• The Korean Journal of Physiology and Pharmacology
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• v.11 no.6
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• pp.259-262
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• 2007

Diffuse panbronchiolitis (DPB) is a pulmonary disease characterized by chronic inflammation of the bronchioles and chronic infiltration of inflammatory cells in the lungs. Macrolides are effective therapeutic agents for chronic respiratory tract diseases, such as DPB. However, the mechanisms by which macrolides modulate the immune responses in patients with DPB remain unclear. To understand clinical efficacy for the treatment of DPB by macrolides, the effects of erythromycin (EM) on the expression of pro-inflammatory cytokines such as interleukin-6 (IL-6) and interleukin-8 (IL-8) by human neutrophils were examined. Pre-treatment with EM significantly decreased the expression of IL-6 and IL-8 transcripts by lipopolysaccharide (LPS)-stimulated human neutrophils. EM also reversed the enhanced survival of human neutrophils by LPS. These data indicate that EM has achieved therapeutic effect for patients with DPB, in part, through decreasing the expression of pro-inflammatory cytokines and the survival of neutrophils.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.97-100
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• 2005

Diffuse panbronchiolitis(DPB) is a chronic inflammatory airway disease predominantly affecting respiratory bronchioles, with distinct clinicopathological profiles. It was first described in 1966 by Yamanaka et al. The etiology of DPB is not yet clear, and the natural history of the disease is respiratory failure leading to cor pulmonale and ultimately death. But the long-term use of low-dose macrolide has proven to be highly effective in treating patients with DPB. Usual age at diagnosis is over 40. A few cases of DPB have been reported in Korea since 1992 but there have been no reports in children. We experienced a case of DPB in a 12-year-old girl. Therefore, we report the case with a brief review of the related literature.

• Tuberculosis and Respiratory Diseases
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• v.41 no.2
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• pp.127-134
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• 1994

Background : Diffuse panbronchiolitis(DPB) is a chronic inflammatory lung disease of unknown etiology which has characteristic clinical, radiological and pathological features, and is distinguished from bronchial asthma, chronic bronchitis, pulmonary emphysema, bronchiectasis, or alveolitis. Clinically, patients with DPB have chronic cough, purulent sputum, exertional dyspnea, and finally respiratory failure. Until a few years ago, the prognosis of DPB had been thought to be very grave, because there had been no effective treatment for the disease. But recent1y, low-dose long-term erythromycin was found to be very effective on DPB. Even though DPB is prevalent in Japan, and is known to be rare outside of Japan, we have a1ready reported the clinical features of 16 DPB cases in Korea. We tried low-dose long-term erythromycin on DPB patients and analyzed the clinical effect of erythromycin. Methods : We analyzed the changes of subjective symptoms, physical signs, pulmonary function tests and chest X-rays on 14 DPB patients with more than 6 months erythromycin treatment during the period from September 1989 to August 1992 in Seoul National University Hospital. Results : 1) Subjective symptoms improved in all patients within 2-3 months, and 54.5% of the patients showed no symptom after one year of treatment. 2) Crackles and wheezing decreased in 92.9% of the patients after 3 months and completely disapppeared in 63.5% of the patients after one year of treatment. 3) FVC and FEV1 increased remarkably during the first 3 months, and slowly increased thereafter, reaching normal level after one year of treatment. 4) Small nodular lesions on chest X-ray decreased in all patients, and chest PA was normal in 36.4% of the patients after one year of treatment. 5) There was side effect in one patient, stopping medication because of dyspepsia. One patient stopped medication because of no symptom after 16 months of treatment, but her symptom recurring after one month, improving again after retreatment. Conclusion: Low-dose long-term erythromycin showed ramarkable effectiveness on DPB. Further studies are needed on the mechanism of the drug and the duration of the treatment.

