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Churg-Strauss Disease Follow-up

  • Author: Robert Stanley Rust, Jr, MD, MA; Chief Editor: Amy Kao, MD  more...
 
Updated: Oct 13, 2014
 

Further Outpatient Care

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  • Administration of antacids or histamine-blocking agents to reduce the risk for gastrointestinal hemorrhage should be continued for as long as patients are administered oral corticosteroids.
  • Long-term corticosteroid administration entails risk for electrolyte disturbances, infections, and fractures because of diminished bone mass. These risks must be reassessed continually during the course of therapy.
  • Alternative steroid-sparing anti-inflammatory therapies, selected from among the options noted in the Medication section, should be considered in patients requiring long-term corticosteroid therapy.
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Further Inpatient Care

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  • Patients hospitalized for treatment of Churg-Strauss disease should be assessed, as should any hospitalized patient, for the risk of deep vein thrombosis, pulmonary embolus and, if cardiac dysfunction has been noted, cardiogenic embolus.
  • Treatment with steroids entails risks for gastrointestinal hemorrhage, electrolyte disturbance, and infection, which must be considered. When steroids are administered, antacids or appropriate histamine-blocking agents should be administered to reduce the risk for gastrointestinal hemorrhage.
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Deterrence/Prevention

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  • A number of authorities believe that the administration of cysteinyl leukotriene-receptor antagonists for the treatment of asthma may provoke development of Churg-Strauss disease in some individuals. The use of inhaled steroids, rather than cysteinyl leukotriene-receptor antagonists, during the taper phase of steroid treatment of an acute exacerbation of asthma may be a valuable alternative for avoiding this potentially provocative circumstance.
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Complications

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  • Particularly characteristic complications of the second phase of Churg-Strauss disease are chronic eosinophilic pneumonia and eosinophilic gastroenteritis.
  • Abdominal pain, diarrhea, gastrointestinal bleeding, and bowel perforation are important complications.
  • Raynaud phenomenon, arthralgias, or joint effusions are occasional complications.
  • Treatment-related complications include those associated with immunosuppression (eg, risk for infection) and other effects of anti-inflammatory medications such as Cushing ulcer with or without gastrointestinal perforation.
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Prognosis

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  • Once an appropriate therapeutic intervention is undertaken, a good response usually is achieved within 4 weeks. Thereafter, the disease can usually be well controlled with low maintenance steroid doses.
  • Most individuals who showed favorable response to corticosteroid treatment of Churg-Strauss associated neuropathy do not manifest disease relapse within the ensuing 8 months.
  • Individuals with neuropathy associated with Churg-Strauss disease who show poor response to initial corticosteroid treatment may experience improvement once cyclophosphamide is administered.
  • With modern therapy, the outlook for Churg-Strauss disease appears to be much better than in early reports. More than 90% of patients achieve remission after initial steroid treatment. Whether the apparently improved outlook, as compared to earlier reports, represents a change in the average severity of disease, improved recognition and diagnosis of milder cases, or improvements in therapy is not well understood. Patients demonstrating a favorable response usually retain an independent existence on steroid maintenance therapy. The relapse rate is approximately 25-30%.
  • Characteristically, patients with severe systemic vasculitis have a poor response to the initial phases of treatment. Both systolic and diastolic dysfunction associated with cardiomyopathy may improve with steroid therapy, although very low myocardial shortening fractions may not improve with this therapy. The outlook for patients with severe systemic vasculitis is guarded because they have a considerable risk for dependent existence and progressive decline or premature death. The overall mortality rate may be as high as 25% within 5 years of diagnosis, half of these patients dying directly from vasculitis and half from secondary complications of vasculitis. The patients at highest risk for death or severe morbidity are those with severe myocardial or gastrointestinal vasculitis.
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Contributor Information and Disclosures
Author

Robert Stanley Rust, Jr, MD, MA Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia School of Medicine; Chair-Elect, Child Neurology Section, American Academy of Neurology

Robert Stanley Rust, Jr, MD, MA is a member of the following medical societies: Child Neurology Society, Society for Pediatric Research, American Headache Society, International Child Neurology Association, American Academy of Neurology, American Epilepsy Society, American Neurological 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.

Kenneth J Mack, MD, PhD Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic

Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, Society for Neuroscience

Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, Child Neurology Society

Disclosure: Have stock from Cellectar Biosciences; have stock from Varian medical systems; have stock from Express Scripts.

Additional Contributors

Robert J Baumann, MD Professor of Neurology and Pediatrics, Department of Neurology, University of Kentucky College of Medicine

Robert J Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, Child Neurology Society

Disclosure: Nothing to disclose.

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