Interstital Lung Disease

Question 1

symptoms of interstitial lung disease?

Answer 1

• Dyspnea–> often progressive (duration and pace)
• decreased exercise tolerance
• chronic non-productive cough
• fatigue
• extrapulmonary symptoms
• ROS (signs/symptoms of CTD)

Question 2

who is usually affected in ILD?

Answer 2

female- CTD, sarcoidosis , others
males- occupational diseases (asbestos) IPF
< 40 - Sarcoidosis, CTD, inherited disease (pulmonary fibrosis) others
> 50 - IPF, asbestosis, fibrotic HP

Question 3

common co-morbidities of ILD? and medications that have a relation to ILD?

Answer 3

Common co-morbidities

• OSA, GERD, Afib, lung cancer/cancer, emphysema, pulmonary hypertension
• Medications: nitrofurantoin, amiodarone, statins, methotrexate, chemotherapy, check point inhibitors

Question 4

what are some things that may be seen on physical examination of ILD?

Answer 4

• resting exertional hypoxemia (6MWT)
• bilateral velcro inspiration crackles
• abnormal heart sounds
• clubbing

evaulate skin/joints for signs of CTD

Question 5

What PFT should be done in ILD?

Answer 5

spirometry, lung volumes and DLCO

Question 6

What is considered the linear pattern in imaging of ILD?

Answer 6

Septal pattern: thickening of the interlobular septa

• lung edema, lymphangitic disease

reticular pattern: interstitial thickening/ fibrosis (curvilinear/lacy)

• UIP, NSIP, Fibrotic HP

Question 7

UIP pattern on HRCT

Answer 7

• 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)

Question 8

NSIP pattern in HSCT

Answer 8

• symmetric reticular opacities with lower lung zone predominance
• traction bronchiectasis and lobar volume loss
• ground glass opacities
• subplerual sparing

Question 9

• 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

Answer 9

Idiopathic pulmonary fibrosis (IPF)

Question 10

treatment for IPF?

Answer 10

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

Question 11

• 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

Answer 11

chronic hypersensitivity pneumonitis

Question 12

diagnosis of hypersensitivity pneumonitis?

Answer 12

• 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

Question 13

management of HP?

Answer 13

• one trial with success of corticosteroids in acute HP (> 20 years old)
• exposure avoidance
• Pharmacologic treatment: corticosteroids, immune-modulating agents, INBUILD trial, lung transplantation

Question 14

• 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

Answer 14

Connective tissue Disease- related ILD

Question 15

Presentation of CTD-ILD

Answer 15

• 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

Question 16

• caused by the inhalation of asbestos fibers
• two different mineral silicates: Sepentine (chrysolite) and amphibole
• 3 forms of exposure: direct work, bystander exposure (electricians, masons, and painters), general community expsure (road surfaces, playground material, landfills and chemical plants) most common form.
• Risk of diseae is more with amphibole than serpentine fibers

Answer 16

Asbestosis

Question 17

pathophsyiology of asbestosis?

Answer 17

• deposition of asbestosis fibers in the lung- accumulation of macrophages followed by fibroblasts that lay the foundation of fibrosis- longer fibers are more potent
• fibrosis increases overtiem
• amphibole is linked to autoantibodies production
• asbestos functions as a tumor initiator and promotor

Question 18

evaluation of asbestosis will show?

• PFT?
• FEV1/FVC ration?
• DLCO?
• ABG?
• CXR?
• HRCT

Answer 18

• PFT lung volumes, FVC, FEV1, TLC; are reduced
• FEV1/FVC ratio is either normal or increased
• DLCO is reduced
• ABG shows paO2 normal or may reveal hypoxia and respiratory alkalosis
• CXR: diffuse reticulonodular infiltrates at the lung bases- shaggy heart borders
• HRCT: ground glass opacities, along with diffuse interstitial fibrosis. pleural thickening and calcified pleural plaques in tomography

Question 19

Histopathology of asbestosis will show?

Answer 19

• Ferruginous bodies

Question 20

treatment of asbestosis?

Answer 20

• Mostly supportive (prevention is the best management)
• PO prednisone (low success)
• colchicine is an antifibrotic (mildly effective)
• tobacco cessation and the use of COPD bronchodilators
• oxygen and pulmonay rehab have shown improved quality of life
• lung transplant

Question 21

• cancer cause by asbestos exposure
• most commonly forms in the lining around the luncs (calcified plerual plaques) or abdomen
• average life expectency is 18-31 months
• sx: symptoms can include chest pain, SOB, and fatigue. can take 10-50 years to present after initial exposure
• tx: plans with surgery, chemotherapy, and radiation can help improve prognosis

Answer 21

mesothelioma

Question 22

• sx: fatigue, lethargy, cough, fever, wt loss, arthralgias, lungs CTA, eye, skin and other
• Dx: non-caseating granulomatous inflammation
• AA population and women
• tx: Prednisone

Answer 22

Sarcoidosis