• Tuberculosis and Respiratory Diseases
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• v.39 no.3
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• pp.271-277
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• 1992

Diffuse panbronchiollitis (DPB), a rare progressive disorder, has lately been receiving increasing attention. DPB is a disease of obscure etiology, characterized by chronic inflammation localized mainly in the region of respiratory bronchiole just distal to the terminal bronchioles. In 1983, Homma and coworkers reported 82 cases of a new clinicopathological entity, DPB, in Japan. Also DPB is a disease largely restricted geographically to Japan but the prevalence in other countries is extremely low. Histoloically, it is characterized by a suppurative bronchiolitis involving primarily the respiratory and terminal bronchioles with subsequent progression to bronchiectasis. The disease progresses rapidly and results in respiratory failure due to repeated respiratory infections. We experienced a cases of DPB accompanied with chronic maxillary sinusitis in both sinuses. Diagnosis of DPB was comfirmed by pathological results from thoracoscopic lung biopsy, typical radiological findings, clinical symptoms and pulmonary function test. After treatment with erythromycin for 6 months, the patient's condition and the typical micronodular densities on the chest radiography improved. A few case of DPB was reported in Korea. We report a case of DPB through thoracoscopic lung biopsy.

• Tuberculosis and Respiratory Diseases
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• v.44 no.1
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• pp.124-135
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• 1997

Background : Diffuse panbronchiolitis(DPB) is a disease characterized clinically by chronic cough, expectoration and dyspnea; and histologically by chronic inflammation localized mainly in the region of the respiratory bronchiole. It is prevalent in Japanese, but is known to be rare in Americans and Europians. Only a few cases in Chinese, Italians, North Americans and Koreans have been reported. It is diagnosed by characteristic clinical, radiological and pathologic features. High-resolution CT(HRCT) is known to be valuable in the study of the disease process and response to therapy in DPB. To our knowledge, there has been no correlation of its appearance on HRCT with the severity of the disease process, and radioaerosol scan(RAS) of the lung has not previously been used for the diagnosis of DPB. Method : During recent two years we have found 12 cases of DPB in Kangnam St. Mary's Hospital, Catholic University Medical College. We analysed the clinical characteristics, compared HRCT classifications with clinical stages of DPB, and determined characteristic RAS manifestations of DPB. Results : 1. The ages ranged from 31 to 83 years old(mean 54.5 years old), and male female ratio was 4:8. 75%(9/12) of patients had paranasal sinusitis, and only one patient was a smoker. 2. The patients were assigned to one of three clinical stages of DPB on the basis of clinical findings, sputum bacterology and arterial blood gas analysis. of 12 cases, 5 were in the first stage, 4 were in the second stage, and 3 were in the third stage. In most of the patients, pulmonary function tests showed marked obstructive and slight restrictive impairments. Sputum culture yielded P.aeruginosa in 3 cases of our 12 cases, K.pneumoniae in 2 cases, H.influenzae in 2 cases, and S.aureus in 2 cases. 3. Of 12 patients, none had stage I characteristics as classified on HRCT scans, 4 had slage II findings, 5 had stage III findings, and 3 had stage IV characteristics. 4. We peformed RAS in 7 of 12 patients With DPB. In 71.4% (5/7) of the patients, RAS showed mottled aerosol deposits characteristically in the transitional and intermediary airways with peripheral airspace defects, which contrasted sharply with central aerosol deposition of COPD. 5. There were significant correlations between HRCT stages and clinical stages(r= 0.614, P < 0.05), between HRCT types and Pa02(r= -0.614, P < 0.05), and between HRCT types and ESR(r= 0.618, P < 0.01). Conclusion : The HRCT classifications correspond well to the clinical stage. Therfore in the examination of patients with DPB, HRCT is useful in the evaluation of both the location and severity of the lesions. Also, RAS apears to be a convenient, noninvasive and useful diagnostic method of DPB.

