Hamman-Rich syndrome overview: Difference between revisions

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Latest revision as of 02:49, 25 March 2018

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [2]

Overview

Acute interstitial pneumonitis is a rare, and fulminant disease leading to acute respiratory failure and, or death. Acute interstitial pneumonitis is an entity of a group of Idiopathic interstitial lung diseases first described by two pathologists Hamman and Rich. The etiology is unknown (idiopathic). Acute interstitial pneumonitis occurs typically previously healthy individuals in the age group of 50 to 55years with out pre eexisting lung disease. It affects men and women equally. Acute interstitial pneumonitis shows the histopathologic appearance of diffuse alveolar damage. On gross examination, lungs appear firm, heavy and have a dark red or beefy appearance and show irregular areas of consolidation and fibrosis. On microscopic examination, acute interstitial pneumonitis shows bilateral, temporal uniformity of the diffuse alveolar damage, hyaline membrane deposition and extensive fibroblastic and myofibroblastic proliferation. Acute interstitial pneumonitis may be considered as an idiopathic cause of ARDS. Acute interstitial pneumonitis must be differentiated from other diseases that present with respiratory failure and show diffuse alveolar damage on histopathological examination. These include ARDS, acute eosinophilic pneumonitis, Infections, hypersensitivity pneumonitis, connective tissue diseases, and drug-induced lung toxicity. Patients with acute interstitial pneumonitis usually present with flu-like viral illness or upper respiratory tract infection, which progresses very rapidly to acute respiratory failure. Common symptoms include fatigue, headache, myalgia, cough, fever, and dyspnea. The acute onset of symptoms is characteristic of acute interstitial pneumonitis. Physical examination shows tachypnea, tachycardia, crackles, wheezing and signs of hypoxemia. Chest radiograph of patients with Acute interstitial pneumonitis shows bilateral airspace opacifications. Most of the patients with acute interstitial pneumonitis on HRCT will show bilateral ground-glass attenuation, traction bronchiectasis, airspace consolidation, architectural distortion. Bronchioalveolar lavage and surgical lung biopsy can be helpful in diagnosing other diseases that causing diffuse alveolar damage that present same as acute interstitial pneumonitis There is no effective treatment for acute interstitial pneumonitis, Management in general includes supportive therapy and administration of glucocorticosteroids and Immunosuppressive agents. Lung transplantation may be considered as an alternative treatment for patients with acute interstitial pneumonitis if the conventional therapy fails. Prognosis is very poor.

Historical Perspective

In 1935, Hamman and rich first described cases with rapidly progressing pulmonary fibrosis of unknown etiology. After that, the eponym, Hamman-Rich syndrome have been used to describe idiopathic pulmonary fibrosis. In 1975, Liebow came up with classification to distinguish between pulmonary fibrosis and idiopathic interstitial lung diseases. In 1986, Katzenstein coined the term acute interstitial pneumonitis. Further studies helped to differentiate acute interstitial pneumonitis from pulmonary fibrosis.

Classification

According to American Thoracic Society/European Respiratory Society (ATS/ERS) 2002 consensus, Acute interstitial pneumonitis is an entity of a group of Idiopathic interstitial lung diseases. The classification is based on clinical, radiological and histopathologic findings. The classification has been updated by ATS/ERS International multidisciplinary panel recently based on the literature review on idiopathic interstitial lung diseases published between 2000-2011.

Pathophysiology

Acute interstitial pneumonitis shows the histopathologic appearance of diffuse alveolar damage. On gross examination, lungs appear firm, heavy and have a dark red or beefy appearance and show irregular areas of consolidation and fibrosis. On microscopic examination, acute interstitial pneumonitis shows bilateral, temporal uniformity of the diffuse alveolar damage, hyaline membrane deposition and extensive fibroblastic and myofibroblastic proliferation.

Causes

There is no specific etiology (idiopathic), that is responsible for developing acute interstitial pneumonitis.

Differentiating [disease name] from other Diseases

Acute interstitial pneumonitis must be differentiated from other diseases that present with respiratory failure and diffuse infiltrates on chest radiographs. Some of the differentials include ARDS, acute eosinophilic pneumonitis, Infections, hypersensitivity pneumonitis, connective tissue diseases, and drug-induced lung toxicity.

Epidemiology and Demographics

The world wide incidence of acute interstitial pneumonitis is approximately 97 cases per 100,000 individuals. The prevalence of acute interstitial pneumonitis is not known.

Age

Acute interstitial pneumonitis occurs typically previously healthy individuals in the age group of 50 to 55years with out pre-existing lung disease.

Gender

Acute interstitial pneumonitis affects men and women equally.

Race

In general there is no racial predilection to acute interstitial pneumonitis.

Risk Factors

There are no established risk factors associated with acute interstitial pneumonitis.

