Churg-Strauss, syndrome
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(Allergic granulomatous angiitis, eosinophilic granulomatosis with polyangeitis)
Rare (1/100,000). Sporadic, likely of autoimmune origin. Systemic disease that combines asthma, transient pulmonary infiltrates, hypereosinophilia, and vasculitis involving many organs (lungs, heart, eyes, CNS, gastrointestinal tract, skin). More common in asthmatic patients: intake of leukotrienes receptor antagonists could be a contributing factor. Onset after 15 years of age.
Its evolution usually follows 3 successive phases:
- prodromal: asthma with or without allergic rhinitis
- steady-state phase: blood and tissue eosinophilia (pneumonia or eosinophilic gastroenteritis)
- vasculitis (skin damage in 2/3 of cases).
Cardiac involvement (coronary heart disease, rhythm disturbances, heart failure, cardiomyopathy) is of poor prognosis.
Diagnostic criteria of the American College of Rheumatology: presence of 4 of the 6 following: asthma, eosinophilia > 10 %, neuropathy, pulmonary infiltrates, paranasal sinus abnormalities and eosinophilic vasculitis.
Treatment: mepolizumab (anti-TL5) subcutaneously once every 4 weeks. Corticosteroid therapy with high-dose immunosuppression in case of failure.
Anesthetic implications:
echocardiography; hyperreactivity of the airways; side effects of corticosteroid therapy or immunosuppression
References :
Updated: June 2022