Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Follow-up

Updated: Dec 24, 2018
  • Author: Spencer T Lowe, MD; Chief Editor: Herbert S Diamond, MD  more...
  • Print
Follow-up

Further Inpatient Care

Eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome) 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, erythrocyte sedimentation rate, and eosinophil count should be monitored. In EGPA patients with antineutrophil cytoplasmic antibodies (ANCAs), the ANCA level does not correlate well with disease activity. [45]

Next:

Inpatient & Outpatient Medications

Inpatient medications include the following:

  • Prednisone: Start at 0.5-1 mg/kg/d PO.

  • Methylprednisolone is administered in intravenous (IV) form at higher doses in patients with major organ involvement, including cardiac, pulmonary (hemorrhage), renal, or neuropathy.

  • Cyclophosphamide is administered in patients with severe or life-threatening complications. [46, 47] The single IV 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 similarly as in microscopic polyangiitis or granulomatosis with polyangiitis (Wegener granulomatosis). [48]

  • Plasmapheresis is probably not helpful. [42]

  • Interferon alpha might be helpful in treatment. [49, 50]

Previous
Next:

Prognosis

Overall, without treatment, the 5-year survival rate in EGPA 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

Previous