Cryptogenic pulmonary eosinophilia

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Abstract
The clinical and immunological features of 15 cases of cryptogenic pulmonary eosinophilia are reported. There were 10 women (mean age 35.4 yr) and 5 men (mean age 42 yr). Eight gave previous history of asthma and seven had none. Of the 15 patients, 13 negative skin tests to common allergens [pollen, house dust, animal danders, molds, foods, feathers]. Many features of a systemic illness were present in the asthmatic and non-asthmatic groups including anemia, weight loss, fever and a grossly raised ESR [erythrocyte sedimentation rate]. An absolute polymorphonuclear leukocytosis was frequent as well as the obligatory increase in blood eosinophils used as criteria for inclusion. Hepatomegaly (3 cases), splenomegaly (4 cases) and hilar node enlargement (1 case) were seen in the group without asthma. Evidence of renal involvement or necrotizing vasculitis was notably absent, and the response to small doses of corticosteroids was dramatic. Immunologically, the striking feature was a disproportionate increase in blood eosinophils compared with only minor elevations in the total serum IgE [immunoglobulin E] levels. This stands in contrast to patients with bronchopulmonary aspergillosis and helminth infestation. Studies of cytophilic antibodies using histamine liberation after challenge with antibodies to Ig sub-classes in 6 patients showed a marked increase in IgG2 and lesser increases of IgE and IgG3. No evidence of antibodies specific to Aspergillus fumigatus was found. The amount of cytophilic antibody was in contrast to that found in bronchopulmonary aspergillosis.