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    Drugs & Diseases > Neurology

    Churg-Strauss Disease Follow-up

    Updated: Jul 21, 2020
    • Author: Nir Shimony, MD; Chief Editor: Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA  more...
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    Follow-up

    Further Outpatient Care

    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

    Patients hospitalized for treatment of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), 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

    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 (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), 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

    Particularly characteristic complications of the second phase of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), 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

    Once an appropriate therapeutic intervention is undertaken, a good response usually is achieved within 4 weeks. Thereafter, Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), can usually be well controlled with low maintenance steroid doses.

    Most individuals who showed favorable response to corticosteroid treatment of EGPA associated neuropathy do not manifest disease relapse within the ensuing 8 months.

    Individuals with neuropathy associated with EGPA who show poor response to initial corticosteroid treatment may experience improvement once cyclophosphamide is administered.

    With modern therapy, the outlook for EGPA 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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