1740 CHURG-STRAUSS SYNDROME (ALLERGIC GRANULOMATOSIS)IN AM ADOLESCENT MALE: DEVELOPMENT OF CARDIOMYOPATHY DURING TREATMENT WITH STEROIDS

Abstract

This case represents the first in-depth prospective report of an adolescent with Churg-Strauss syndrome and the first to be reported in the pediatric literature. The patient presented with pulmonary lesions, vasculitis, severe gastrointestinal involvement and leukocytosis with hypereosinophilia.

Pathological examination of a wedge section from the lung revealed necrotizing granulomata filled with necrotic eosinophils. Arteries and veins of all sizes were affected with perivascular eosinophilic-rich palisaded giant cell granulomata with evidence of partial or complete occlusion.

The patient was treated with prednisone with excellent resolution of his symptoms. He had recurrent episodes of pneumonia and wheezing which responded to increased levels of prednisone plus bronchodilators and antibiotics.

Cardiomyopathy developed after two years while on steroid therapy. ECG studies revealed left anterior hemiblock, frontal QRS axis of -30°, LA and LV hypertrophy and non-specific ST and T wave changes. Marked depression of the ejection fraction was also noted on echocardiogram studies. He was treated with digoxin, diuretics and increased amounts of steroids. Although clinical improvement has been noted, objective studies show minimal resolution of his cardiomyopathy.

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