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
What are the ocular findings in sarcoidosis?
Anterior or posterior granulomatous uveitis
Conjunctival nodules
scleral plaques
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 are all the txnoptions for acute sarcoidosis depending on the number of organs involved?
What are the 2 different presentations of Coal Workers Pneumoconiosis?
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
Why is asbestos considered especially harmful for the lungs?
because the minerals are shaped like long, thin fibers
**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
