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Diffuse panbronchiolitis (DPB)[1] is an idiopathic (of unknown cause) inflammatory lung disease,[2] considered to be a type of COPD.[3][4] It is a severe, progressive form of bronchiolitis, mainly affecting the respiratory bronchioles (the section of the bronchioles involved in gas exchange).[2]
The term “diffuse” refers to the lesions which appear throughout both lungs, while the term “pan-” refers to the inflammation found in all layers of the respiratory bronchioles, both terms describing a common pathology for the disease.[2]
If left untreated, DPB is fatal, usually progressing to bronchiectasis, an irreversible lung conditon that causes respiratory failure.[2]
DPB has the highest incidence among Japanese.[2][5][6]
Korean,[7][4][8] Chinese[6][9] and Thai cases[10] have been reported as well. A genetic predisposition among East Asians is indicated,[3][4][8] with a lower worldwide prevalence of DPB still usually related to Asian ancestry.[11] However, rare cases of DPB, being those in the western world in individuals with non-Asian lineage,[12] have also been noted.[13][14][12]
DPB can be characterized by a number of symptoms and histological features (those detected by analysis of tissues).
These include: chronic bronchial and bronchiolar inflammation, lung tissue lesions, proliferation of lymphocytes (white blood cells that fight infection), neutrophil granulocytes (white blood cells involved with inflammation) and foamy histiocytes (tissue macrophages) in the lung lumen;[2][5] detectable haemophilus influenzae (an opportunistic bacterium),[2] pseudomonas aeruginosa (another opportunistic bacterium) and pus in the sputum (coughed-up phlegm);[1][5] chronic sinusitis (inflamed paranasal sinuses),[13][14] hypoxemia (low levels of oxygen in the blood),[15] nodules within terminal and respiratory bronchioles in both lungs,[10][12] dyspnea (shortness of breath),[9][14] elevated IgG and IgA (classes of immunoglobulins),[6] occurrences of elevated rheumatoid factor (an indicator of autoimmunity),[6] and hemagglutination (clumping of red-blood cells in response to the presence of antibodies in the blood).[6][16]
If left untreated, as DPB progresses, signs of bronchiectasis begin to present themselves. These symptoms include chronic shortness of breath, severe cough, pooling of sputum, thickening of bronchiolar walls, hypoxemia, and dilation (enlargement) of the bronchiolar passages.[1][14] This eventually becomes life-threatening, leading to respiratory failure.[1]
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