38/41: Pathology of the Lung Part 3 and Pneumoconioses - Carnevale

Question 1

what is a restrictive disease briefly?

Answer 1

expansion of lung is hampered by either things external to lung (deformed chest wall, pleural space filled with fluid) or things in the lung (parenchymal = edema, acellular material, granulomas, etc)

Question 2

heterogenous group of disorders characterized by inflammation and fibrosis of the pulmonary connective tissue, principally the most peripheral and delicate interstitium in the alveolar walls

Answer 2

chronic diffuse interstitial diseases

Question 3

hallmark of chronic diffuse interstitial diseases

Answer 3

reduced compliance (stiff lungs) –> dyspnea –> hypoxia

Question 4

how can you clinically distinguish obstructive from restrictive diseases?

Answer 4

FEV1/FVC ratio is not reduced in restrictive disease

Question 5

decreased total lung capacity

FEV normal

FEV/FVC ratio not reduced

=

Answer 5

intersitial lung disease

Question 6

Presentation:
dyspnea, hypoxia, end-inspiratory crackles and eventual cyanosis

X-rays: diffuse bilateral infiltrative lesion by nodules, irregular lines, or ground glass shadows (infiltrative)

Answer 6

interstitial lung disease

Question 7

interstitial lung disease can lead to …

Answer 7

pulmonary HTN or cor pulmonale

end stage = “ honeycomb lung”

Question 8

non-cardiogenic pulmonary edema aka

Answer 8

acute lung injury ALI

abrupt onset of significant hypoxemia and pulmonary infiltrates in absence of cardiac failure; spectrum

Question 9

severe ALI with greater hypoxemia

Answer 9

ARDS

Question 10

what is the histological manifestation in both ARDS and ALI?

Answer 10

diffuse alveolar damage DAD

both are also associated with acute interstitial pneumonia

Question 11

rapid onset of life-threatening respiratory insufficieny refractory to oxygen therapy caused by DAD

Answer 11

ARDS

most common cause of non-cardiogenic pulmonary edema

Question 12

damage to capillary and alveolar membranes leads to…

Answer 12

ALI and ARDS

either direct or indirect injuries

Question 13

bilateral pulmonary infiltrates on chest x-ray and pulmonary capillary wedge pressure less than 18mmHg

Answer 13

ALI/ARDS characteristic

Question 14

PaOz/FiO2 less than 300 =

PaO2/FiO2 less than 200 =

Answer 14

300 ALI
200 ARDS

ie on 60% O2 the patients Pa)2 is less than 120 mmHg

Question 15

explain the basic pathogenesis of ARDS

Answer 15

uncontrolled activation of acute inflammatory system; imbalance between proinflammatory and anti-inflammatory mediators. Sequestration and activation of PMN; diffuse damage to alveolar capillary walls

leads to increased vascular permeability and alveolar thickening

loss of diffusion capacity

widespread surfactant abnormalities

Question 16

two key things seen in early phase of ALi and ARDS in the alveolus

Answer 16

hyaline membrane formation

PMN sequestration and migration into alveolus

Question 17

adult v. neonate ARDS

Answer 17

adults: diffuse damage to alveolar capillary walls (inflammation gone out of control)
neonates: surfactant issues

Question 18

what helps ARDS?

Answer 18

NO inhalation

- decreases PA pressure and arterial resistance

Question 19

three phases of ARDS

Answer 19

acute exudative 0-7 d

proliferative phase 1-3 wk

fibrotic/healing phase 3-4 wk —> possible honeycomb lung

Question 20

TRALI =

Answer 20

transfusion related ALI

no pre-existing acute lung injury before transfusion - occurs during or within 6 hours of transfusion due to anti-HLA or HNA antibodies

Question 21

final stage of DID diffuse interstitial disease

Answer 21

end stage fibrotic lung aka honeycomb lung

Question 22

what cell plays a central role in fibrosis with DID?

