Diffuse panbronchiolitis overview

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.

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[dpb-1] 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.

[dpb06-2] 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.

[hla98-3] 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.

[hla99-4] 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.

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