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Pulmonology > Lecture 17-18: Interstitial Lung Disease and Pathology > Flashcards

Lecture 17-18: Interstitial Lung Disease and Pathology Flashcards

1
Q

PFT shows what in interstitial lung disease

A

Restrictive physiology and impaired gas exchange

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2
Q

ILD: acute or chronic?

A

Chronic typically

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3
Q

ILD: symptoms

A

Nonspecific: dyspnea, dry cough

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4
Q

ILD: FVC & TLC, DLCO, A-a gradient

A

Decreased FVC and TLC with PRESERVED FEV1 / FVC ratio, decreased DLCO, increased A-a gradient

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5
Q

ILD: hypoxemia?

A

Yes

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6
Q

ILD: imaging dx

A

High resolution CT scan to visualize fibrosis

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7
Q

Signs of fibrosis from early –> late

A

Increased reticular densities, traction bronchiectasis, honeycomb changes, cysts

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8
Q

What does reticular densities look like? Early or late?

A

Increased “lines” (thickened interalveolar septum); early fibrosis

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9
Q

What is traction bronchiectasis?

A

Dilated airways pulled apart (traction) by fibrosis

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10
Q

Honeycomb and cysts are evidence of what stage of firbrosis

A

Advanced

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11
Q

What is the histological pattern identified for idiopathic pulmonary fibrosis? Can this be associated with other disease?

A

Usual interstitial pneumonia (UIP); yes, known causes of pulmonary fibrosis can look like this

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12
Q

Is idiopathic pulmonary fibrosis deadly? Survival length?

A

YES: most deadly, median survival is 2.8 years

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13
Q

IPF: clinical features

A

Subacute, insidious dyspnea, cough, rales, CLUBBING, PFT’s restrictive, impaired gas exchange (DLCO, A-a gradient)

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14
Q

IPF: diagnostic blood test?

A

Nope

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15
Q

IPF: dx (2 findings from two different modalities)

A

CT scan: interstitial lung disease and biopsy: fibrotic changes –> fibroblast foci

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16
Q

IPF: gross

A

Dense, collagenous, dilated airways with large ares of scarring

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17
Q

IPF: pathogenesis

A

Alveolar epithelial cells injured (unidentified) with PERMANENT cell death and ABNORMAL cell growth = myofibroblast –> collagen and vascular remodeling

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18
Q

What is a myofibroblast

A

Both a SM cell (contractile properties) and a fibroblast cell that produces collagen

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19
Q

IPF: genetics (AD or AR?)

A

25% of IPF patients have a family member with interstitial lung disease; AD

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20
Q

If someone has family members with IPF and they get IPF, what is their course like?

A

More aggressive and occurs at a younger age

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21
Q

IPF: medical tx (2) and surgical tx

A

Pirfenidone (blocks pro-fibrotic cytokines) and nintedanib (trile tyorsine kinase inhibitor); lung transplant

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22
Q

Pirfenidone blocks what two factors?

A

TGF-beta and PDGF (platelet derived GF)

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23
Q

Nintedanib inhibits what three factors?

A

VEGF (vascular endothelial GF), FGF (fibroblast GF), PDGF

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24
Q

Lung transplant survival

A

5 year = 50%

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25
Q

Pulmonary manifestations of CT disease: systems that can be affected (5)

A

Parenchyma, airway, vasculature, serosal membranes (including effusion) and MSK

26
Q

What is a drug-induced cause of interstitial lung disease?

A

Nitrofuranton (used to treat UTIs)

27
Q

Describe sarcoidosis: main finding and organ systems

A

Noncaseating granulomas: delayed hypersensitivity reaction with unknown cause; multi-systemic disease (lungs in 80% of cases)

28
Q

Sarcoidosis is a diagnosis of…

A

Exclusion (must rule out TB, fungal infections)

29
Q

Sarcoidosis: radiological and histological finding

A

X-ray: enlarged lymph nodes in hilar areas; biopsy = large granulomas with multinucleated giant cells and NO NECROSIS

30
Q

What is a typical outcome of ILD?

