Diffuse panbronchiolitis overview: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Diffuse panbronchiolitis}} | {{Diffuse panbronchiolitis}} | ||
{{CMG}} {{AE}} | |||
{{CMG}} | |||
==Overview== | ==Overview== | ||
'''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>{{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> | '''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>{{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> | ||
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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>{{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> | If left untreated, DPB is fatal, usually progressing to [[bronchiectasis]], an irreversible lung conditon that causes [[respiratory failure]].<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> | ||
==Historical Perspective== | |||
*[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event]. | |||
*In [year], [gene] mutations were first identified in the pathogenesis of [disease name]. | |||
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name]. | |||
==Classification== | |||
*[Disease name] may be classified according to [classification method] into [number] subtypes/groups: | |||
:*[group1] | |||
:*[group2] | |||
:*[group3] | |||
*Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3]. | |||
==Pathophysiology== | |||
*The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3]. | |||
*The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway. | |||
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | |||
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | |||
==Causes== | |||
* [Disease name] may be caused by either [cause1], [cause2], or [cause3]. | |||
* [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s]. | |||
* There are no established causes for [disease name]. | |||
==Differentiating [disease name] from other Diseases== | |||
*[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as: | |||
:*[Differential dx1] | |||
:*[Differential dx2] | |||
:*[Differential dx3] | |||
==Epidemiology and Demographics== | |||
* The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide. | |||
* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location]. | |||
===Age=== | |||
*Patients of all age groups may develop [disease name]. | |||
*[Disease name] is more commonly observed among patients aged [age range] years old. | |||
*[Disease name] is more commonly observed among [elderly patients/young patients/children]. | |||
===Gender=== | |||
*[Disease name] affects men and women equally. | |||
*[Gender 1] are more commonly affected with [disease name] than [gender 2]. | |||
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1. | |||
===Race=== | |||
*There is no racial predilection for [disease name]. | |||
*[Disease name] usually affects individuals of the [race 1] race. | |||
*[Race 2] individuals are less likely to develop [disease name]. | |||
==Risk Factors== | |||
*Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4]. | |||
== Natural History, Complications and Prognosis== | |||
*The majority of patients with [disease name] remain asymptomatic for [duration/years]. | |||
*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3]. | |||
*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3]. | |||
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3]. | |||
*Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%]. | |||
== Diagnosis == | |||
===Diagnostic Criteria=== | |||
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: | |||
:*[criterion 1] | |||
:*[criterion 2] | |||
:*[criterion 3] | |||
:*[criterion 4] | |||
=== Symptoms === | |||
*[Disease name] is usually asymptomatic. | |||
*Symptoms of [disease name] may include the following: | |||
:*[symptom 1] | |||
:*[symptom 2] | |||
:*[symptom 3] | |||
:*[symptom 4] | |||
:*[symptom 5] | |||
:*[symptom 6] | |||
=== Physical Examination === | |||
*Patients with [disease name] usually appear [general appearance]. | |||
*Physical examination may be remarkable for: | |||
:*[finding 1] | |||
:*[finding 2] | |||
:*[finding 3] | |||
:*[finding 4] | |||
:*[finding 5] | |||
:*[finding 6] | |||
=== Laboratory Findings === | |||
*There are no specific laboratory findings associated with [disease name]. | |||
*A [positive/negative] [test name] is diagnostic of [disease name]. | |||
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name]. | |||
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3]. | |||
===Imaging Findings=== | |||
*There are no [imaging study] findings associated with [disease name]. | |||
*[Imaging study 1] is the imaging modality of choice for [disease name]. | |||
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3]. | |||
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3]. | |||
=== Other Diagnostic Studies === | |||
*[Disease name] may also be diagnosed using [diagnostic study name]. | |||
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3]. | |||
== Treatment == | |||
=== Medical Therapy === | |||
*There is no treatment for [disease name]; the mainstay of therapy is supportive care. | |||
*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2]. | |||
*[Medical therapy 1] acts by [mechanism of action 1]. | |||
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration]. | |||
=== Surgery === | |||
*Surgery is the mainstay of therapy for [disease name]. | |||
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name]. | |||
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name]. | |||
=== Prevention === | |||
*There are no primary preventive measures available for [disease name]. | |||
*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3]. | |||
*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3]. | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category:Pulmonology]] | [[Category:Pulmonology]] | ||
{{WH}} | |||
{{WS}} | |||
Latest revision as of 13:38, 6 June 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
Overview
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]
Historical Perspective
- [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
- In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
- In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
Classification
- [Disease name] may be classified according to [classification method] into [number] subtypes/groups:
- [group1]
- [group2]
- [group3]
- Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
Pathophysiology
- The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
- The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
- On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
- On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Causes
- [Disease name] may be caused by either [cause1], [cause2], or [cause3].
