, MD  Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching; Takeda Honoraria Speaking and teaching; UCB Speaking and teaching; Omnicare Consulting fee Consulting; Centocor Consulting fee Consulting

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

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Transient pulmonary infiltrates in a patient with Churg-Strauss syndrome (CSS).
Eosinophilic granuloma in a patient with Churg-Strauss syndrome (CSS).
The skin rashes of Churg-Strauss syndrome. The biopsy of this rash showed eosinophilic leukocytoclastic angiitis with poorly formed granulomas.
 
 
 
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