To ascertain the frequency and clinicoradiologic characteristics of pulmonary involvement in hypereosinophilic syndrome (HES).
Retrospective review of patients with HES encountered over a 5-year period from January 1, 2004 to December 31, 2008 was completed. Inclusion criteria included (1) evidence of chronic illness lasting more than 6 months with blood eosinophilia of greater than 1,500/ul (> 1.5 × 109/L) present on at least 2 occasions; (2) absence of other identifiable etiologies for eosinophilia including drugs, parasitic infection, malignancies, and allergic disease; (3) presence of signs and symptoms of organ dysfunction; (4) absence of recognized specific disease entities including Churg Strauss vasculitis, chronic eosinophilic pneumonia, and eosinophilic fasciitis.
A total of 49 patients (25 males and 24 females) with a median age of 50 years (range, 12 to 88 years) met the criteria. Nineteen patients (39%) had coexisting asthma. Eighteen patients (37%) had a history of present or past tobacco use. Thirty-two patients (65%) had respiratory symptoms including dyspnea, cough, and/or wheezing. Chest imaging studies were abnormal in 20 patients (41%). CT scan abnormalities included parenchymal lung infiltrates in (17 patients, 35%), intrathoracic lymphadenopathy (7 patients, 14%), and pleural effusion (9 patients, 18%). Parenchymal lung infiltrates included ground-glass opacities (11 patients, 65%), consolidation (7 patients, 41%), reticulation (3 patients, 18%), nodular infiltrates (4, 24%), and honeycombing (1 patient, 6%). Pulmonary function data available in 29 patients revealed obstruction in 13 patients (45%) and restriction in one (3%). Treatment included corticosteroids in 43 patients (88%). Clinical course was characterized by improvement in 31 patients (63%). Three patients (6%) died during the follow-up period (median, 12 months). Cause of death was multi-organ failure in two patients.
Pulmonary manifestations are relatively common in patients with hypereosinophilic syndrome. Although many patients had respiratory symptoms, they were not always due to pulmonary involvement and radiologic findings were variable. There was a relatively high prevalence of asthma in these patients.
There is a high prevalence of asthma and variable radiologic findings in HES.
Megan Dulohery, No Financial Disclosure Information; No Product/Research Disclosure Information