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==Background==
==Background==
* General group of diseases that involve the lung parenchyma
* General group of diseases that involve the scarring of lung parenchyma
* Grouped together because of similar clinical, physiologic, radiographic, or pathologic findings  
* Grouped together because of similar clinical, physiologic, radiographic, or pathologic findings  
* Specific diseases separated into ones with known causes and idiopathic  
* Specific diseases separated into ones with known causes and idiopathic  
* Treatment and prognosis differs between specific diseases
* Treatment and prognosis differs between specific diseases
* Examples of specific diseases: interstitial pneumonia, hypersensitivity pneumonitis, cryptogenic organizing pneumonia, acute interstitial pneumonitis, sarcoidosis, idiopathic pulmonary fibrosis  
* Examples of specific diseases:
**Interstitial pneumonia
**Hypersensitivity pneumonitis
**Cryptogenic organizing pneumonia
**Acute interstitial pneumonitis
**[[Sarcoidosis]]
**[[Idiopathic pulmonary fibrosis]]


==Clinical Features==
==Clinical Features==
* Symptoms: progressive exertional dyspnea, persistent nonproductive cough, hemoptysis, pleuritic chest pain
* Symptoms
* History of occupational exposure: asbestosis, silicosis
**Progressive exertional [[dyspnea]]
* Abnormal chest imaging or pulmonary function tests
**Persistent, nonproductive [[cough]]
* Take detailed history making sure to focus on past medical history (connective tissue disease, inflammatory bowel disease, malignancy), smoking history, family history, medications, occupational and environmental exposures
*+/- [[hemoptysis]], pleuritic [[chest pain]]
* Physical exam is generally nonspecific
* May have history of smoking, occupational exposures (asbestosis, silicosis, grain dust, bird droppings), medications that can cause pulmonary damage, connective tissue disease, IBD
*Physical exam
**Usually nonspecific
**Increased work of breathing, [[hypoxia]]
**+/- Abnormal lung sounds (classically fine, "velcro-like" crackles)


==Differential Diagnosis==
==Differential Diagnosis==
* Pneumonia
 
* Asbestosis
{{Pulmonary fibrosis differential}}
* Berylliosis
* Pulmonary edema
* Coal worker's pneumoconiosis
* Cryptogenic organizing pneumonia
* Drug induced pulmonary toxicity
* Farmer's lung
* Hypersensitivity pneumonitis
* Interstitial pulmonary fibrosis  
* Collagen-vascular disease
* Lung malignancy
* Restrictive lung disease
* Sarcoidosis
* Silicosis


{{SOB DDX}}
{{SOB DDX}}


==Evaluation==
==Evaluation==
''Work up and test results differ between types of interstitial lung diseases. Below are common studies obtained to determine disease. See page for specific disease for specific labs/imaging and findings. ''
*Definitive diagnosis of ILD rarely made in the ED
* CBC/CMP
*ED workup should focus on assessing for reversible pathologies that may be causing or exacerbating symptoms (e.g. infection, [[PE]], [[pulmonary hypertension]], [[pulmonary edema]], concomitant [[COPD]] exacerbation, etc.)
* Serologic studies: ANA, Rheumatoid factor
**VBG/ABG
* Imaging: Chest x-ray, chest CT
**CBC, BMP
* Pulmonary function tests
**[[CXR]]
* Cardiac ECHO
**Consider CT chest (to rule out PE or better characterize lung parenchyma)
* Bronchoalveolar lavage
**+/- Infectious workup, echo as appropriate
* Lung biopsy
*Inpatient/outpatient workup may include serologic studies (ANA, rheumatoid factor), PFTs, BAL, lung biopsy


==Management==
==Management==
* Management differs between specific types of interstitial lung diseases. This is why it is important to determine specific cause of disease. See specific disease page for management.
*Treat underlying/exacerbating factors (e.g. antimicrobials for infection, optimize volume/hemodynamics for pulmonary hypertension patients, bronchodilators for concomitant obstructive lung disease)
*Support [[oxygen therapy|oxygenation]] and [[ventilation]]
*Specific disease treatments vary, but many respond to [[steroids]], sometimes at high doses
**See management section of [[idiopathic pulmonary fibrosis]]


==Disposition==
==Disposition==
* Depending on how stable the patient is, patient can be admitted for work up or discharged for outpatient work up.
* Low threshold to admit
* Important to determine acute vs chronic symptoms.


==See Also==
==See Also==
* [[Sarcoidosis ]]
* [[Sarcoidosis ]]
* Idiopathic pulmonary fibrosis
* [[Idiopathic pulmonary fibrosis]]
* [[Pneumoconiosis]]
* [[Hypoxia]]
* [[Dyspnea]]


==References==
==References==

Revisión del 23:52 25 ene 2019

Background

  • General group of diseases that involve the scarring of lung parenchyma
  • Grouped together because of similar clinical, physiologic, radiographic, or pathologic findings
  • Specific diseases separated into ones with known causes and idiopathic
  • Treatment and prognosis differs between specific diseases
  • Examples of specific diseases:

Clinical Features

  • Symptoms
    • Progressive exertional dyspnea
    • Persistent, nonproductive cough
  • +/- hemoptysis, pleuritic chest pain
  • May have history of smoking, occupational exposures (asbestosis, silicosis, grain dust, bird droppings), medications that can cause pulmonary damage, connective tissue disease, IBD
  • Physical exam
    • Usually nonspecific
    • Increased work of breathing, hypoxia
    • +/- Abnormal lung sounds (classically fine, "velcro-like" crackles)

Differential Diagnosis

Pulmonary Fibrosis

Acute dyspnea

Emergent

Non-Emergent

Evaluation

  • Definitive diagnosis of ILD rarely made in the ED
  • ED workup should focus on assessing for reversible pathologies that may be causing or exacerbating symptoms (e.g. infection, PE, pulmonary hypertension, pulmonary edema, concomitant COPD exacerbation, etc.)
    • VBG/ABG
    • CBC, BMP
    • CXR
    • Consider CT chest (to rule out PE or better characterize lung parenchyma)
    • +/- Infectious workup, echo as appropriate
  • Inpatient/outpatient workup may include serologic studies (ANA, rheumatoid factor), PFTs, BAL, lung biopsy

Management

  • Treat underlying/exacerbating factors (e.g. antimicrobials for infection, optimize volume/hemodynamics for pulmonary hypertension patients, bronchodilators for concomitant obstructive lung disease)
  • Support oxygenation and ventilation
  • Specific disease treatments vary, but many respond to steroids, sometimes at high doses

Disposition

  • Low threshold to admit

See Also

References