Interstitial Lung Disease - Exam 2 Flashcards
What is lung interstitium?
the region of the alveolar wall exclusive of and separating the basement membranes of alveolar epithelial and pulmonary capillary endothelial cells
What are the 5 parts of the pulmonary interstitial anatomy?
alveolar epithelium
pulmonary capillary endothelium
alveolar basement membrane
perivascular tissues
perilymphatic tissues
What is interstitial lung disease? **Give 4 characteristic findings of ILD. What will the CXR look like?
a group of pulmonary disorders (>200) characterized by a similar pathology with an insidious and progressive presentation
-damaged alveoli and surrounding tissue
-dyspnea on exertion (DOE)
-persistent dry cough
-late inspiratory rales on PE
CXR - septal thickening and reticulonodular changes
What are 3 known causes of ILD?
drugs
connective tissue disease
environmental exposures
**What is the MC type of ILD?
idiopathic pulmonary fibrosis
Lung parenchyma is composed of the ____, _____ and ______
bronchioles, alveoli and capillaries
What is the pathophys behind ILD? What does it result in?
injury to the alveolar epithelial or capillary endothelial cells (alveolitis) leads to progressive, irreversible scarring and stiffness of lung parenchyma
repetitive and/or excessive injury
FOLLOWED BY
dysregulation of tissue repair
results in poor O2 exchange
What are the 2 histopathological categories that lead to ILD?
granulomatous lung disease
inflammation and fibrosis
What is happening in granulomatous lung disease?
accumulation of T lymphocytes, macrophages, and epithelioid cells¹ organized into discrete structures (granulomas) within in the lung parenchyma
granulomas becomes fibrotic
What is happening in inflammation and fibrosis ILD?
repetitive injury results in chronic inflammation leading to fibrotic alveoli
What is a granuloma?
hard cluster of cells that is scarred
When trying to determine a cause, what is important to note about ILD?
requires a thorough past/present history!!! including meds, social, family/past medical history
How do the majority of ILD present?
usually takes months to years to occur aka the onset is SLOW
present when a pt is in their 20s-40s
SMOKING!!
**What are the 2 MC PE findings in ILD? What will their lungs sound like? Is wheezing common?
**progressive dyspnea (usually on exertion)
**nonproductive cough
lungs: LATE inspiratory rales , rhonchi, and tachypnea
wheezing is NOT common
ILD will have (early/late) inspiratory rales. Where will the rales be heard?
LATE inspiratory rales
often heard first bibasilar, in the posterior axillary line
What will late disease ILD present like?
-Pulmonary Hypertension
-Loud P2 component of the 2nd heart sound
-a fixed split S2
-a holosystolic tricuspid regurgitation murmur
-pedal edema
-digital clubbing
What will a CXR/CT show on a pt with ILD? Which testing is better?
bibasilar reticular and/or reticulonodular pattern with honeycombing in late stage
high resolution CT w/o contrast is preferred to CXR
CXR is normal in 10% of pts with ILD
What does honeycombing on a CXR indicate?
Honeycombing - indicates small cystic spaces with fibrosis
What are some tests that you should order if you suspect ILD?
spirometry
DLCO
pulse oximetry
6 minute walk test
ABG
EKG
CMP
CBC
rheumatic testing (ANA, RF)
Most PFTs for ILD will be _______. What will their TLC, FEV1 and FVC be?
RESTRICTIVE!!
reduced total lung capacity (TLC)
reduced TLC → reduction in FEV1 and FVC
What type of PFT pattern will sarcoidosis show? These patients will have a reduced _____
obstructive
reduced FEV1/FVC ratio
a 6 minute walk test of ______ during 6 minute walk test (6MWT) is associated with increased mortality
desaturation <88%
an EKG for ILD will be _____ unless ______ is present
normal
pulmonary hypertension is present
_______ can be ordered to obtains samples of cells and pulmonary fluid for assessment of cell count, cultures and cytologic analysis. What is the result in ILD?
Bronchoalveolar Lavage during a flexible bronchoscopy
BAL in ILD is usually nonspecific
_______ is used as a last resort to confirm the dx of ILD and/or to stage disease. How?
lung bx
the histopathologic pattern is evaluated in combination with the clinical information to determine the diagnosis
What is the tx for ILD?
-remove the offending agent (med or occupation)
-supplemental oxygen if less than 88% at rest or exertion
-prednisone 0.5-1mg qd for 4-12 weeks, if improved or stable taper dose for an additional 4-12 weeks. No improvement add immunosuppressant
What is the goal O2 stat range? How do you determine the dose?
