Diffuse panbronchiolitis is an inflammatory lung disease of unknown cause. DPB causes severe inflammation and nodule-like lesions of terminal bronchioles, chronic sinusitis, and intense coughing with large amounts of sputum production. It is associated with progressive respiratory failure, hypercapnia, and can eventually lead to pulmonary hypertension and cor pulmonale. The highest incidence occurs among Japanese people, followed by Koreans. Daily treatment of DPB with macrolide antibiotics such as erythromycin eases symptoms and increases survival time, but the disease currently has no known cure.
About Diffuse panbronchiolitis in brief
Diffuse panbronchiolitis is an inflammatory lung disease of unknown cause. DPB causes severe inflammation and nodule-like lesions of terminal bronchioles, chronic sinusitis, and intense coughing with large amounts of sputum production. The disease is believed to occur when there is susceptibility, or a lack of immune system resistance, to DPB-causing bacteria or viruses. The highest incidence occurs among Japanese people, followed by Koreans. Daily treatment of DPB with macrolide antibiotics such as erythromycin eases symptoms and increases survival time, but the disease currently has no known cure. It is associated with progressive respiratory failure, hypercapnia, and can eventually lead to pulmonary hypertension and cor pulmonale. It was recognized as a distinct new disease in the early 1960s and was formally named diffuse panbronsiolitis in 1969. The exact physiological, environmental, or pathogenic cause of the disease is unknown. Several factors are suspected to be involved with its pathogenesis. Genetic predisposition for DPB susceptibility has been localized to two HLA haplotypes common to people of East Asian descent. HLA-B54 is associated. with DPB in the Japanese, while H LA-A11 is. associated with the disease in Koreans. Further, it is possible that a number of genetic recombination events around the disease locus could have resulted in the disease being associated with HLAB locus at 6p21p21 in Japanese and HLAA11 in Korean individuals. The common genetic background of affected individuals were considered in the search for a DPB gene for a suspected disease-susceptibility gene.
It has been suggested that a mutation of a suspected gene called DPB could have occurred on an ancestral chromosome carrying both HLAb54 and HlaA11. This suggests that DPB is located near the 3p21 locus of the gene HLAP-3B, which is located on chromosome 6 in humans. This locus is also believed to be responsible for the common genetic profile of affected Japanese and Korean individuals and for increased susceptibility to the disease. If left untreated, DPB progresses to bronchiectasis, an irreversible lung condition that involves enlargement of the bronchioes, and pooling of mucus in theBronchiolar passages. Typical signs of DPB progression include dilation and thickening of the walls of the lungs and hypoxemia. It can lead to respiratory failure and heart problems, and may also cause pulmonary hypertension, cor pulmonsale, and other conditions such as emphysema. The eventual result of DP B can be respiratory failure or heart problems. It occurs more often in males and usually begins around age 40. It is classified as a form of primary bronchiolaitis, which means that the underlying cause of broncholitis is originating from or is confined to the bron chioles. Unlike DPB, other forms of primary Bronchioliti include bronchiorgitis obliterans, follicular bronchiotitis, respiratory broncholititis, mineral dust airway disease, and a. number of others.
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This page is based on the article Diffuse panbronchiolitis published in Wikipedia (as of Nov. 03, 2020) and was automatically summarized using artificial intelligence.
