eMedicine Specialties > Rheumatology > Vasculitis

Churg-Strauss Syndrome

Author: Mehran Farid-Moayer, MD, Adjunct Clinical Faculty, Department of Psychiatry, Sleep Disorders Clinic, Stanford Medical Center
Coauthor(s): Spencer T Lowe, MD, Rheumatologist, Private Practice, Peninsula Medical Group, Burlingame, CA
Contributor Information and Disclosures

Updated: Jul 31, 2009

Introduction

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

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

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.

Clinical

History

Churg-Strauss syndrome (CSS) has 3 phases— allergic rhinitis and asthma; eosinophilic infiltrative disease, such as eosinophilic pneumonia or gastroenteritis; and systemic medium- and small-vessel vasculitis with granulomatous inflammation. The vasculitic phase usually develops within 3 years of the onset of asthma, although it may be delayed for several decades. The most prominent symptoms and signs are those related to pulmonary, cardiac, dermatologic, renal, and peripheral nerve involvement. Mononeuritis multiplex is a major clinical finding.

The following list includes the symptoms and signs of the disease as reported by Guillevin et al (1999) in their case series:1

  • Constitutional symptoms - Malaise, fatigue, flulike symptoms, weight loss (70%), fever (57%), myalgias (52%)
  • Asthma symptoms (Asthma is a central feature of Churg-Strauss syndrome, occurring in 97% of patients. Asthma may precede vasculitis by up to 10 years or, less frequently, may coincide with the appearance of vasculitis. Asthma symptoms are usually persistent; therefore, patients are usually treated with steroids. This, in turn, might mask other features of the syndrome.)
  • Paranasal sinusitis (61%) - Usually responds to oral steroids
  • Allergic rhinitis (This is a common symptom. Additionally, recurrent sinusitis and polyposis are seen. But, unlike in Wegener granulomatosis, necrotizing lesions of the upper airway are unusual.)
  • Pulmonary symptoms (37%), including cough and hemoptysis
  • Arthralgias (40%)
  • Skin manifestations (49%)
  • Purpura
    • Skin nodules
    • Urticarial rash
    • Necrotic bullae
    • Digital ischemia
  • Cardiac manifestations - Symptoms related to heart failure, myocarditis, pericarditis, constrictive pericarditis, and myocardial infarction
  • Gastrointestinal symptoms (31%) - Symptoms related to GI vasculitis, eosinophilic gastritis, colitis (This includes abdominal pain [59%], diarrhea [33%], and GI bleeding [18%].)
  • Peripheral neuropathy - Mononeuritis multiplex (most frequent form, occurring in as many as 77% of patients)
  • Less frequent symptoms - Symptoms related to stroke, ophthalmologic involvement, and other rare symptoms

Physical

The physical findings in Churg-Strauss syndrome are specific to organ-system involvement. Pulmonary involvement is the most prominent. In fact, a pneumonitis plus eosinophilia warrants consideration of this syndrome and a search for evidence of systemic vasculitis elsewhere.1 In addition to asthma and eosinophilia, a dermato-pulmonary-renal syndrome is the feature of this disease. Mononeuritis multiplex is common.

  • Fever
  • Skin involvement (60%)
    • Leukocytoclastic angiitis with palpable purpura
    • Livedo reticularis, skin necrosis and gangrene, digital ischemia, urticaria, and subcutaneous nodules

      The skin rashes of Churg-Strauss syndrome. The bi...

      The skin rashes of Churg-Strauss syndrome. The biopsy of this rash showed eosinophilic leukocytoclastic angiitis with poorly formed granulomas.

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      The skin rashes of Churg-Strauss syndrome. The bi...

      The skin rashes of Churg-Strauss syndrome. The biopsy of this rash showed eosinophilic leukocytoclastic angiitis with poorly formed granulomas.

  • Upper respiratory involvement
    • Allergic rhinitis
    • Paranasal sinusitis
    • Nasal polyposis
  • Lower respiratory system physical findings related to the following:
    • Asthma (ie, wheeze), expiratory rhonchi
    • Pneumonitis
    • Hemoptysis secondary to pulmonary alveolar hemorrhage (alveolar capillaritis)
  • Cardiovascular system
    • Myocarditis and signs related to heart failure
    • Myocardial infarction secondary to coronary vasculitis
  • Renal system
    • Hypertension
    • Signs of uremia and advanced renal failure
  • Gastrointestinal system
    • GI bleeding
    • Bowel ischemia and perforation
    • Gastroenteritis
    • Appendicitis
    • Pancreatitis
  • Nervous system
    • Peripheral neuropathy (includes mononeuritis multiplex [77%])
    • Central nervous system (includes stroke [5%])

Causes

Causes of Churg-Strauss syndrome are unknown.7 Churg-Strauss syndrome is possibly an allergic or autoimmune reaction to an environmental agent or drug.

Several case reports have described drug-induced forms of Churg-Strauss syndrome. Mesalazine-induced Churg-Strauss syndrome has been reported in a patient with Crohn disease and sclerosing cholangitis16 ; 4 publications have addressed the association between propylthiouracil, methimazole, and vasculitides, including Churg-Strauss syndrome. One report is available on the association of freebase cocaine and Churg-Strauss syndrome.17

More on Churg-Strauss Syndrome

Overview: Churg-Strauss Syndrome
Differential Diagnoses & Workup: Churg-Strauss Syndrome
Treatment & Medication: Churg-Strauss Syndrome
Follow-up: Churg-Strauss Syndrome
Multimedia: Churg-Strauss Syndrome
References
Further Reading
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References

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Keywords

Churg-Strauss syndrome, CSS, allergic granulomatosis angiitis, allergic granulomatous angiitis, granulomatous small-vessel vasculitis, syndrome of small- to medium-sized arteries and veins, antibodies to neutrophil cytoplasmic antigens, ANCA, asthma, wheezing, expiratory rhonchi, eosinophilia, paranasal sinusitis, pulmonary infiltrates, vasculitis with extravascular eosinophils, mononeuritis multiplex or polyneuropathy, Churg-Strauss–like syndrome, CSS-like syndrome, allergic rhinitis, eosinophilic pneumonia, gastroenteritis

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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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, 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 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; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, 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: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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