Long-term Follow-up of Patients with Diffuse Panbronchiolitis after Erythromycin Therapy

Kim, Cheol Hyeon; Kho, Won Jung; Jang, Seung Hun; Yoo, Chul Gyu; Kim, Young Whan; Heo, Dae Seog; Han, Sung Koo; Shim, Young Soo

Abstract

BACKGROUND: Diffuse panbronchiolitis(DPB) is a chronic inflammatory disease affecting the respiratory bronchioles which was first described in Japan in 1966. DPB is prevalent in Japan and is known to be very rare in western countries. The first cases of DPB were reported in Korea in 1992 and the number of the patients has been increasing. The prognosis of DPR had been very poor because there had been no effective treatment for the disease. Hut it has been dramatically changed since the introduction of low-dose long-term erythromycin therapy. In Korea, there is rare experience of 1ong-term follow-up of DPH patients and we presents the results of mean 21.6 months of follow-up after erythromycin treatment.
METHODS
We analyzed the long-term follow-up data of 25 DPH patients who were diagnosed in Seoul National University Hospital during the period from September 1989 to December 1994 and followed up more than 6 months with erythromycin therapy. We tried erythromycin 250mg b.i.d. on all the patients and analyzed the changes of subjective symptoms, physical signs, pulmonary function tests, and chest X-rays.
RESULTS
1) The mean follow-up period was 21.6 months. 2) Subjective symptoms improved in 96% of the patients within 3 months and 76% of the patients showed no symptom after 18 months of treatment. 3) Crackles and wheezing decreased in all patients within 3 months and completely disappeared in 76% of the patients after 18 months of treatment. 4) Diffuse small nodular lesions on chest X-ray decreased in 56% of the patients within 3 months and chest PA was normal in 32% of the patients after 12months of treatment. 5) FVC and FEV1 increased remarkably during the first 3 months and slowly increased thereafter, reaching normal level after 12 months of treatment. FEV1/FVC was 60.4% before treatment and in- creased slowly reaching 76.1% after 24 months of treatment. 6) Erythromycin therapy could be finished in 7 patients. The mean duration of medication was 26 months and no evidence of recurrence was found in 6 months of follow-up. 7) No patients had experienced the side effect of erythromycin,
CONCLUSION
The prognosis of DPR is very goad when treated with erythromycin. And at least 2 years of erythromycin treatment seems to be needed for DPB patients.