Churg-Strauss Syndrome Clinical Presentation

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

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
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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 (See the image below.)The skin rashes of Churg-Strauss syndrome. The bioThe 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%])
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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 cholangitis[16] ; 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]

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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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