Goal: O2 sat 90-92%
Dose is determined by performing pulse ox testing (at rest and with exertion) while slowly titrating supplemental oxygen
How often should you f/u with a pt who has ILD?
follow up q3-6 months to reassess PFT and development for comorbid conditions
What are 2 important pt education points to remember about ILD?
fibrosis is irreversible
compliance is vital to slow progression
What is the pathophys behind idiopathic pulmonary fibrosis?
An epithelial-fibroblastic disease, in which endogenous or environmental stimuli disrupt the homeostasis of alveolar epithelial cells leading to abnormal epithelial cell repair and fibrosis
Excessive production and dysregulation of myofibroblasts
How will idiopathic pulmonary fibrosis present? What is the MC pt population?
gradual onset of exertional dyspnea with nonproductive cough
fine inspiratory rales/crackles with or without digital clubbing
55-60 y/o with slight male predominance
What will the PFT look like for idiopathic pulmonary fibrosis?
often reveals a restrictive pattern on PFT , a reduced DLCO and hypoxemia that is exaggerated or elicited by exercise
What will imaging look like for idiopathic pulmonary fibrosis?
nonspecific reticular markings
HRCT scan typically shows:
bibasilar
reticular opacities
traction bronchiectasis
honeycombing
What is VATS? When is it used?
Video-assisted thoracic surgery (VATS) is a minimally invasive procedure that allows surgeons to diagnose and treat chest conditions using a small camera and incisions
used to dx idiopathic pulmonary fibrosis: will show alternating areas of healthy lung, interstitial inflammation, fibrosis, and honeycomb change with FIBROSIS predominating over inflammation
What is the tx for IPF? What are the drug class for each? Why do you use them?
nintedanib (Ofev) - tyrosine kinase inhibitor
pirfenidone (Esbriet) - anti-inflammatory agent; antifibrotic agent
refer for lung transplant
supportive care (need to get covid vaccine)
Doesn’t reverse fibrosis but can prevent further scarring
What is the major SE of nintedanib (Ofev) and pirfenidone (Esbriet)?
high risk of drug induced liver injury so need to monitor LFTs q1m x 6 months then q3m
What is sarcoidosis? What is the MC organ affected? What are the 2nd MC organs?
an inflammatory disease, of unknown etiology¹, characterized by the presence of noncaseating² (non-necrotizing) granulomas involving two or more organ systems
lungs -MC
skin and eyes- 2nd MC
sarciodosis has the highest prevalence in those of _____ and ______ descent. How does the dz present in each? What age range?
African American (AA): acute and severe
Northern European (NE): mild and chronic
onset is between 20-60 years old
Dyspnea
cough
fatigue
fevers
night sweats
weight loss
Erythema nodosum
Lupus pernio
Maculopapular lesions
What am I?
What will the lung exam sound like?
Sarcoidosis
lung exam will likely be normal, wheezing heard if endobronchial involvement or traction bronchiectasis due to scarring
What is Erythema nodosum? What dz is it associated with?
A lower-extremity panniculitis¹ with painful, erythematous nodules
sarcoidosis
What is lupus pernio? What dz is it associated with?
Violaceous rash on the cheeks or nose
sarcoidosis
What is the MC form of sarcoidosis?
Maculopapular lesions (flat, raised lesions)
What are the ocular findings in sarcoidosis?
Anterior or posterior granulomatous uveitis
Conjunctival nodules
scleral plaques
What are 3 common lab findings in sarcoidosis?
high calcium (suppressed PTH)
elevated ESR
elevated ACE levels
Why do you see elevated calcium in sarcoidosis?
Granulomas produce 1,25 dihydroxyvitamin D which increases intestinal absorption of Ca - ultimately results in a suppressed PTH
Why do you see elevated ACE levels in sarcoidosis?
Pulmonary granulomas secrete ACE, ACE is naturally produced in the lung endothelium
ACE elevation is NOT sensitive nor specific for sarcoidosis
How do you stage sarcoidosis? What are the staging requirements? Where are the infiltrates predominantly found?
staged based on lung involvement on the CXR
Stage I - hilar adenopathy alone
Stage 2 - a combination of adenopathy plus infiltrates
Stage 3 - infiltrates alone
Stage 4 - fibrosis
Usually the infiltrates are predominantly found in the upper lobes
What will HRCT in sarcoidosis show?
adenopathy >2 cm in the short axis supports the diagnosis of sarcoidosis over other interstitial lung diseases
infiltrates that are patchy reticular nodularity
confluent nodularity
______ is needed to confirm dx of sarcoidosis. What will it show?
transbronchial biopsy via bronchoscopy for pulmonary granuloma OR extrapulmonary location is acceptable: skin lesion, palpable lymph node with histologic finding of NONCASEATING GRANULOMAS
When would a lung and/or mediastinal lymph node bx via thoracoscopy be indicated when working a pt up for sarcoidosis?
only if atypical on imaging or less invasive testing is indeterminate
What will a bronchoalveolar lavage during bronchoscopy show if a pt has sarcoidosis?