Natural History, Complications and Prognosis

If left untreated, patients with acute interstitial pneumonitis have high fatality rate and die because of severe respiratory failure. Most of the survivors after initial hospitalization may develop recurrent disease or chronic lung fibrosis. Acute interstitial pneumonitis usually has a very poor prognosis.

Diagnosis

Diagnostic Criteria

Abrupt onset of respiratory symptoms resulting in acute respiratory failure
Chest radiographs show bilateral lung infiltrates
The absence of an identifiable etiology
Absence of predisposing condition
Organising diffuse alveolar damage seen on histopathological examination

Symptoms

Patients with acute interstitial pneumonitis usually present with flu-like viral illness or upper respiratory tract infection, which progresses very rapidly to acute respiratory failure. Common symptoms include fatigue, headache, myalgia, cough, fever, and dyspnea. The acute onset of symptoms is characteristic of acute interstitial pneumonitis.

Physical Examination

Patients with acute interstitial pneumonitis usually appear ill. Physical examination shows tachypnea, tachycardia, crackles, wheezing and signs of hypoxemia.

Laboratory Findings

There are no diagnostic laboratory findings associated with acute interstitial pneumonitis. However, routine laboratory tests may help in identifying alternative diagnoses rather than making a diagnosis of acute interstitial pneumonitis, include abnormal arterial blood gases, physiologic lung testing, complete blood count, and sputum examination, and microbiologic tests.

Imaging Findings

Chest radiograph of patients with Acute interstitial pneumonitis shows bilateral airspace opacifications.
Most of the patients with acute interstitial pneumonitis on HRCT will show bilateral ground-glass attenuation, traction bronchiectasis, airspace consolidation, architectural distortion. This pattern of abnormality is typically found in acute interstitial pneumonitis but it is not diagnostic.

Other Diagnostic Studies

Bronchioalveolar lavage and surgical lung biopsy can be helpful in diagnosing other diseases that causing diffuse alveolar damage that present same as acute interstitial pneumonitis.

Treatment

Medical Therapy

There is no effective treatment for acute interstitial pneumonitis, Management in general includes supportive therapy and administration of glucocorticosteroids and Immunosuppressive agents

Surgery

Lung transplantation may be considered as an alternative treatment for patients with acute interstitial pneumonitis if the conventional therapy fails.

Prevention

There are no sufficient guidelines for the primary prevention of acute interstitial pneumonitis. However, preventing general triggering agents that leads to fibrotic changes in lungs including smoking cessation and vaccination against influenza may be helpful in preventing pulmonary fibrosis and other idiopathic fibrotic lung conditions

