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| {{Infobox_Disease | | | {{Infobox_Disease | |
| Name = Diffuse panbronchiolitis | | | Name = Diffuse panbronchiolitis | |
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| {{SI}} | | {{Diffuse panbronchiolitis}} |
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| | {{CMG}} {{AE}} |
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| '''Diffuse panbronchiolitis''' (DPB)<ref name=dpb>{{cite journal |author=Homma H, Yamanaka A, Tanimoto S, Tamura M, Chijimatsu Y, Kira S, Izumi T |title=Diffuse panbronchiolitis. A disease of the transitional zone of the lung |journal=Chest. |volume=83 |issue=1 |pages=63-69 |year=1983 |pmid=6848335 }}</ref> is an [[idiopathic]] (of unknown cause) [[inflammation|inflammatory]] [[lung]] [[disease]],<ref name=dpb06>{{cite journal |author=Poletti V, Casoni G, Chilosi M, Zompatori M |title=Diffuse panbronchiolitis |journal=Eur Respir J. |volume=28 |issue=4 |pages=862-871 |year=2006 |pmid=17012632 }}</ref> considered to be a type of [[chronic obstructive pulmonary disease|COPD]].<ref name=hla98>{{cite journal |author=Keicho N, Tokunaga K, Nakata K, Taguchi Y, Azuma A, Bannai M, Emi M, Ohishi N, Yazaki Y, Kudoh S |title=Contribution of HLA genes in genetic predisposition for diffuse panbronchiolitis |journal=Am J Respir Crit Care Med. |volume=158 |issue=3 |pages=846-850 |year=1998 |pmid=9731015 }}</ref><ref name=hla99>{{cite journal |author=Park MH, Kim YW, Yoon HI, Yoo CD, Han SK, Shim YS, Kim WD |title=Association of HLA class I antigens with diffuse panbronchiolitis in Korean patients |journal=Am J Respir Crit Care Med. |volume=159 |issue=2 |pages=526-529 |year=1999 |pmid=9927368 }}</ref> It is a severe, progressive form of [[bronchiolitis]], mainly affecting the [[respiratory bronchiole]]s (the section of the [[bronchiole]]s involved in [[gas exchange]]).<ref name=dpb06/>
| | {{SK}} Panbronchiolitis; DPB |
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| The term ''"diffuse"'' refers to the [[lesion]]s 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.<ref name=dpb06/>
| | ==[[Diffuse panbronchiolitis overview|Overview]]== |
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| If left untreated, DPB is fatal, usually progressing to [[bronchiectasis]], an irreversible lung conditon that causes [[respiratory failure]].<ref name=dpb06/>
| | ==[[Diffuse panbronchiolitis historical perspective|Historical Perspective]]== |
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| [[Image:diagrama de los pulmones.svg|thumb|left|330px|A simple diagram of the [[respiratory system]]: 1. [[trachea]], 2. [[pulmonary artery]], 3. [[pulmonary vein]], 4. [[alveolar duct]], 5. [[alveolus|alveoli]], 6. [[cardiac notch]], 7. '''[[bronchiole]]''', 8. [[tertiary bronchus|tertiary bronchi]], 9. [[secondary bronchus|secondary bronchi]], 10. [[bronchus|primary bronchi]], 11. [[larynx]] ]] | | ==[[Diffuse panbronchiolitis classification|Classification]]== |
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| == Prevalence == | | ==[[Diffuse panbronchiolitis pathophysiology|Pathophysiology]]== |
