Churg-Strauss Syndrome Follow-up

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

Further Inpatient Care

  • Churg-Strauss syndrome (CSS) cases must be followed up very closely at a rheumatology clinic or another specialty clinic. Patients with this syndrome usually need long-term immunosuppressive medications.
  • The patient's clinical status, ESR, and eosinophilia should be monitored. The ANCA level does not correlate well with disease activity.[32]
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Inpatient & Outpatient Medications

  • Inpatient medications include the following:
    • Prednisone: Start at 0.5-1 mg/kg/d PO.
    • Methylprednisolone is administered in intravenous form at higher doses in the presence of major organ involvement, including cardiac, pulmonary (hemorrhage), renal, or neuropathy.
    • Cyclophosphamide is administered in patients with severe or life-threatening complications.[33, 34] The single intravenous dose is 500 mg/m2 of body-surface area. The dose should be reduced in patients with renal failure.
    • Other medications include azathioprine, mycophenolate mofetil, and intravenous immune globulin administered in similar fashion as it is used in microscopic polyangiitis or Wegener granulomatosis.[35]
    • Plasmapheresis is probably not helpful.[36]
    • Interferon alpha might be helpful in treatment.[37, 38]
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Complications

  • Complications of vasculitis depend on the specific organ system involvement.
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Prognosis

  • Overall, without treatment, the 5-year survival rate in Churg-Strauss syndrome is about 25%.
  • With treatment, the 1-year survival rate is 90% and the 5-year survival rate is 62%.
  • Causes of death associated with Churg-Strauss syndrome include the following:
    • Cardiac failure, myocardial infarction, or both (most common cause)
    • Renal failure
    • Cerebral hemorrhage
    • Gastrointestinal bleeding
    • Status asthmaticus
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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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