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Churg-Strauss syndrome: survival for 26 years
Preeti Vemuri1, Paul A Greenberger, Roy Patterson
1Ernest S. Bazley Asthma and Allergic Diseases Center of the Department of Medicine, Northwestern Memorial Hospital and Northwestern University Medical School, Chicago, Illinois, USA.
Background:
Churg-Strauss syndrome (CSS), a necrotizing vasculitis characterized by asthma and eosinophilia, was described initially in 1951. Before the use of oral corticosteroids, the average patient survived for only a few months. Today with the use of oral corticosteroids and immunosuppressants, survival has increased significantly.
Methods:
We report the case of a patient with CSS treated with prednisone and azathioprine. A review of the English literature was performed with MEDLINE from 1966 to the present using these keywords: Churg-Strauss syndrome, survival, prognosis, morbidity, mortality, and treatment.
Results:
This patient survived 26 years after the diagnosis of CSS and died without autopsy findings of active vasculitis. This is the longest reported survival with CSS in the English literature to our knowledge. The patient's disease course was marked by two acute (vasculitic) episodes, with intermittent subacute disease, and finally a state of disease remission.
Conclusions:
Survival in patients with CSS can be prolonged with early initiation of corticosteroids and immunosuppressants, close outpatient followup, and prompt, aggressive treatment of relapses. This case exemplifies the disease progression and remission as reported by other authors. We propose that CSS can be classified into acute (vasculitic), subacute, and remittable stages.