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| DPB has the highest incidence among [[Japan]]ese.<ref name=dpb06/><ref name=path>{{cite journal |author=Yanagihara K, Kadoto J, Kohno S |title=Diffuse panbronchiolitis--pathophysiology and treatment mechanisms |journal=Int J Antimicrob Agents |volume=18 |issue=Suppl. 1 |pages=S83-87 |year=2001 |pmid=11574201 }}</ref><ref name=chi>{{cite journal |author=Tsang KW, Ooi CG, Ip MS, Lam WK, Ngan H, Chan EY, Hawkins B, Ho CS, Amitani R, Tanaka E, Inoh H |title=Clinical profiles of Chinese patients with diffuse panbronchiolitis |journal=Thorax |volume=53 |issue=4 |pages=274-280 |year=1998 |pmid=9741370 }}</ref> [[Korea]]n,<ref name=kor>{{cite journal |author=Kim YW, Han SK, Shim YS, Kim KY, Han YC, Seo JW, Im JG |title=The first report of diffuse panbronchiolitis in Korea: 5 case reports |journal=Intern Med. |volume=31 |issue=5 |pages=695-701 |year=1992 |pmid=1504438 }}</ref><ref name=hla99/><ref name=hla00>{{cite journal |author=Keicho N, Ohashi J, Tamiya G, Nakata K, Taguchi Y, Azuma A, Ohishi N, Emi M, Park MH, Inoko H, Tokunaga K, Kudoh S |title=Fine localization of a major disease-susceptibility locus for diffuse panbronchiolitis |journal=Am J Hum Genet. |volume=66 |issue=2 |pages=501-507 |year=2000 |pmid=10677310 }}</ref> [[China|Chinese]]<ref name=chi/><ref name=chitwo>{{cite journal |author=Chen Y, Kang J, Li S |title=Diffuse panbronchiolitis in China |journal=Respirology |volume=10 |issue=1 |pages=70-75 |year=2005 |pmid=15691241 }}</ref> and [[Thailand|Thai]] cases<ref name=thai>{{cite journal |author=Chantarotorn S, Palwatwichai A, Vattanathum A, Tantamacharik D |title=Diffuse panbronchiolitis, the first case reports in Thailand |journal=J Med Assoc Thai. |volume=82 |issue=8 |pages=833-838 |year=1999 |pmid=10511794 }}</ref> have been reported as well. A genetic predisposition among [[East Asia]]ns is indicated,<ref name=hla98/><ref name=hla99/><ref name=hla00/> with a lower worldwide prevalence of DPB still usually related to [[Asia]]n [[ancestry]].<ref name=ew>{{cite journal |author=Hoiby N |title=Diffuse panbronchiolitis and cystic fibrosis: East meets West |journal=Thorax |volume=49 |issue=6 |pages=531-532 |year=1994 |pmid=8016786 }}</ref> However, rare cases of DPB, being those in the [[western world]] in individuals with non-Asian lineage,<ref name=can>{{cite journal |author=Sandrini A, Balter MS, Chapman KR |title=Diffuse panbronchiolitis in a Caucasian man in Canada |journal=Can Respir J. |volume=10 |issue=8 |pages=449-451 |year=2003 |pmid=14679410 }}</ref> have also been noted.<ref name=lat>{{cite journal |author=Martinez JA, Guimaraes SM, Ferreira RG, Pereira CA |title=Diffuse panbronchiolitis in Latin America |journal=Am J Med Sci. |volume=319|issue=3 |pages=183-185 |year=2000 |pmid=10746829 }}</ref><ref name=usa>{{cite journal |author=Fitzgerald JE, King TE Jr., Lynch DA, Tuder RM, Schwarz MI |title=Diffuse panbronchiolitis in the United States |journal=Am J Respir Crit Care Med. |volume=154 |issue=2 pt. 1 |pages=497-503 |year=1996 |pmid=8756828 }}</ref><ref name=can/>
| | ==[[Diffuse panbronchiolitis causes|Causes]]== |
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| == Symptoms == | | ==[[Diffuse panbronchiolitis differential diagnosis|Differentiating Diffuse panbronchiolitis from other Diseases]]== |
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| DPB can be characterized by a number of symptoms and [[histopathology|histological]] features (those detected by analysis of tissues).
