Of 65 patients presenting with
pulmonary eosinophilia to one Respiratory Unit during a 20-year period, 12 (18%) had systemic features associated with their
pulmonary disease. Eleven had
fever, three night sweats, three
arthralgia, three vasculitic rashes and two
weight loss. Anaemia,
myalgia,
peripheral neuropathy,
mononeuritis,
pericardial effusion and photosensitivity
rash were each recorded in single patients. None had evidence of
hypersensitivity to drugs, helminthes or other
allergens. Ten of the 12 patients could be classified as cryptogenic
pulmonary eosinophilia and two as
Churg Strauss syndrome. Ten were female. The maximum recorded eosinophil counts were higher in the 12 patients with systemic features compared with the remaining 53 patients [mean (SD) 5613 (3883) vs. 2359 (3046) x 10(6) 1(-1), P < 0.02], whereas both
asthma and recurrent episodes of
eosinophilia were significantly less common.
Steroid therapy achieved a good clinical response and radiological clearing in the majority of patients. All 12 patients were treated with prolonged duration oral
prednisolone [mean (SEM) dose 8.5 (3.8) mg day-1 duration 5.5 (1.3) years]. The two patients with
Churg Strauss syndrome required
azathioprine in addition to long-term
prednisolone. There were no deaths and currently four patients are off all
steroids and six receive less than 5 mg day-1. During a median follow-up period of 11 years, there was no significant decline in FEV1 or VC, measured as percent predicted values. Persistent radiographic abnormalities consistent with
fibrosis or
bronchiectasis were not seen.(ABSTRACT TRUNCATED AT 250 WORDS)