Shows increased lymphocytes, high CD4/CD8 cell ratio
What is the standard of care in ACUTE sarcoidosis? What are the 2nd line options?
oral/topical steroids
2nd line: immunomodulators (MTX, hydroxychloroquine, azathioprine
**What are all the txnoptions for acute sarcoidosis depending on the number of organs involved?
What is considered chronic sarcoidosis?
acute that has NOT resolved within 2-5 years
What is the tx for pts with advanced pulmonary fibrosis due to sarcoidosis? All pts dx with sarcoidosis need to see _______
lung transplant!!
Ophthalmology at onset and yearly
Occupational and Environmental Lung Disease that is uncomplicated with show a ____ pattern and ____ DLCO. What will complicated disease show?
uncomplicated disease - restrictive pattern and a decreased DLCO
complicated disease - obstructive pattern with decreased DLCO
What is pneumoconiosis? What are the 3 subtypes? What is the tx?
A chronic fibrotic lung disease caused by the inhalation of inorganic dusts
Coal Workers Pneumoconiosis aka “black lung”
Silicosis
Asbestosis
supportive care!!
What is the pathophys behind Coal Workers Pneumoconiosis?
alveolar macrophages ingest inhaled coal dust leading to the formation of “coal macules”, usually 2–5 mm in diameter
What are the 2 different presentations of Coal Workers Pneumoconiosis?
What is silicosis? What is the pathophys?
Silicosis is a fibronodular lung disease caused by inhalation of dust containing crystalline silica
alveolar macrophages ingest the particles inducing an inflammatory response resulting in cell damage and fibroblast release leading to fibrosis
What are the different presentations of silicosis? What are the 2 types of ____ silicosis?
acute silicosis vs chronic silicosis
chronic simple and chronic complicated
silicosis will have ____ heard while listening for lung sounds. What will acute silicosis look like on CXR?
rales
Acute Silicosis - bilateral, perihilar or basilar, diffuse ground glass opacities
(acute is usually the LOWER lobes)
What will simple silicosis look like on imaging?
Simple Silicosis multiple small (< 10 mm) nodules that are scattered diffusely throughout the lungs but may be more prominent in the upper lung fields.
What will complicated silicosis look like on CXR? CT?
Complicated Silicosis bilateral upper lobe masses, which are formed by the coalescence of nodules
CT: small nodules are seen coalesced into larger masses
silicosis pts are at an increased risk for ______. Why?
pulmonary TB
Silica causes alveolar macrophage dysfunction and the body’s initial immune response to TB exposure is through alveolar macrophages
What should silicosis pts be screened for yearly?
All patients with silicosis should have a yearly tuberculin skin test (PPD) and screening CXR
Why is asbestos considered especially harmful for the lungs?
because the minerals are shaped like long, thin fibers
What is the pathophys behind asbestosis?
asbestos fibers are inhaled and completely or partially ingested by macrophages depending on the size of the fiber resulting in an inflammatory response, fibroblast proliferation and chronic scarring
When will s/s of asbestosis appera? What is the MC symptom? What will the PE look like?
Symptoms appear only after a latent period of 20 years or longer
Dyspnea on exertion (DOE) that gets worse with time
PE: normal or bibasilar, fine end-inspiratory crackles
clubbing
What will a CXR with asbestosis look like? What is the highlighted finding?
linear (reticular) opacities (often seen first)
multinodular parenchymal opacities of various size and shape
pleural plaques
honeycomb changes in advanced cases
asbestosis: Visceral pleura may be _____ and associated with parietal ______, while the central portions of the lung are relatively spared
fibrotic
pleural plaques
up to 30% of asbestos exposed patients have normal ____ and abnormal _____
CXR
CT
When is a Bronchoalveolar Lavage (BAL) used in asbestosis? What will it show?
only used if HRCT is NOT diagnostic
BAL fluid will show asbestos bodies,
even in asymptomatic patients
asbestosis pts need to stop smoking due to increased risk of _______, especially ______
lung carcinoma
especially mesothelioma (Mesothelioma is a rare cancer that affects the lining of the pleura most commonly the pleura of the lungs)
**What is one ILD that will present acutely? What is it?
Hypersensitivity Pneumonitis
A nonatopic, nonasthmatic inflammatory pulmonary disease resulting from exposure to inhaled organic antigens leading to an acute illness
What is the pathophys behind Hypersensitivity Pneumonitis?
immune-mediated disorders characterized by diffuse inflammation of interstitial lung, terminal bronchioles, and alveoli
acute onset of flu like illness with fever, chills, malaise, cough, chest tightness, dyspnea, and headache
What am I?