| | ==[[Diffuse panbronchiolitis epidemiology and demographics|Epidemiology and Demographics]]== |
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| These include: chronic [[bronchi]]al and bronchiolar inflammation, lung tissue lesions, proliferation of [[lymphocyte]]s ([[white blood cell]]s that fight infection), [[neutrophil]] [[granulocyte]]s (white blood cells involved with inflammation) and [[histiocyte|foamy histiocytes]] (tissue [[macrophage]]s) in the lung [[lumen]];<ref name=dpb06/><ref name=path/> detectable ''[[haemophilus influenzae]]'' (an [[opportunistic infection|opportunistic]] [[bacterium]]),<ref name=dpb06/> ''[[pseudomonas aeruginosa]]'' (another opportunistic bacterium) and [[pus]] in the [[sputum]] (coughed-up [[phlegm]]);<ref name=dpb/><ref name=path/> [[chronic sinusitis]] (inflamed [[paranasal sinus]]es),<ref name=lat/><ref name=usa/> [[hypoxemia]] (low levels of [[oxygen]] in the [[blood]]),<ref name=rep>{{cite journal |author=Chu YC, Yeh SZ, Chen CL, Chen CY, Chang CY, Chiang CD |title=Diffuse panbronchiolitis: report of a case |journal=J Formos Med Assoc. |volume=91 |issue=9 |pages=912-915 |year=1992 |pmid=1363394 }}</ref> nodules within [[terminal bronchiole|terminal]] and [[respiratory bronchiole]]s in both lungs,<ref name=thai/><ref name=can/> [[dyspnea]] (shortness of breath),<ref name=chitwo/><ref name=usa/>
| | ==[[Diffuse panbronchiolitis risk factors|Risk Factors]]== |
| elevated [[IgG]] and [[IgA]] (classes of [[immunoglobulin]]s),<ref name=chi/> occurrences of elevated [[rheumatoid factor]] (an indicator of [[autoimmunity]]),<ref name=chi/> and [[hemagglutination]] (clumping of [[antibodies]] in the blood).<ref name=chi/><ref name=cold>{{cite journal |author=Schulte W, Szrepka A, Bauer PC, Guzman J, Costabel U |title=Diffuse panbronchiolitis. A differential diagnosis of chronic obstructive lung disease |journal=Dtsch Med Wochenschr. |volume=124 |issue=19 |pages=584-588 |year=1999 |pmid=10365176 }}</ref>
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| 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.<ref name=dpb/><ref name=usa/> This eventually becomes life-threatening, leading to respiratory failure.<ref name=dpb/>
| | ==[[Diffuse panbronchiolitis natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| == Pathogenesis == | | ==Diagnosis== |
| | [[Diffuse panbronchiolitis history and symptoms|History and Symptoms]] | [[Diffuse panbronchiolitis physical examination|Physical Examination]] | [[Diffuse panbronchiolitis laboratory findings|Laboratory Findings]] | [[Diffuse panbronchiolitis chest x ray|Chest X Ray]] | [[Diffuse panbronchiolitis CT|CT]] | [[Diffuse panbronchiolitis MRI|MRI]] | [[Diffuse panbronchiolitis other imaging findings|Other Imaging Findings]] | [[Diffuse panbronchiolitis other diagnostic studies|Other Diagnostic Studies]] |
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| DPB remains idiopathic, which means an exact [[physiological]], [[Environment (biophysical)|environment]]al, or [[pathogen]]ic cause of the disease is unknown.<ref name=dpb06/> However, several known factors are involved with the pathogenesis of DPB.<ref name=hla98/><ref name=hla99/>
| | ==Treatment== |
| | [[Diffuse panbronchiolitis medical therapy|Medical Therapy]] | [[Diffuse panbronchiolitis primary prevention|Primary Prevention]] | [[Diffuse panbronchiolitis secondary prevention|Secondary Prevention]] | [[Diffuse panbronchiolitis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Diffuse panbronchiolitis future or investigational therapies|Future or Investigational Therapies]] |
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| The [[major histocompatibility complex]] (MHC) is a large [[genome|genomic]] region found in most [[vertebrate]]s, that is associated with [[mating]] and the [[immune system]]. It is located on [[chromosome 6]] in humans. A subset of the human MHC is [[human leukocyte antigen]] (HLA), which controls the [[antigen presentation|antigen presenting system]], as part of [[adaptive immunity]] against [[pathogen]]s such as [[bacteria]] and [[virus]]es.