- [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
- There are no established causes for [disease name].
Differentiating [disease name] from other Diseases
- [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
- [Differential dx1]
- [Differential dx2]
- [Differential dx3]
Epidemiology and Demographics
- The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
- In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
Age
- Patients of all age groups may develop [disease name].
- [Disease name] is more commonly observed among patients aged [age range] years old.
- [Disease name] is more commonly observed among [elderly patients/young patients/children].
Gender
- [Disease name] affects men and women equally.
- [Gender 1] are more commonly affected with [disease name] than [gender 2].
- The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
Race
- There is no racial predilection for [disease name].
- [Disease name] usually affects individuals of the [race 1] race.
- [Race 2] individuals are less likely to develop [disease name].
Risk Factors
- Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
Natural History, Complications and Prognosis
- The majority of patients with [disease name] remain asymptomatic for [duration/years].
- Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
- If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
- Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
- Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].
Diagnosis
Diagnostic Criteria
- The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
- [criterion 1]
- [criterion 2]
- [criterion 3]
- [criterion 4]
Symptoms
- [Disease name] is usually asymptomatic.
- Symptoms of [disease name] may include the following:
- [symptom 1]
- [symptom 2]
- [symptom 3]
- [symptom 4]
- [symptom 5]
- [symptom 6]
Physical Examination
- Patients with [disease name] usually appear [general appearance].
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
- [finding 5]
- [finding 6]
Laboratory Findings
- There are no specific laboratory findings associated with [disease name].
- A [positive/negative] [test name] is diagnostic of [disease name].
- An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
- Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
Imaging Findings
- There are no [imaging study] findings associated with [disease name].
- [Imaging study 1] is the imaging modality of choice for [disease name].
- On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
- [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
- [Disease name] may also be diagnosed using [diagnostic study name].
- Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
Treatment
Medical Therapy
- There is no treatment for [disease name]; the mainstay of therapy is supportive care.
- The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
- [Medical therapy 1] acts by [mechanism of action 1].
- Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
Surgery
- Surgery is the mainstay of therapy for [disease name].
- [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
- [Surgical procedure] can only be performed for patients with [disease stage] [disease name].
Prevention
- There are no primary preventive measures available for [disease name].
- Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
- Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
References
- ↑ Homma H, Yamanaka A, Tanimoto S, Tamura M, Chijimatsu Y, Kira S, Izumi T (1983). "Diffuse panbronchiolitis. A disease of the transitional zone of the lung". Chest. 83 (1): 63–69. PMID 6848335.
- ↑ 2.0 2.1 2.2 2.3 Poletti V, Casoni G, Chilosi M, Zompatori M (2006). "Diffuse panbronchiolitis". Eur Respir J. 28 (4): 862–871. PMID 17012632.
- ↑ Keicho N, Tokunaga K, Nakata K, Taguchi Y, Azuma A, Bannai M, Emi M, Ohishi N, Yazaki Y, Kudoh S (1998). "Contribution of HLA genes in genetic predisposition for diffuse panbronchiolitis". Am J Respir Crit Care Med. 158 (3): 846–850. PMID 9731015.
- ↑ Park MH, Kim YW, Yoon HI, Yoo CD, Han SK, Shim YS, Kim WD (1999). "Association of HLA class I antigens with diffuse panbronchiolitis in Korean patients". Am J Respir Crit Care Med. 159 (2): 526–529. PMID 9927368.