References

@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Hamman-Rich syndrome }}
-{{CMG}}
+{{CMG}}; {{AE}} {{CK}}
 ==Overview==
-'''Hamman-Rich syndrome''' is a rare, severe lung disease which usually affects otherwise healthy individuals. HR syndrome is often categorized as both an [[interstitial lung disease]] and a form of [[acute respiratory distress syndrome]] (ARDS).
+[[Hamman-Rich syndrome|'''Acute interstitial pneumonitis''']]  is a rare, and  fulminant disease  leading to [[Respiratory failure|acute respiratory failure]] and, or death.  [[Hamman-Rich syndrome|Acute interstitial pneumonitis]] is an entity of a group of Idiopathic interstitial lung diseases first described by two pathologists Hamman and Rich. The [[etiology]] is unknown [[Idiopathic|(idiopathic]]). [[Hamman-Rich syndrome|Acute interstitial pneumonitis]] occurs typically previously healthy individuals in the [[age]] group of 50 to 55years with out pre eexisting lung disease. It affects men and women equally.  [[Hamman-Rich syndrome|Acute interstitial pneumonitis]] shows the [[Histopathology|histopathologic appearance]] of diffuse alveolar damage. On [[gross examination]], [[Lung|lungs]] appear firm, heavy and have a dark red or beefy appearance and show irregular areas of [[Consolidation (medicine)|consolidation]] and fibrosis. On [[Microscopy|microscopic examination]], [[Hamman-Rich syndrome|acute interstitial pneumonitis]] shows [[bilateral]], temporal uniformity of the diffuse alveolar damage, hyaline membrane deposition and extensive [[Fibroblast|fibroblastic]] and [[Myofibroblast|myofibroblastic]] proliferation. [[Hamman-Rich syndrome|Acute interstitial pneumonitis]] may be considered as an [[idiopathic]] cause of [[Acute respiratory distress syndrome|ARDS]]. [[Hamman-Rich syndrome|Acute interstitial pneumonitis]] must be differentiated from other diseases that present with [[respiratory failure]] and show diffuse alveolar damage on [[Histopathology|histopathological]] examination. These include  [[Acute respiratory distress syndrome|ARDS,]] [[Eosinophilic pneumonitis|acute eosinophilic pneumonitis]], [[Infection|Infections]], [[hypersensitivity pneumonitis]], [[Connective tissue disease|connective tissue diseases]], and drug-induced lung toxicity. Patients with [[Hamman-Rich syndrome|acute interstitial pneumonitis]] usually present with [[Flu|flu-like viral illness]] or [[upper respiratory tract infection]], which progresses very rapidly to acute [[respiratory failure]]. Common symptoms include [[fatigue]], [[headache]], [[myalgia]], [[cough]], [[fever]], and [[dyspnea]]. The acute onset of symptoms is characteristic of [[Hamman-Rich syndrome|acute interstitial pneumonitis]]. Physical examination shows [[tachypnea]], [[tachycardia]], [[Rales|crackles]], [[Wheeze|wheezing]] and signs of [[hypoxemia]]. [[Chest X-ray|Chest radiograph]] of patients with [[Hamman-Rich syndrome|Acute interstitial pneumonitis]] shows bilateral airspace opacifications. Most of the patients with [[Hamman-Rich syndrome|acute interstitial pneumonitis]] on [[High Resolution CT|HRCT]] will show bilateral ground-glass [[attenuation]], traction bronchiectasis, airspace [[Consolidation (medicine)|consolidation]], architectural distortion. Bronchioalveolar lavage and surgical lung [[biopsy]] can be helpful in diagnosing other diseases that causing diffuse alveolar damage that present same as [[Hamman-Rich syndrome|acute interstitial pneumonitis]] There is no effective treatment for [[Hamman-Rich syndrome|acute interstitial pneumonitis]], Management in general includes supportive therapy and administration of glucocorticosteroids and Immunosuppressive agents. [[Lung transplantation]] may be considered as an alternative treatment for patients with [[Hamman-Rich syndrome|acute interstitial pneumonitis]] if the conventional therapy fails. [[Prognosis]] is very poor.
 ==Historical Perspective==
-*[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
+In 1935, Hamman and rich first described cases with rapidly progressing pulmonary fibrosis of unknown [[etiology]]. After that, the eponym, '''Hamman-Rich syndrome''' have been used to describe [[idiopathic pulmonary fibrosis]]. In 1975, Liebow came up with [[classification]] to distinguish between pulmonary fibrosis and idiopathic interstitial lung diseases. In 1986, Katzenstein coined the term [[Hamman-Rich syndrome|acute interstitial pneumonitis]]. Further studies helped to differentiate [[Hamman-Rich syndrome|acute interstitial pneumonitis]] from pulmonary [[fibrosis]].
-*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:
+According to American Thoracic Society/European Respiratory Society (ATS/ERS) 2002 consensus, [[Hamman-Rich syndrome|Acute interstitial pneumonitis]] is an entity of a group of Idiopathic interstitial lung diseases. The classification is based on [[clinical]], [[Radiology|radiological]] and [[Histopathology|histopathologic]] findings. The classification has been updated by ATS/ERS International multidisciplinary panel recently based on the [[literature review]] on idiopathic interstitial lung diseases published between 2000-2011.
-:*[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].
+[[Hamman-Rich syndrome|Acute interstitial pneumonitis]] shows the [[Histopathology|histopathologic appearance]] of diffuse alveolar damage. On [[gross examination]], [[Lung|lungs]] appear firm, heavy and have a dark red or beefy appearance and show irregular areas of [[Consolidation (medicine)|consolidation]] and fibrosis. On [[Microscopy|microscopic examination]], [[Hamman-Rich syndrome|acute interstitial pneumonitis]] shows [[bilateral]], temporal uniformity of the diffuse alveolar damage, hyaline membrane deposition and extensive [[Fibroblast|fibroblastic]] and [[Myofibroblast|myofibroblastic]] proliferation.
-*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].
+There is no specific [[etiology]] ([[idiopathic]]), that is responsible for developing [[Hamman-Rich syndrome|acute interstitial pneumonitis]].