| | ==Case Studies== |
| | [[Diffuse panbronchiolitis case study one|Case #1]] |
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| Genetic predisposition for DPB has been localized to two HLA [[haplotype]]s unique to Asians, particularly of East Asian descent.<ref name=hla98/><ref name=hla00/> [[HLA-B54]] is associated with DPB in Japanese patients,<ref name=hla98/> while [[HLA-A11]] is associated with the disease in Koreans.<ref name=hla99/> One or more [[candidate gene]]s<ref name=orf>{{cite journal |author=Matsuzaka Y, Tounai K, Denda A, Tomizawa M, Makino S, Okamoto K, Keicho N, Oka A, Kulski JK, Tamiya G, Inoko H |title=Identification of novel candidate genes in the diffuse panbronchiolitis critical region of the class I human MHC |journal=Immunogenetics |volume=54 |issue=5 |pages=301-309 |year=2002 |pmid=12185533 }}</ref> (a gene suspected to be responsible for a trait or disease) within this region of [[human leukocyte antigen|class I HLA]] are believed to be the genetic factor responsible for DPB, allowing disease susceptibility<ref name=hla00/> related to the structure of the [[antigen presentation|antigen presenting]] [[molecules]] selected by these genes.<ref name=tap>{{cite journal |author=Keicho N, Tokunaga K, Nakata K, Taguchi Y, Azuma A, Tanabe K, Matsushita M, Emi M, Ohishi N, Kudoh S |title=Contribution of TAP genes to genetic predisposition for diffuse panbronchiolitis |journal=Tissue Antigens |volume=53 |issue=4 pt. 1 |pages=366-373 |year=1999 |pmid=10323341 }}</ref>
| | ==Related Chapters== |
| | | *[[Asthma]] |
| Candidate genes within HLA that are most likely involved with DPB suceptibility include: [[FAM46A|C6orf37]]<ref name=orf/> and [[TAP2]].<ref name=tap/>
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| Another such gene, though not a part of the HLA system, is the gene for [[interleukin 8]] (IL-8)<ref name=il8>{{cite journal |author=Emi M, Keicho N, Tokunaga K, Katsumata H, Souma S, Nakata K, Taguchi Y, Ohishi N, Azuma A, Kudoh S |title=Association of diffuse panbronchiolitis with microsatellite polymorphisms of the human interleukin 8 (IL-8) gene |journal=J Hum Genet. |volume=44 |issue=3 |pages=169-172 |year=1999 |pmid=10319580 }}</ref>
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| located on [[chromosome 4]]. The role of IL-8 to produce inflammation by causing the proliferation of neutrophil granulocytes at any site of pathogenic involvement, in conjunction with strong [[microsatellite]] identification with DPB, implicates IL-8 as another candidate gene associated with DPB pathogenesis.<ref name=il8/> This also supports the idea that several factors, including those unrelated to HLA as well as non-genetic, and unknown factors, may cause the disease.<ref name=il8/>
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| The inflammation common to DPB also provides a means to determine other mechanisms of disease pathogenesis.<ref name=cd8>{{cite journal |author=Kadota J, Mukae H, Tomono K, Kohno S |title=High concentrations of beta-chemokines in BAL fluid of patients with diffuse panbronchiolitis |journal=Chest |volume=120 |issue=2 |pages=602-607 |year=2001 |pmid=11502665 }}</ref> This may be partly due to the persistence of inflammation in DPB, with ''or'' without the presence of the two opportunistic bacteria sometimes found with the disease (''haemophilus influenzae'', ''pseudomonas aeruginosa'').<ref name=path/> Inflammation caused by the [[chemokine]] [[CCL4|MIP-1alpha]] and its involvement with [[CD8|CD8+]] [[T-cell]]s is believed to be one such mechanism of DPB pathogenesis.<ref name=cd8/>
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| Other factors found with DPB play a part in its pathogenesis by sometimes causing minor variations of it.
