PURPOSE: Severe asthma with rhinosinusitis and peripheral eosinophilia is not unusual in children. These children have eosinophilia on the basis of atopic disease and have positive skin tests and elevated IgE levels. In the absence of atopy, progression to pulmonary and/or systemic eosinophilic vasculitis (Churg-Strauss Syndrome) is often seen in adults but rarely in children. We report such a case.
METHODS: We have followed a 12 year old girl for over 2 years with severe asthma, peripheral, nasal, and sputum eosinophila, negative skin tests, elevated IgE levels, and chronic airflow obstruction with chronic rhinusinusitis on CT-scan who required multiple courses of systemic corticosteroids (cs)in addition to high dose inhaled cs and leukotriene modifiers to control her disease until admitted to ICU this past February with full-blown systemic vasculitis.
RESULTS: On admission she had evidence of peripheral eosinophilia (45%), pulmonary eosinophilic pneumonitis, myocarditis ( troponin 11.72ng/ml, normal< 0.1), and gastroenteritis. A CT-scan of the thorax was compatible with extensive involvement characteristic of eosinophilic pneumonitis. Echocardiogram revealed severe myocardial dysfunction with ejection fraction of 15%. Colon and rectal biopsys were diagnostic of Churg-Strauss Syndrome and were characterized by extensive eosinophilic granulomatous vasculitis. She received high-dose systemic cs with excellent response to therapy. She was discharged after 11 days in ICU on oral cs, inhaled cs, and intranasl cs. On follow up, the dose of oral cs was reduced to 10mg prednisolone bid while she remained free of systemic manifestation.
CONCLUSION: Churg-Strauss Syndrome should be considered in the differential diagnosis of children with severe asthma, eosinophilia, and chronic rhinosunusitis who have negative skin tests but elevated IgE. These patients require immunosuppressants rather than immunotherapy or anti-IgE therapy.
CLINICAL IMPLICATIONS: Churg-Strauss Syndrome with systemic involvement of the heart and gastrointestinal tract- although rare in childhood- carries a significant mortality (45% in some adult series) and should be treated aggressively. The concern over leukotriene antagonists as potential inducers of this syndrome has not received further support from their post-marketing experience now spanning well over a decade.
DISCLOSURE: Gabriel Ortiz, None.