What is important to note?
acute Hypersensitivity Pneumonitis
presents within HOURS following a significant exposure
What is the course of acute hypersensitivity pneumonitis? What does a CXR look like?
gradual improvement within 12 hours to several days following exposure removal
a poorly defined micronodular or diffuse interstitial pattern
onset over weeks or months
productive cough, dyspnea, fatigue, anorexia, and weight loss
What am I?
What does it progress to?
subacute/chronic hypersensitivity pneumonitis
progresses to persistent cough and dyspnea
What does a CXR look like with a pt who has subacute/chronic hypersensitivity pneumonitis?
progressive fibrotic changes with loss of lung volume and coarse linear opacities are common
What is super important in hypersensitivity pneumonitis? What is the tx?
Prompt diagnosis is IMPORTANT
tx: identify and remove the offending agent
oral steroids
in radiation lung injury, what are 5 factors that determine the severity of injury?
-Volume of lung irradiated
-Dose and rate of exposure
-concurrent chemo
-previous radiation therapy in the same area
-withdrawal of corticosteroids
What are the top 3 previous cancer diagnosis that is associated with radiation lung injury? What are the 2 phases of the pulmonary response to radiation?
breast, lung and lymphoma
an acute phase (radiation pneumonitis)
chronic phase (pulmonary radiation fibrosis)
When does radiation pneumonitis onset? What can be heard when listening to the lungs?
2-3 months (range 1-12 months) after completion of radiation therapy
Inspiratory rales may be heard in the involved area
insidious onset of dyspnea, intractable dry cough, chest fullness or pain, weakness, and fever
In severe disease, respiratory distress and cyanosis occur that are characteristic of ARDS
What am I?
What is the tx?
radiation pneumonitis
supportive care +/- steroids
CXR showing:
alveolar or nodular opacities limited to the irradiated area
Air bronchograms are often observed
What am I?
radiation pneumonitis
Pulmonary Radiation Fibrosis is MC in patients who ????? How does it present?
receive a full course of radiation therapy for cancer of the lung or breast
most patients are asymptomatic, although slowly progressive dyspnea may occur
CXR for _____ will show obliteration of normal lung markings
dense interstitial and pleural fibrosis
reduced lung volumes
tenting of the diaphragm
sharp delineation of the irradiated area
What is the tx?
Pulmonary Radiation Fibrosis
no specific tx is indicated, steroids have NO VALUE
What is the source behind ILD in connective tissue disorders?
CTD’s are autoimmune disorders of unknown etiology that lead to inflammation and damage to the connective tissues
What are the top 5 connective tissue disorders associated with ILD?
Rheumatoid Arthritis
Systemic Lupus Erythematosus
Sjögren Syndrome
Progressive Systemic Sclerosis
Polymyositis and Dermatomyositis
_____ symptoms occasionally precede the more typical systemic manifestations of CTD’s by months or years. What should you do next? Name a few additional organ systems
Pulmonary
Patients with ILD should always be evaluated for clinical findings suggestive of a CTDs including musculoskeletal pain, weakness, fatigue, fever, joint pain or swelling, photosensitivity, Raynaud’s phenomenon, pleuritis, dry eyes, dry mouth
**What are the top 9 drugs that are known to induce ILD?
amiodarone
nitrofurantoin
sulfonamides
bleomycin
cyclophosphamide
methotrexate
nitrosoureas
gold salts
penicillamine
phenytoin
drug class: antiarrhythmic, antibacterial, antineoplastic, antirheumatic
What are the MC symptoms of drug induced ILD? What is the normal timeframe it? What is the tx?
exertional dyspnea and nonproductive cough
The drug may have been taken for several years before a reaction develops and symptoms may begin weeks to years after the drug has been discontinued
tx: d/c medication causes it and supportive care
ILD are a group of disorders that occur due to an inflammatory reaction resulting in _______.
_____ and ______ are the most common symptoms
Most ILD’s are _____ in nature
_______ are the most common findings on imaging
scarring of the lung tissue
Dry cough and dyspnea
restrictive
Reticulonodular changes
