interstitial lung disease Flashcards
What are other names for interstitial lung disease
Pulmonary fibrosis
Parenchymal disease
What is occurring in the lungs with interstitial lung disease
inflammation causing fibrosis and leading to impaired gas exchange
What is the interstitium
Space that is between the capillary and the alveoli
What is the most common type of interstitial lung disease
idiopathic
What is the epidemiology of interstitial lung disease
diagnosis of exclusion
What is the most common presentations of interstitial lung disease
progressive, exertion dyspnea is most common
Often have chronic symptoms for months to years
What known causes is interstitial lung disease associated with
occupational exposures
autoimmune conditions
How do you workup interstitial lung disease
need thorough history
Lab tests to rule out other causes
What in a persons PMH would raise concern for interstitial lung disease
Connective tissue disorder
Malignancy
Rheumatologic conditions
What is first line imaging for interstitial lung disease
Chest xray (supportive NOT diagnostic)
What is the best imaging test for interstitial lung disease
CT
*Biopsy if CT is inconclusive
How can you assess the severity of interstitial lung disease
PFTs
- normal early on
- restrictive pattern later on in disease
What are supportive Xray findings for interstitial lung disease
Bronchiectasis (tram-track appearance)
Honeycombing
Kerley lines
reticular opacities
military pattern
What is the treatment for interstitial lung disease
No real treatment
-goal is to prevent/limit further damage
What are pharmacologic options for interstitial lung disease
-Corticosteroids
-antifibrotic agents (pirfenidone/nintedanib) -> idiopathic only
-treat GERD w/ H2 blockers
When is a lung transplant beneficial in interstitial lung disease
For refractory disease in patients under 65 who are otherwise healthy
What is the survival rate for idiopathic pulmonary fibrosis
5 year survival around 50%
Which patient population is most affected by idiopathic pulmonary fibrosis
Males > 50
How do patients with IPF generally present
DOE and dry cough for the past year w/ bibasilar end inspiratory crackles on exam
What is seen on imaging with IPF
“usual interstitial pneumonia” patter
-basilar reticular opacities
-honeycombing
-bronchiectasis
What characteristics does a patient need on exam to make a diagnosis of IPF
age >65
Idiopathic
-inspiratory crackles
-restrictive PFT
-Classic UIP imaging findings
What is sarcoidosis
Systemic inflammatory disorder
*Lungs and lymphatic systems are most commonly effected
Which population is sarcoidosis most common in
black patients
females
20-40 years old (bi-modal w/ second peak around 60)
What time of year is sarcoidosis generally present
Winter / early spring
How is sarcoidosis characterized
non-caveating granuloma with no necrotic center
What is present in most patients with sarcoidosis
pulmonary disease
What are extra-pulmonary manifestations of sarcoidosis
Dacryocystitis
erythema nodosum
myopathy
arthritic symptoms
Lupus pernio
What is lupus pernio
violations plaques on the face
What is the primary means for diagnosis of sarcoidosis
CXR
-hilar adenopathy
-central nodular opacities
0 predilection for upper lobes
What is CXR also used for in sarcoidosis besides diagnosis
Staging
What is stage 1 sarcoidosis
hilar adenopathy only
What is stage 2 sarcoidosis
hilar lymphadenopathy + infiltrates
What is stage 3 sarcoidosis
infiltrates alone -> adenopathy resolves
What is stage 4 sarcoidosis
pulmonary fibrosis
How do you get a definitive diagnosis of sarcoidosis
Biopsy from most accessible lesion
When do you treat sarcoidosis
If 1+ organs are involved
What pharmacological agents can be used for sarcoidosis
Glucocorticoids (1st line)
Methotrexate (2nd line)
Azathiopine
hydroxychloroquine