A

Honeycomb lung

31
Q

Basics of ILD (4)

A

Decreased compliance, lung volumes, impaired diffusion/gas-exchange, development of pulmonary hypertension

32
Q

One theory of ILD points to _________ as a possible cause

A

Abnormal wound healing

33
Q

What components make up the interstitium

A

BM, fibroblasts, collagen fibers, elastic tissue

34
Q

Describe UIP

A

Age: 50+; insidious onset; can be idiopathic, due to drug rxn, or collagen vascular/autoimmune disease

35
Q

UIP pattern on histology plus idiopathic disease clinically =

A

IPF

36
Q

UIP: pathoglogy (2 prime findings)

A

PATCHY fibrosis pronounced beneath pleura/interlobular septa; fibrosis exhibits TEMPORAL HETEROGENEITY (not all the same age)

37
Q

What do we call the new region of temporal heterogeneity found on UIP?

A

Fibroblast focus (next to an area of dense, old fibrosis)

38
Q

Two subtypes of non-specific interstitial pneumonia (NSIP). Worse/better than UIP?

A

Cellular and fibrosing; better (affects younger patients)

39
Q

Cellular NSIP

A

Diffuse chronic inflammatory cell infiltrates without significant alveolar expansion; better prognosis than fibrosing

40
Q

Fibrosing NSIP

A

Diffuse interstitial fibrosis with uniform appearance (temporally uniform), preserved lung architecture and chronic inflammation

41
Q

How can we distinguish fibrosing NSIP from UIP

A

NSIP is not patchy and is all the same age

42
Q

Describe lymphocytic interstitial pneumonia (LIP): who gets it, presentation, radiological finding

A

Rare: seen primarily in patient’s with Sjogren’s syndrome and HIV; presents as cough/dyspnea; radiology shows diffuse ground glass changes with cysts

43
Q

LIP: histological finding

A

Small mature lymphocytes with variable numbers of plasma cells EXPANDING the alveolar septa

44
Q

Interstitial lung diseases with graulomas: two

A

Sarcoidosis, hypersensitivity pneumonitis

45
Q

Classic sarcoidosis findings (2)

A

Bilateraly hilar adenopathy and NON-NECROTIZING epithelioid granulomas

46
Q

Who gets sarcoidosis?

A

20-40 years of age, F > M, black, rare in Asia

47
Q

% sarcoidosis recovery, % with permanent pulmonary loss, rare outcome?

A

65%; 20%; small percentage progress to diffuse interstitial fibrosis

48
Q

Describe sarcoid granulomas

A

Non-necrotizing epithelioid granulomas with tightly packed epithelioid cells, giant cells, lymphocytes; generally adjacent to bronchioles/vessels, etc

49
Q

Dx of sarcoidosis requires…

A

Biopsy

50
Q

Describe hypersensitivity pneumonitis

A

Lung disease due to an organic antigen, can be acute (within hours of exposure) or chronic (extended exposure, maybe with progressive fibrosis)

51
Q

Hypersensitivity pneumonitis: classic triad

A

Chronic bronchiolotis, poorly-formed granulomas, organizing pneumonia

52
Q

Hypersensitivity pneumonitis: long-standing disease may evolve into…

A

Diffuse fibrosis mimicking UIP or NSIP

53
Q

Smoking-related interstitial lung disease (3)

A

Respiratory bronchiolitis associated interstitial lung diseae (RB-ILD), desquamative interstitial pneumonia (DIP), Langerhans cell histiocytosis

54
Q

Describe RB-ILD

A

Clinical symptoms of an interstitial lung disease and only finding on biopsy is RB (the accumulation of macrophages with finely granular brown pigment ONLY around airways)

55
Q

Describe Desquamative Interstitial Pneumonia (DIP)

A

Similar to RB-ILD but more diffuse and severe with insidious onset of SOB

56
Q

DIP: histological findings

A

DIFFUSE collections of intraalveolar macrophages (as opposed to only around airways)

57
Q

What is ABSENT in DIP

A

Minimal changes in alveolar septa and absent significant fibrosis

58
Q

Describe Langerhans cell histiocytosis (on EM?)

A

Bronchiolocentric fibrosis with “stellate” scar formation and variable numbers of Langerhans cells (EM = Birbeck granule)

59
Q

What does a Langerhans cell look like?

A

Polygonal with irregular nuclei with grooves

60
Q

What do we call a macrophage filled with brown smoke material?

A

Smokers’ macrophages

Pulmonology (21 decks)

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