Interstitial Lung Disease (ILD) (INC) Flashcards
What is the interstitum of the lung?
Space that sits between alveolar wall and the blood vessel - where there is O2 and CO2 exchange
What is the clincal presentaion of intertsituim>
damaged alveoli and surrounding tissue
dyspnea on exertion (DOE)
persistent dry cough
late inspiratory rales on PE
results from forced opening of alveoli
CXR - septal thickening and reticulonodular changes (sacs of alveoli)
occasionally ILD will be found incidentally during work-up for another condition
Why is there late inspiratory rales?
Difficulity of alveoli to move at the end
What structures are affected by ILD
alveolar epithelium
pulmonary capillary endothelium
alveolar basement membrane
perivascular tissues
perilymphatic tissues
Layman’s Terms: the tissue and space around the air sacs of the lungs
What are the classifications of ILD
Drugs, connective tissue, environmental exposures, idiopathic pulmonary fibrosis (MC), sarcodoidosis
What is the pathophys of ILD?
Chronic damage to alveolar epithelial or capillary endothelial cells (alveolitis) leads to progressive, irreversible scarring and stiffness of lung parenchyma
results in poor O2 exchange (hypoxia and hypercapnia)
this receptive injury leads to dysregulation of tissue repair
What things can cause ILD?
genetic predisposition
autoimmune d/o
superimposed disease (multiple problems)
not really sure
What are the two histopahological categories of ILD?
Granulomatous Lung Disease
Inflammation and Fibrosis
both lead to poor O2 delivery
Explain Inflammation and Fibrosis
repetitive injury results in chronic inflammation leading to fibrotic alveoli
Explain Granulomatous Lung Disease
accumulation of T lymphocytes, macrophages, and epithelioid cells¹ organized into discrete structures (granulomas) within in the lung parenchyma
granulomas become fibrotic
If you suspect ILD, what should you ask patients?
Medication history
Social history (exposures)
Family history
What is the onset of ILD typically? At what age?
Chronic - months to years
majority of ILD’s
typically at 20-40 years old and not older (typically because older patients think it is there normal baseline)
What is the social history that signifies ILD?
Smoking is common
What is the ROS of ILD?
Dyspnea (exertion, then at rest)
Cough
all other ROS is negative
What is the ILD of PE as disease progresses?
SOB
cachexia
ronchi
What is the CXR of ILD?
bibasilar reticular and/or reticulonodular pattern with honeycombing in late stage
honeycombing indicates poor prognosis (scar tissue pulls bronchiole open too much)
What type of lung disease is ILD?
Typically restrictive
If you suspect pulmonary hyertension, what should you do?
EKG and Echo
What labs do you typically order for ILD?
CMP
Bronchoalveolar lavage (bronchoscopy, looks at cells to r/o malginant, infection causes)
Lung biopsy
r/o the other differentials
What is the lung biopsy with ILD?
Shows normal, then scarred, normal, then scarred pattern
Honeycomb
If patients are symptomatic, what do you do for ILD?
remove offending agent (if known)
manage hypoxemia (oxygen)
suppression of inflammatory process (steroids)
improve quality of life (pulmonary rehab)
manage complications (pulmonary hypertension and cor pulmonale¹)
When do you use O2 supplementation and what is the goal?
<88%
Goal O2 90-92%
Typically start with 2 L O2 with Nasal Calculi
What is the thought behind using glucocorticoids for ILD?
Reduce inflammation
Start high, taper off
prednisone
Low success rate
If a patient is non-responsive to glucocorticoid, what do you use?
Immunosuppresent to stop immune response
What type of therapy is used for ILD?
Pulmonary rehabilitation
a program of exercise, education, and support to help patients function at the highest level possible
Exercise - close monitoring of VS
Breathing techniques
Nutrition
Relaxation
Emotional and group support
Learning more about your medications
How do you monitor ILD patients?
Follow up every 3-6 months
reassess symptoms, PFT (spirometry, DLCO, pulse oximetry)
monitoring for development of comorbid conditions
hypoxemia, pulmonary hypertension, thromboembolic disease, COPD, heart failure, obstructive sleep apnea, depression
evaluate the clinical course and identify patients who develop accelerated deterioration
Is ILD reversible?
No - only if acute
treatment just to reduce progression
What is the MC ILD?
Idiopathic pulmonary fibrosis
What is the pathophys for 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 myofibroblasts¹ leads to hard stiff lungs
Management is to reduce the # of fibroblasts
What are the clinical findings of IPF (idiopathic pulmonary fibrosis)?
gradual onset of exertional dyspnea with nonproductive cough
MC onset 55-60 y/o with slight male predominance
fine inspiratory rales/crackles with or without digital clubbing
PFT
often reveals a restrictive pattern on PFT , a reduced DLCO and hypoxemia that is exaggerated or elicited by exercise
If a CT scan does not show idiopathic pulmonary fibrosis, what do you do? What do you see?
Lung biopsy w/ video assisted throacic surgery (VATS)
sent to lab and given a report
fibrosis and
What was the conclusion of PANTHER-IPF study
Glucocorticoids and other anti-inflammatories are no longer indicated in IPF
What is now recommended for idiopathic pulmonary fibrosis?
Antifibrotic therapy
What are the antifbribtoics and SE
nintedanib (Ofev) - tyrosine kinase inhibitor²
pirfenidone (Esbriet) - anti-inflammatory agent; antifibrotic agent³
Doesn’t reverse fibrosis but can prevent further scarring
High risk of drug induced liver injury; CI in severe liver disease
monitor LFT’s before, during, and after therapy
How do you manage idiopathic pulmonary fibrosis?
Early referral for lung transplant
What should you encourage patients to do if they have IPF?
Join a clinical trial!
