Clinical Comprehensive Analysis Of 36 Patients With Diffuse Panbronchiolitis

Objective To improve the diagnosis and treatment level of diffuse panbronchiolitis (DPB) by the analysis of laboratory tests, imaging features, pathology, diagnosis, treatment and prognosis of Chinese patients with DPB.Methods The details of two cases of DPB treated at the second affiliated hospital of Zhejiang University medical college were retrospectively analyzed and reviewed together with those of 34 cases previously reported from the literature.Results Among the 36 patients, 22 cases were male(61.1%),and 14 cases were female(38.9%)·Mean age was 52.7±18.5 years (range,13 to 82 years)·Course of disease when diagnosed was 9.5±7.3 years (range,2 months-30 years).The clinical features include chronic cough (36/36,100%), expectoration (36/36,100%), exertional dyspnea (35/36,97.2%), hemoptysis (7/36,19.4%), crackles (36/36,100%), digital clubbings (15/36,41.7%).32 patients had a history of sinusitis (88.9%).12 of 24 patients (50.0%) had an elevation of cold agglutinin. One of two patients possessed the HLA-B54 antigen.17 of 28 cases (60.7%) had P. aeruginosa in their sputum. CT scan shows bilateral, diffuse, small centrilobular nodular. Significantly in inferior lobe. Mean FEV1/FVC was 56.4±10.7%, FEV1% pred was 48.4±14.3%. Mean PaO2 was 66.0±10.4mmHg, PaCO2 was 47.8±15.7mmHg; 4 patients had bronchoscopic biopsy, all show lymphocytes, plasma cells infiltrate around bronchioles.27 patients had been misdiagnosed as other diseases, the most common misdiagnosing disease is chronic bronchitis (13/27,48.1%), bronchiectasis (13/27,48.1%), asthma (6/27, 22.2%), phthisis miliaris (4/27,14.8%);32cases received therapy of 14-membered or 15-membered ring macrolides. Doses are 0.3-0.6g/d, after 3 months to 2years of treatment (mean 6.2±4.9 months), the symptoms were improved in 16 patients (94.1%),1 patient (5.9%) died after six months.Conclusion DPB is not rare in Chinese population, and the clinical feasures are not specific.DPB is easily misdiagnosed. The prognosis of DPB is good if treated early. Long-term low-dose macrolides therapy is generally accepted. To increase the cognition of DPB is the key to decrease misdiagnosis rate and improve prognosis.