Interstital Lung Disease Flashcards
symptoms of interstitial lung disease?
- Dyspnea–> often progressive (duration and pace)
- decreased exercise tolerance
- chronic non-productive cough
- fatigue
- extrapulmonary symptoms
- ROS (signs/symptoms of CTD)
who is usually affected in ILD?
female- CTD, sarcoidosis , others
males- occupational diseases (asbestos) IPF
< 40 - Sarcoidosis, CTD, inherited disease (pulmonary fibrosis) others
> 50 - IPF, asbestosis, fibrotic HP
common co-morbidities of ILD? and medications that have a relation to ILD?
Common co-morbidities
- OSA, GERD, Afib, lung cancer/cancer, emphysema, pulmonary hypertension
- Medications: nitrofurantoin, amiodarone, statins, methotrexate, chemotherapy, check point inhibitors
what are some things that may be seen on physical examination of ILD?
- resting exertional hypoxemia (6MWT)
- bilateral velcro inspiration crackles
- abnormal heart sounds
- clubbing
evaulate skin/joints for signs of CTD
What PFT should be done in ILD?
spirometry, lung volumes and DLCO
What is considered the linear pattern in imaging of ILD?
Septal pattern: thickening of the interlobular septa
- lung edema, lymphangitic disease
reticular pattern: interstitial thickening/ fibrosis (curvilinear/lacy)
- UIP, NSIP, Fibrotic HP
UIP pattern on HRCT
- Subpleural, basal predominance
- reticular abnormality
- honeycombing (looks like cysts) with or without traction bronchiectasis ( when the airway stays open as you get to the the periphery)
NSIP pattern in HSCT
- symmetric reticular opacities with lower lung zone predominance
- traction bronchiectasis and lobar volume loss
- ground glass opacities
- subplerual sparing
- chronic progressive fibrosing interstitial pneumonia of unknown etiology
- clinical M > F, smoking history, age > 60, insidious onset dry cough and or dyspnea, lungs with bibasilar crackles
- radiology: HRCT with UIP (unusual interstitial pneumonia) pattern
- histopath: UIP
- heterogenous appearance of fibrosis (dense collagen, Fibroblastic foci) alternating with normal/less affected parenchyma
diagnosis of exclusion
Idiopathic pulmonary fibrosis (IPF)
treatment for IPF?
Pirfenidone
- inhibits TGF-beta stimulated collagen synthesis and blocks fibroblast proliferation
- SE: GI upset (nausea, dyspepsia, vomiting, anorexia) photosensitivity, rashes, minor disturbances of liver enzymes
Nintedanib: BID dosing
- Receptor blocker for multiple tyrosine kinases
- SE: diarrhea, elevation of liver enzymes
Both medication slow disease progression
- syndrome which results from repeated inhalation of/ sensitization to aerosolized antigens
- numerous culprit agents (farmer’s lung & bird fanciers disease)
- acute: flu like sxs, fever, chills, cough, dyspnea, myalgias
- chronic: DOE, cough, Crackles, Insp, squeaks
- T-cell hyperreactivity & immune complex formation
- various categorization: “classic” acute, subacute, chronic HP vs acute/inflammatory or chronic/fibrotic
chronic hypersensitivity pneumonitis
diagnosis of hypersensitivity pneumonitis?
- high index of suspicion
- radiology (CT- upper lobe predominance, ground glass and fibrosis, honeycombing)
- antigen detection (IgGO
- bronchoscopy
- +/- lung biopsy
- if confident clincal diagnosis, then no need for biopsy
management of HP?
- one trial with success of corticosteroids in acute HP (> 20 years old)
- exposure avoidance
- Pharmacologic treatment: corticosteroids, immune-modulating agents, INBUILD trial, lung transplantation
- Pulmonary involvement common in CTD
- highest prevalence in systemic sclerosis, RA, and PM/DM
- leading cause of mortality
- challenges with diagnosis, lack of treatment trials, management
- etiology- lung targeted by disease-specific autoantibodies, certain CTD start in the lungs
Connective tissue Disease- related ILD
Presentation of CTD-ILD
- Overt respiratory symptoms
- incidental finding (subclincal CTD-ILD)
- ILD + “autoimmune-ish” features (do no meet classification of criteria) IPAF
- can precede formal diagnosis of CTD