-* [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:
+[[Hamman-Rich syndrome|Acute interstitial pneumonitis]] must be differentiated from other diseases that present with [[respiratory failure]] and diffuse infiltrates on [[Chest X-ray|chest radiographs]]. Some of the differentials include [[Acute respiratory distress syndrome|ARDS]], [[Eosinophilic pneumonitis|acute eosinophilic pneumonitis]], [[Infection|Infections]], [[hypersensitivity pneumonitis]], [[Connective tissue disease|connective tissue diseases]], and drug-induced lung toxicity.
-:*[Differential dx1]
-:*[Differential dx2]
-:*[Differential dx3]
 ==Epidemiology and Demographics==
-* The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
+* The world wide [[incidence]] of [[Hamman-Rich syndrome|acute interstitial pneumonitis]] is approximately 97 cases per 100,000 individuals. The [[prevalence]] of [[Hamman-Rich syndrome|acute interstitial pneumonitis]] is not known.
-* 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].
+*[[Hamman-Rich syndrome|Acute interstitial pneumonitis]] occurs typically previously healthy individuals in the [[age]] group of 50 to 55years with out pre-existing lung disease.
-*[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.
+* [[Hamman-Rich syndrome|Acute interstitial pneumonitis]] 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].
+* In general there is no [[Race|racial]] predilection to [[Hamman-Rich syndrome|acute interstitial pneumonitis]].
-*[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].
+*There are no established [[Risk factor|risk factors]] associated with [[Hamman-Rich syndrome|acute interstitial pneumonitis]].
 == Natural History, Complications and Prognosis==
-*The majority of patients with [disease name] remain asymptomatic for [duration/years].
+If left untreated, patients with [[Hamman-Rich syndrome|acute interstitial pneumonitis]] have high [[fatality rate]] and die because of severe [[respiratory failure]]. Most of the survivors after initial hospitalization may develop recurrent disease or chronic lung fibrosis. [[Hamman-Rich syndrome|Acute interstitial pneumonitis]] usually has a very poor [[prognosis]].
-*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:
+*Abrupt onset of respiratory [[Symptom|symptoms]] resulting in [[Respiratory failure|acute respiratory failure]]
-:*[criterion 1]
+*[[Chest X-ray|Chest radiographs]] show bilateral [[lung]] infiltrates
-:*[criterion 2]
+*The absence of an identifiable [[etiology]]
-:*[criterion 3]
+*Absence of predisposing condition
-:*[criterion 4]
+*Organising diffuse alveolar damage seen on [[Histopathology|histopathological examination]]
 === Symptoms ===
-*[Disease name] is usually asymptomatic.
+*Patients with [[Hamman-Rich syndrome|acute interstitial pneumonitis]] usually present with [[Flu|flu-like viral illness]] or [[upper respiratory tract infection]], which progresses very rapidly to acute [[respiratory failure]]. Common symptoms include [[fatigue]], [[headache]], [[myalgia]], [[cough]], [[fever]], and [[dyspnea]]. The acute onset of symptoms is characteristic of [[Hamman-Rich syndrome|acute interstitial pneumonitis]].
-*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].
+* Patients with [[Hamman-Rich syndrome|acute interstitial pneumonitis]] usually appear ill. Physical examination shows [[tachypnea]], [[tachycardia]], [[Rales|crackles]], [[Wheeze|wheezing]] and signs of [[hypoxemia]].
-*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].
+*There are no diagnostic laboratory findings associated with acute interstitial pneumonitis. However, routine laboratory tests may help in identifying alternative diagnoses rather than making a diagnosis of acute interstitial pneumonitis, include abnormal [[Arterial blood gas|arterial blood gases]], physiologic lung testing, [[complete blood count]], and [[Sputum|sputum examination]], and microbiologic tests.
+===Imaging Findings===
+*[[Chest X-ray|Chest radiograph]] of patients with [[Hamman-Rich syndrome|Acute interstitial pneumonitis]] shows bilateral airspace opacifications.
+*Most of the patients with [[Hamman-Rich syndrome|acute interstitial pneumonitis]] on [[High Resolution CT|HRCT]] will show bilateral ground-glass [[attenuation]], traction bronchiectasis, airspace [[Consolidation (medicine)|consolidation]], architectural distortion. This pattern of abnormality is typically found in [[Hamman-Rich syndrome|acute interstitial pneumonitis]] but it is not diagnostic.
-*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].
+*Bronchioalveolar lavage and surgical lung [[biopsy]] can be helpful in diagnosing other diseases that causing diffuse alveolar damage that present same as [[Hamman-Rich syndrome|acute interstitial pneumonitis]].
-*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.
+*There is no effective treatment for [[Hamman-Rich syndrome|acute interstitial pneumonitis]], Management in general includes supportive therapy and administration of glucocorticosteroids and Immunosuppressive agents
-*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].
+* [[Lung transplantation]] may be considered as an alternative treatment for patients with [[Hamman-Rich syndrome|acute interstitial pneumonitis]] if the conventional therapy fails.
-*[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].
+*There are no sufficient guidelines for the primary prevention of [[Hamman-Rich syndrome|acute interstitial pneumonitis]]. However, preventing general triggering agents that leads to fibrotic changes in [[Lung|lungs]] including [[smoking cessation]] and [[vaccination]] against [[influenza]] may be helpful in preventing [[Idiopathic pulmonary fibrosis|pulmonary fibrosis]] and other idiopathic fibrotic [[lung]] conditions
-*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==
-{{reflist|2}}
-{{WH}}
-{{WS}}
-[[Category:Pulmonology]]
+[[Category: Pulmonology]]