A benefit of corticosteroid treatment was not shown in previous studies. 16 Daily EM must be administered for long periods. If the response is good after 6 months, the drug can be discontinued after 2 years. EM is known to affect the function of lymphocytes and macrophages, as well as to inhibit hypersecretion and neutrophil accumulation. Until the development of EM treatment, DPB had an extremely poor prognosis.
Death is most often due to pneumonia or cor pulmonale.
Pseudomonas superinfection has been found to be major reason for the poor prognosis. Figure 4 shows the survival in three groups. The patients who presented with disease between 1970 and 1979 were not given EM until a later stage. Those who presented between 1980 and 1984 were not given EM at the time of initial presentation, but did receive the drug later. The patients seen after 1985 were given EM from the initial presentation.