Interstitial Lung Disease and IPF Flashcards

1
Q

interstitial lung diseases

A

-Diverse group of disorders that involve the distal pulmonary parenchyma (as opposed to the airways)
-Etiology:
-Idiopathic
-Systemic diseases (connective tissue disorders)
-Toxic, radiologic, environmental, occupational exposures

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2
Q

interstitial lung disease are a diverse group of conditions that may result in lung fibrosis

A

-idiopathic ILDs
-hypersensitivity pneumonitis- inflammatory
-connective tissue disease- ILDs- associated with rheumatologic disease
-sarcoidosis
-other ILDs

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3
Q

interstitial lung diseases: approach to treatment

A

-Is There an Identifiable Cause or is it Idiopathic? -> If There is an Identifiable Cause, It Should be Specifically Addressed
-Is it Primarily Inflammatory or Fibrotic? ->
-Inflammatory ILD’s are treated with Anti-Inflammatory and Immunosuppressive Medications -> reversible
-Fibrotic ILD’s are treated with Anti-Fibrotic Medications -> scarring that isnt reversible
**Inflammatory ILD’s May Develop Progressive Fibrosis Over Time
-Any ILD With Evidence of Progressive Fibrosis May Be Treated with Anti-Fibrotic Medicationc

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4
Q

challenges to dx interstitial lung diseases

A

-identifying
-Signs and symptoms are very non-specific (dyspnea and cough)
-More than half of patients will be initially misdiagnosed with more common problems such as COPD and heart disease
-Similar symptoms
-Similar demographic
-75% will consult 3 or more physicians
-34% will have a delay in diagnosis of over 2 years
-Accurate Diagnosis depends on High Resolution CT scan:
-Most CT’s done are not the right type of CT (Low dose for nodules, or CTA for thromboembolic disease)
-you need a high resolution CT
-Most community radiologists are not dedicated Chest Radiologists
-Most radiologists will not give a definitive ILD diagnosis

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5
Q

when should I suspect ILD

A

*“fIeLD-> FENCES X”

-family hx of ILD
-exertional dyspnea- SOB with exertion
-non productive cough (COPD is productive usually)
- crackles (especially at bases) -> no crackles with COPD
- -exertional desaturation-
- -spirometry (low FVC) or low DLCO
-X-ray: abnormal chest xray*

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6
Q

ILD pathophysiology

A

-Any process that results in inflammatory-fibrotic infiltration of the alveolar septa resulting in effects on the capillary endothelium and alveolar epithelium
-does not affect the airway!
-Generic term used to describe many conditions that cause breathlessness and/or cough and are associated with radiographic bilateral lung abnormalities
-interstitium- between the capillary and capillary -> with ILD the interstitium is thickened -> lengthens the space for diffusion
-fibrosis- deposition outside alveoli -> thickens

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7
Q

current definition of idiopathic pulmonary fibrosis

A

-progressive chronic fibrosing interstitial pneumonia
-Unknown cause
-Limited to the lungs
-Has typical HRCT findings
-Associated with a histologic pattern similar to UIP
-fibrotic disease - little inflammation

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8
Q

IPF typical presentation

A

-middle ages 50-70s
-new onset of progressive exertional dyspnea and non-productive cough
-most have symptoms for 12-18 months prior to definitive evaluation
-systemic symptoms are uncommon
-dry crackles MC physical finding

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9
Q

IPF: Hx + PE + Labs

A

-Comprehensive medical history (social history, occupational and environmental exposures [including pets and birds], drug exposures, and family history) -> see if you can find the cause
-PFTs –FVC, DLco, TLC
-6 minute walk test- see if pt desaturates and get a baseline to monitor progression
-Chest Auscultation
-Serologic Testing
-Radiologic Assessment:
-Chest X-Ray
-High Resolution Chest CT*
-Surgical Assessment- Surgical Lung Biopsy (if Necessary)- now this is rarely done

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10
Q

physical exam IPF

A

-bibasilar late inspiratory fine crackles (velcro rales)
-tachypnea
-cyanosis
-progressed ds:
-clubbing- 40-70% late in disease course
-cardiac exam usually normal until middle late stages -> augmented P2, right sided heave, S3 gallop

-rash, arthritis, myositis should suggest an alternate dx

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11
Q

look, listen, and walk

A

-Look at the skin and fingers
Listen carefully for crackles
-Both Lung Bases
-Laterally in mid-axillary line
-Walk the patient in the hallway (or in a staircase):
-Try to elicit exertional dyspnea if possible
-Measure SpO2 before and after walking
-A 3% drop in SpO2 indicates exertional desaturation
-Walk the Patient Again with oxygen titration -> Determine O2 flow that prevents desaturation below 88%

