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Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Clinical Presentation

  • Author: Spencer T Lowe, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Jul 05, 2016
 

History

Churg-Strauss syndrome (CSS) has three phases, as follows:

  1. Allergic rhinitis and asthma
  2. Eosinophilic infiltrative disease (eg, eosinophilic pneumonia or gastroenteritis)
  3. 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 symptoms and signs of the disease were reported by Guillevin et al in their case series[2] :

  • 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. [19] )
  • 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 granulomatosis with polyangiitis [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 bulla, 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, or colitis; these include 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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Causes

Causes of Churg-Strauss syndrome are unknown.[8] 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[20] ; 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.[21]

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Physical Examination

Except for fever, 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.[2] In addition to asthma and eosinophilia, a dermato-pulmonary-renal syndrome is the feature of this disease. Mononeuritis multiplex is common.

Skin involvement (60%) may include the following:

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

Upper respiratory involvement may include the following:

  • Allergic rhinitis
  • Paranasal sinusitis
  • Nasal polyposis

Lower respiratory system physical findings are related to the following:

  • Asthma (ie, wheeze), expiratory rhonchi
  • Pneumonitis
  • Hemoptysis secondary to pulmonary alveolar hemorrhage (alveolar capillaritis)

Cardiovascular findings may include the following:

  • Myocarditis and signs related to heart failure
  • Myocardial infarction secondary to coronary vasculitis

Renal findings may include the following:

  • Hypertension
  • Signs of uremia and advanced renal failure

Gastrointestinal findings may include the following:

  • GI bleeding
  • Bowel ischemia and perforation
  • Gastroenteritis
  • Appendicitis
  • Pancreatitis

Nervous system findings may include the following:

  • Peripheral neuropathy (includes mononeuritis multiplex [77%])
  • Central nervous system (includes stroke [5%])

In a study of 15 ANCA-positive and 35 ANCA-negative patients with Churg-Strauss syndrome, ANCA-positive patients had a significantly higher incidence of renal involvement, skin involvement, and peripheral neuropathy in the form of mononeuritis multiplex at the time of diagnosis. Over the entire follow-up period, ANCA-negative patients had significantly more frequent cardiac manifestations. ANCA-positive patients required significantly higher steroid doses to maintain remission.[22]

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Contributor Information and Disclosures
Author

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, International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher L Tracy, MD Associate Program Director, Rheumatology Fellowship Program, Walter Reed National Military Medical Center

Christopher L Tracy, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Genentech; Pfizer; Questcor.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; 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, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

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

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous coauthor, Sandra L Sessoms, MD, to the development and writing of this article.

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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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