Clin Med: Interstitial Lung Disease Flashcards
Interstitial Lung Disease is AKA
pulmonary fibrosis
ILD pathophysiology
inflammation – fibrosis (deposits) – impaired gas exchange
Types of ILD
exposure related
autoimmune related
idiopathic (m/c)
ILD presentation
often chronic sx
progressive exertional dyspnea
dry cough
ssx of autoimmune disorder
asymptomatic
other: pleuritic CP, hemoptysis
ILD associated with
occupational exposures
autoimmune conditions
Imaging for ILD
usually CXR firstline
High resolution CT is diagnostic and is best imaging
If CT not diagnostic –> bx
PFTs added to assess severity
PE for ILD
non-specific:
bibasilar crackles
clubbing of fingers
PE consistent with Pulm HTN
tachypnea
reduced chest expansion
ILD findings on CXR
bronchiectasis (tram-track appearance)
miliary pattern (1-4mm nodules - looks fuzzy)
honeycombing (lacy reticular pattern)
kerley lines (linear opacities)
reticular or reticulonodular opacities (finer pattern than honeycombing)
Treatment goal
fibrosis is irreversible so goal is to prevent and limit further damage
Treatment (pharm)
Corticosteroids (prednisone)
antifibrotic agents (pirfenidone and nintedanib) for idiopathic pulmonary fibrosis
treatment of GERD (omeprazole or pantoprazole)
Treatment (pharm)
Corticosteroids (prednisone)
antifibrotic agents (pirfenidone and nintedanib) for idiopathic pulmonary fibrosis
treatment of GERD (omeprazole or pantoprazole)
Treatment (supportive)
supplemental O2
pulm rehab
tobacco cessation
flu and pneumococcal vaccines
lung transplant
For refractory ILD only definitive treatment is
lung transplant (for pts under 65 and otherwise healthy and have refractory disease)
Idiopathic Pulmonary Fibrosis is when
there is recurrent injury to alveolar epithelium
no significant occupation hx, medication exposures, or PMH
Presentation of Idiopathic Pulmonary fibrosis
present with DOE and dry cough, often chronic for 1 yr
classically with bibasilar end inspiratory crackles on exam
Characteristic usual interstitial pneumonia pattern on imaging
basilar reticular opacities
honeycombing
bronchiectasis
DX criteria for IPF
age less than 65
no known cause of ssx
inspiratory crackles on PE
restrictive PFT
classic UIP imaging findings
Treatment of IPF
Corticosteroids (prednisone)
antifibrotic agents (pirfenidone and nintedanib) for idiopathic pulmonary fibrosis
treatment of GERD (omeprazole or pantoprazole)
O2, vaccines, tobacco cessation, lung transplant
Sarcoidosis is a __________ disorder
autoimmune
systemic inflammatory disorder
Sarcoidosis is less strongly correlated with
fHx
Sarcoidosis is characterized by
non-caseating granuloma formation (no necrotic center)
Difference between sarcoidosis and tb in granuloma formations
TB - caseating granulomas
Sarcoidosis - non-caseating granulomas
Sarcoidosis presentation
DOE, cough, CP, crackles
other systemic sx - autoimmune sx
fatigue, malaise, fever, wt loss, weakness, anorexia
CXR sarcoidosis findings
hilar adenopathy
central nodular opacities
predilection for the upper lobes
What is used for definitive dx in sarcoidosis
bx - non-caseating granuloma is pathognomonic
Sarcoidosis treatment (pharm)
glucocorticoids - prednisone is 1st line
methotrexate - second line
azathioprine
hydroxychloroquine
TNF alpha inhibitors (infliximab)
Sarcoidosis: if acute and no to mild sx =
no treatment necessary
Sarcoidosis: if one organ involved =
topical/ localized therapy
Sarcoidosis: if multi-organ involvement
systemic therapy