Answer 22

macrophage

Question 23

pulmonary disorder of unknown etiology characterized by diffuse interstitial fibrosis aka cryptogenic fibrosing alveolitis

Answer 23

idiopathic pulmonary fibrosis

lung transplant is the only definitive therapy - diagnosis of exclusion

Question 24

pathogenesis stages of IPF

Answer 24

early- fibroblastic foci

temporal heterogenity

late - dense fibrosis and honeycomb lung

Question 25

UIP/IPF needs transplant , what do DIP, COP, BOOP, NSIP, PAP, HSP need?

Answer 25

responsive to steroids

Question 26

non-neoplastic lung reaction to inhalation of any aerosol including mineral dusts, organic and inorganic particles, fumes or vapors =

Answer 26

pneumoconioses

Question 27

smaller particles –>

larger particles –>

Answer 27

small - ALI

large - Fibrosis

Question 28

most dangerous particle size

Answer 28

1-5um

reach terminal small airways and alveoli; lodge in bifurcation of distal airways

Question 29

coal –>
silica –>
asbestos –>

Answer 29

focal dust emphysema
silicotic nodule
fibrosis

Question 30

is there an increased risk for cancer with CWP coal workers pneumoconioisis

Answer 30

no increased risk for lung cancer or TB

Question 31

” black lung disease”

Answer 31

pulmonary massive fibrosis

Question 32

caplan syndrome?

Answer 32

coexistence of RA with a pneumoconiosis –> development of distinctive nodular pulmonary lesions

Question 33

most prevalent chronic occupational disease in world

Answer 33

silicosis

whorls of collagen

from gold, copper, sandblasting, stone cutting, quartz

Question 34

is there an increased risk of cancer with silicosis?

Answer 34

YES - carcinogenic and increased susceptibility to TB

Question 35

asbestos-related diseases form…

Answer 35

serpentines and amphiboles (more pathogenic, brittle)

only amphibole exposure correlates with mesothelioma

Question 36

“dumbbell shaped”

Answer 36

asbestos bodies

Question 37

bleomycin –>

Answer 37

pneumonitis and fibrosis

Question 38

methotrexate –>

Answer 38

hypersensitivity pneumonitis

Question 39

amiodarone –>

Answer 39

pneumonitis and fibrosis

Question 40

nitrofurantoin –>

Answer 40

hypersensitiivty pneumonitis

Question 41

aspirin and b-antagonists –>

Answer 41

bronchospasm

Question 42

1-6 mo after radiation therapy develop acute radiation pneumonitis with fever, pleural effusion, infiltrates. treat with?

Answer 42

steroids- may resolve

Question 43

well-formed non-caseating granulomas in many tissues and organs; diagnosis of exclusion

Answer 43

sarcoidosis

fever, night sweats, weight loss, erythema nodooosum, polyarthritis

diagnosis of exclusion made by biopsy showing non-caseating granulomas, special stains and/or cultures

Question 44

if sarcoidosis involves the eye and salivary gland it is called

Answer 44

mikulicz syndrome

Question 45

what oddities are found in the granulomas of sarcoidosis?

Answer 45

schaumann bodies and asteroid bodies

Question 46

immunologically mediated, predominantly interstitial disorder caused by intense, often prolonged exposure to inhaled organic dusts or antigens

Answer 46

hypersensitivity pneumonitis (type III hypersensitivity)

predominantly restrictive disease

Question 47

spores of thermophilic actinomyces in hay –>

Answer 47

farmers lung hypersensitivity pneumonitis

Question 48

breathe brown gas in silo, may get pulmonary edema in minutes or hours; may get over that and develop widespread bronchiolitis obliterans with scar tissue forming in burned small airways

Answer 48

silo fillers disease- NO/NO2

Question 49

insidious onset of dyspnea and dry cough; no desquamation; numerous intra-alveolar macrophages (smoker’s macrophages); sparse inflammation in thickened alveolar septa; minimal fibrosis; nonprogressive. emphysema often present

Answer 49

desquamative interstitial pneumonia DIP

treat with steroids

Question 50

accumulation of acellular surfactant in intraalvolar and bronchiolar spaces; minimal inflammatory response with normal alveolar walls

Answer 50

pulmonary alveolar proteinosis PAP

inisidious onset of cough; chunks of white gelatinous-appearing sputum “jello” fever

Question 51

treatment for pulmonary alveolar proteinosis

Answer 51

lavage