• Tuberculosis and Respiratory Diseases
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• v.52 no.1
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• pp.37-45
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• 2002

Background: Diffuse panbronchiolitis(DPB) is a chronic inflammatory lung disease that presents as coughing, copious sputum, exertional dyspnea, which progresses to bronchiectasis. The pathogenesis of bronchiectasis is controlled by inflammatory mediators, which are closely related to mucus hypersecretion, goblet cell dysplasia. In recent studies, the epidermal growth factor receptor(EGFR) system was reported to be associated with this process. It was hypothesized that a relationship exists between goblet cell dysplasia, EGFR expression, and inflammatory mediators produced by neutrophil. Method: Alcian blue/periodic acid -Schiff(AB/PAS) stain, MUC5AC, EGFR, CD16 immunohistochemical stain were examined to investigate a role for the EGFR system in a mucus hypersecretion in DPB using the lung biopsy specimens from 13 DPB patients and 6 controls. Results : In the DPB group, the AB/PAS and MUC5AC -stained areas were $8.31{\pm}3.36%$, $11.46{\pm}4.68%$, respectively. In the control group, the AB/PAS- and MUC5AC-stained areas were $50.5{\pm}5.77%$, $53.3%{\pm}6.67%$, which was significantly larger than in the DPB group (each comparison, p<0.05). The percentage of EGFR expression was $9.54{\pm}4.95%$ in the DPB group, but zero in of the control group. The extent of neutrophilic infiltration was $71.92{\pm}3.71$/5HPF in the DPB group and $45.0{\pm}5.73$/5HPF in the control group, which was statistically significant(p=0.002). Conclusion: The EGFR system is highly related to goblet cell dysplasia, mucus hypersecretion and neutrophilic inflammation in DPB.

• Tuberculosis and Respiratory Diseases
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• v.39 no.1
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• pp.28-34
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• 1992

Background: Diffuse panbronchiolitis (DPB) is a chronic obstructive pulmonary disease distinguished from bronchial asthma, chronic bronchitis, pulmonary emphysema, bronchiectasis, or alveolitis. It is prevalent in Japan, but is known to be rare outside of Japan. Only a few cases in Chinese, Italian, Noirth American and Korean have been reported. During recent two years we have found 16 cases of DPB in Seoul National University Hospital and analyzed the clinical characteristics. Method: We reviewed the patients' age, sex, duration of illness, smoking history, occupational history and past medical history. And we analyzed patients' symptoms, physical signs, laboratory findings and responses to treatment. Results: 1) Male: female ratio was 2.2:1, the ages ranged from 27 to 72 years old, and the duration of disease varied from 1 to 20 years. 2) Most of the patients were being treated as bronchiectasis, miliary tuberculosis, chronic bronchitis or bronchial asthma before they were diagnosed as DPB. 3) Only one patient was a smoker, and 25% of patients had the occupational history of exposure to particles or gas. And all patients had paranasal sinusitis. 4) High resolution computed tomography (HRCT) was very useful in diagnosing the disease in most of the patients. 5) PFT showed obstructive and restrictive abnormalities, and blood gas revealed hypoxemia in most of the patients. 6) Pseudomonas aeruginosa was isolated in the sputum of four patients (25%). 7) The titers of cold hemaglutinin, RA factor and CRP were elevated in most of the patients. 8) Most of the patients improved after treatment with erythromycin. Conclusion: DPB might not be a rare disease in Korea. So DPB should be suspected in a patient who has chronic cough, sputum, dyspnea and diffuse fine nodular chest X-ray abnormality. HRCT and open lung biopsy should be considered in a patient suspected of DPB.

• The Korean Journal of Nuclear Medicine
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• v.28 no.2
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• pp.192-199
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• 1994

Diffuse pahbronchiolitis(DPB) is a relatively new, chronic, nonspecific, inflammatory disease of the lung that typically involves the airways in the "transitional" zone of the lung. Clinically, DPB strongly resembles chronic obstructive pulmonary disease (COPD) and the distinction between the two is crucial because the former is often fatal, requiring different therapeutic strategies. This study was prospectively carried out to assess diagnostic usefulness of radioaerosol scan(RAS) in DPB. RAS findings were analyzed with respect to the location and extent of abnormal aerosol deposition in the lung divided into the central, intermediary, and transitional airways and the peripheral airspaces. RAS showed mottled aerosol deposits characteristically in the transitional and intermediary airways with peripheral airspace defects. Such a deposition pattern contrasted sharply with central aerosol deposition of COPD. In conclusion, RAS appears to be a convenient, noninvasive, and useful diagnostic method of DPB.