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| [[Beta defensin]]s, a family of [[antimicrobial]] [[peptide]]s found in the respiratory tract, are responsible for further inflammation in DPB, when associated pathogens like ''pseudomonas aerugenosa'' are present.<ref name=def>{{cite journal |author=Hiratsuka T, Mukae H, Iiboshi H, Ashitani J, Nabeshima K, Minematsu T, Chino N, Ihi T, Kohno S, Nakazato M |title=Increased concentrations of human beta-defensins in bronchoalveolar lavage fluid of patients with diffuse panbronchiolitis |journal=Thorax |volume=58 |issue=5 |pages=425-430 |year=2003 |pmid=12728165 }}</ref>
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| If present in a DPB patient, the [[human T-lymphotropic virus|human T-lymphotropic virus, type I]], a [[retrovirus]], modifies DPB pathogenesis by infecting [[CD4|CD4+]] cells ([[Helper T-cell]]s) and altering there effectiveness in reducing both known and unknown pathogenic involvement with DPB.<ref name=htlv>{{cite journal |author=Yamamoto M, Matsuyama W, Oonakahara K, Watanabe M, Higashimoto I, Kawabata M, Osame M, Arimura K |title=Influence of human T lymphotropic virus type I on diffuse pan-bronchiolitis |journal=Clin Exp Immunol. |volume=136 |issue=3 |pages=513-520 |year=2004 |pmid=15147354 }}</ref> Conversely, an onset of DPB causes increased frequency of [[Adult T-cell leukemia]] in human lymphotropic virus sufferers.<ref name=htlv/>
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| == Treatment ==
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| [[Macrolide]] [[antibiotic]]s, such as [[erythromycin]], [[clarithromycin]] and [[roxithromycin]], have proven to be an effective long-term treatment for DPB.<ref name=mac>{{cite journal |author=Keicho N, Kudoh S |title=Diffuse panbronchiolitis: role of macrolides in therapy |journal=Am J Respir Med. |volume=1 |issue=2 |pages=119-131 |year=2002 |pmid=14720066 }}</ref><ref name=mac08>{{cite journal |author=Lopez-Boado YS, Rubin BK |title=Macrolides as immunomodulatory medications for the therapy of chronic lung diseases |journal=Curr Opin Pharmacol. |volume=Epub ahead of print |issue= |pages= |year=2008 |pmid=18339582 }}</ref> The successful results of macrolides in DPB and similar lung diseases stems from controlling symptoms through [[immunomodulation]] (adjusting the immune response),<ref name=mac08/> with the added benefit of [[dosing|low-dose]] requirements.<ref name=mac/>
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| With macrolide therapy in DPB, great reduction in bronchiolar inflammation and damage is achieved through suppression of not only neutrophil granulocyte proliferation, but also lymphocyte activity and obstructive [[mucus]] and sputum [[secretion]]s in airways.<ref name=mac/> The antimicrobial and antibiotic effects of macrolides, however, are not believed to be involved in their beneficial effects toward treating DPB.<ref name=mac04>{{cite journal |author=Schultz MJ |title=Macrolide activities beyond their antimicrobial effects: macrolides in diffuse panbronchiolitis and cystic fibrosis |journal=J Antimicrob Chemother. |volume=54 |issue=1 |pages=21-28 |year=2004 |pmid=15190022 }}</ref> This is evident, as the treatment dosage is much too low to fight infection, and in DPB cases with the occurrence of macrolide-resistant ''pseudomonas aeruginosa'', macrolide therapy still produces substantial anti-inflammatory results.<ref name=mac/>
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| Advanced cases of DPB, where severely excessive sputum production resistant to macrolides persists, additional therapy with the inhalant [[tiotropium]] has been shown to ease these symptoms and the related shortness of breath.<ref name=tio>{{cite journal |author=Saito Y, Azuma A, Morimoto T, Fujita K, Abe S, Motegi T, Usuki J, Kudoh S |title=Tiotropium ameliorates symptoms in patients with chronic airway mucus hypersecretion which is resistant to macrolide therapy |journal=Intern Med. |volume=47 |issue=7 |pages=585-591 |year=2008 |pmid=18379141 }}</ref>
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| == See also == | |
| *[[Pulmonology]] | |
| *[[Bronchitis]] | | *[[Bronchitis]] |
| *[[Asthma]]
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| *[[Emphysema]] | | *[[Emphysema]] |
| | *[[Pulmonology]] |
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| == References ==
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| {{reflist|2}}
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| {{Respiratory pathology}} | | {{Respiratory pathology}} |
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| [[Category:Pulmonology]] | | [[Category:Pulmonology]] |
| [[Category:Rare diseases]] | | [[Category:Rare diseases]] |
| | [[Category:Disease]] |
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