Respiratory bronchiolitis-interstitial lung disease

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List of terms related to Respiratory bronchiolitis-interstitial lung disease

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Respiratory bronchiolitis-associated interstitial lung disease; RB-ILD; RBILD; RB-AILD; RBAILD

Overview

Respiratory bronchiolitis interstitial lung disease refers to a form of idiopathic interstitial pneumonia associated with smoking.^[1]

It is a histological finding, not a pathological description. When associated with disease, it is known as "Respiratory bronchiolitis-associated interstitial lung disease" or "RB-ILD".^[2]

The appearance is similar to desquamative interstitial pneumonia, and some have suggested that the two conditions are caused by the same processes.^[3]

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

↑ "Idiopathic Interstitial Pneumonias: Interstitial Lung Diseases: Merck Manual Professional". Retrieved 2008-12-09.
↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. p. 741. ISBN 0-7216-0187-1.
↑ Heyneman LE, Ward S, Lynch DA, Remy-Jardin M, Johkoh T, Müller NL (1999). "Respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, and desquamative interstitial pneumonia: different entities or part of the spectrum of the same disease process?". AJR Am J Roentgenol. 173 (6): 1617–22. doi:10.2214/ajr.173.6.10584810. PMID 10584810. Unknown parameter |month= ignored (help)

Template:WH Template:WS

[urlIdiopathic_Interstitial_Pneumonias:_Interstitial_Lung_Diseases:_Merck_Manual_Professional-1] "Idiopathic Interstitial Pneumonias: Interstitial Lung Diseases: Merck Manual Professional". Retrieved 2008-12-09.

[isbn0-7216-0187-1-2] Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. p. 741. ISBN 0-7216-0187-1.

[pmid10584810-3] Heyneman LE, Ward S, Lynch DA, Remy-Jardin M, Johkoh T, Müller NL (1999). "Respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, and desquamative interstitial pneumonia: different entities or part of the spectrum of the same disease process?". AJR Am J Roentgenol. 173 (6): 1617–22. doi:10.2214/ajr.173.6.10584810. PMID 10584810. Unknown parameter |month= ignored (help)

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