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12
Q

pulmonary function tests

A

-Spirometry:
-looking for restrictive pattern
-Reduced FVC and TLC
-Normal or increased FEV1/FVC ratio
-Restriction on Lung Volumes
-Impaired gas exchange:
-Decreased DLCO, PaO2
-Desaturation on exercise oximetry
-Increased A-aPO2 gradient
-Normal PFTs do not exclude early ILD
-flow volume loop- restrictive pattern -> normal shape but smaller

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13
Q

serologic evaluation

A

-Minimum: ANA,CCP, RF (rheumatoid factor), (ATS/ERS guidelines)*
-Based on history & physical exam, consider (dont memorize everything):
-ENA) autoantibody panel: connective ds
-Anti-centromere antibody
-ESR & CRP: inflammation
-ANCA antibodies
-Anti-cardiolipin antibodies, lupus anticoagulant
-Creatine kinase, aldolase: muscular involvement
-Hypersensitivity pneumonitis panel
-Should be performed before a bx ***

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14
Q

inflammatory disease

A

diffuse

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15
Q

reticulation

A

-early signs of fibrosis
-reticulation results from thickening of the inter-or intralobular septa
- appears as linear opacities that resemble a mesh or net on CT

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16
Q

honeycombing

A

-advanced stage fibrosis
-results from deposition of collagen that destroys the characteristic alveolar structure and represents end stage lung disease
- clustered cystic air spaces with well-defined walls
-destruction of normal architecture

17
Q

ground glass opacity

A

-Indicative of inflammation*
-Hazy areas of increased attenuation with preserved anatomy, including bronchial and vascular markings
-tend to more diffuse

18
Q

Usual interstitial pneumonia (UIP): hallmark radiologic pattern of IPF

A

-subpleural, basal predominant
-reticular abnormalities
-honeycombing with or without traction bronchiectasis or bronchiolectasis
-traction bronchiectasis: bronchial dilation
-common at the bases and edgestitches **
-common not to see inflammation-> no ground glass

19
Q

UIP is indicative of IPF

A

-hallmark criteria of UIP
-subpleural, basal predominance
-reticular abnormality
-honeycombing with or without traction bronchiectasis
-absence of features listed as inconsistent with UIP pattern

20
Q

advanced IPF HRCT

A

-Prone HRCT near the lung bases shows extensive reticulation, traction bronchiectasis, and gross honeycombing with a subpleural/basilar predominance
-This appearance indicates advanced IPF

21
Q

pathologic evaluation: IPF

A

-UIP/IPF pattern on HRCT:
-Fibroblastic foci **: active sites of fibrosis
-Temporal heterogeneity:areas of lung with different stages of disease
-Honeycombing appearance
-little to no inflammation
-you should be able to tell everything from the CT

-Surgical lung bx (not really helpful):*- only indicated if atypical clinical and radiographic findings
-Transbronchial biopsy not useful for dx -> Can be used to exclude other dx

22
Q

predictors of disease severity and progression in IPF

A

-Pts with compromised lung function or respiratory events have worse outcomes
-worser outcomes/correlates with mortality:
-low DLCO <35% -> lower survival
-pulmonary hypertension
- FVC change over time
-O2 sat < 85% with walk test
-distance walk
- HR recovery low
-hospitalization
-dyspnea score

23
Q

known therapy as of 2023

A

-Nintedanib (Ofev) + Pirfenidone (Esbriet): oral anti-fibrotic agent
-these drugs have signification GI symptoms -> dont cure but they slow progression
-treating symptoms:
-Oxygen
-Pulmonary rehabilitation
-Treat GERD + cardiac dysfunction
-Bronchodilators for cough
-Optimize nutrition
-Immunizations
-Lung Transplant

24
Q

acute exacerbation of IPF + tx

A

-Definition acute exacerbation:
-Diagnosis of IPF
-Unexplained development of worsening of dyspnea within 30 days
-HRCT with new ground-glass abnormalities
-No evidence of pulmonary infection by ET aspirate or BAL
-Exclusion of alternative causes, e.g. HF, PE
-tx- broad spectrum antibiotics
-high dose steroids (prednisone 1mg/kg)

25
Q

why do pts die from IPF

A

-Acute exacerbations ***
-Cor pulmonale- right heart failure
-Pulmonary infection
-Cardiovascular Disease
-Pulmonary Hypertension
-Lung Cancer

26
Q

ILD summary

A

-Diverse group of parenchymal lung diseases
-Idiopathic or Identifiable cause
-Inflammatory or Fibrotic
-Signs and Symptoms are non-specific
-Keep ILD in mind if the patient has a different diagnosis, but is not responding to treatment.
-LISTEN FOR CRACKLES
-Its ok to ask the radiologist to take another look. Pattern is important.
-Early dx and tx may lead to better prognosis