Table 3—

Clinical guidelines for diffuse panbronchiolitis therapy#

Macrolides should be applied soon after the diagnosis is made, as there is a better clinical response in the earlier stage
 First choice: erythromycin 400 or 600 mg orally
 When it is no longer effective or if administration should be stopped because of adverse effects or drug interactions
 Second choice: clarithromycin 200 or 400 mg orally or roxithromycin 150 or 300 mg orally
Assessment of response and duration of treatment
 Although clinical response can usually be noted within 2–3 months, the treatment should be continued for ≥6 months, after which time the overall response should be assessed
 Treatment should be completed after a period of 2 yrs when clinical manifestations, radiological findings and pulmonary function measurements have improved and stabilised and without any significant impairment of daily activity
 Treatment should be resumed if symptoms reappear after the cessation
 In cases of an effective cure in advanced cases with extensive bronchiectasis or respiratory failure, treatment should be continued for >2 yrs
  • #: developed by a working group of the Diffuse Lung Disease Committee of the Ministry of Health and Welfare of Japan 67; : 16-member ring macrolides do not seem to be effective.