Restrictive (Interstitial) Lung Diseases

Question 1

What are some structures you can expect to see in histopathology slide of sarcoidosis?

Answer 1

• Schaumann bodies: laminated concretions of calcium and protein
• Asteroid bodies: stellate inclusion in giant cells
  THESE ARE NOT PATHOGNOMONIC

Question 2

like IPF, sarcoidosis is a diagnosed by ______

Answer 2

exclusion

Question 3

what are 4 features of restrictive lung diseases?

Answer 3

• fibrosis
• dyspnea
• V/Q mismatch
• progression to respiratory failure with pulmonary HTN and cor pulmonale

Question 4

uniform fibrosing process giving a chicken wire appearance is seen with _____

Answer 4

NSIP (non specific interstitial pneumonia)

Question 5

hypersensitivity pneumonia aka extrinsic allergic alveoli’s is often associated with _____

Answer 5

occupational exposures where patients inhale organic antigens;
ex. farmer’s lung, silo filler’s disease, byssinosis (textile workers with “Monday morning blues”)

Question 6

_____ presents with diffuse alveolar damage and hyaline membrane similar to those found in ARDS

Answer 6

acute interstitial pneumonia

Question 7

what are the 4 major categories of restrictive lung diseases

Answer 7

• Fibrosing: UIP, NSIP, COP (cryptogenic organizing pneumonia), CVD (collagen vascular disease), pneunoconiosis
• Granulomatous: sarcoid, HP (hypersensitivity pneumonia)
• Eosinophilic: (Loeffler, Drug/allergy, chronic eosinophilic pneumonia)
• Smoking related: DIP (desquamative interstitial pneumonia), RB ILD (respiratory bronchiolitis interstitial lung disease )

Question 8

organizing pneumonia can be seen following: (3)

Answer 8

• infectious pneumonia
• transplantation (lung and bone marrow)
• collagen vascular diseases

Question 9

occupational exposures of inhaled antigens is associated with what type of lung disease?

Answer 9

restrictive (hypersensitivity pneumonia)
↓ diffusion capacity
↓ lung compliance
↓ total lung volume (TLC)

Question 10

how can you tell the difference between UIP/IPF and non specific interstitial pneumonia ?

Answer 10

there is NO HONEYCOMB appearance with non specific interstitial pneumonia

Question 11

sarcoidosis is thought to be due to a type ____ HS reaction to an unidentified antigen

Answer 11

type 4 (driven by CD4 helper T cell)

Question 12

non caseating granulomas in sarcoidosis can be found in what layer?

Answer 12

subepithelial

Question 13

the main feature of restrictive lung diseases is _____

Answer 13

FIBROSIS; the decreased lung compliance will cause ↑ work to breathe and cause dyspnea

Question 14

in what cigarette smoking associated lung diseases does the macrophages accumulate within the lumen of the respiratory bronchioles?

Answer 14

respiratory bronchiolitis

Question 15

there is an ↑ or ↓ ratio of CD4:CD8 T cell count;

Answer 15

↑ (almost 10:1) and these CD4 T cells accumulate in the lungs

Question 16

hypersensitivity pneumonia (extrinsic allergic alveolitis) is a type _____ HS reaction

Answer 16

type 3 but in chronic exposure can get granuloma formation and get type 4 HS

Question 17

what is a unique feature on histology of crytogenic organizing pneumonia

Answer 17

• alveolar architecture is NOT destroyed

Question 18

what is the first step of restrictive lung disease

Answer 18

“alveolitis: damage to pneumocytes and endothelial cells

Question 19

what would you expect to see on CXR of someone with sarcoidosis?

Answer 19

• bilateral hilar lymphadenopathy with or w/o parenchymal infiltrates

Question 20

organizing pneumonia can be seen on the histopathology slide of someone with ______

Answer 20

hypersensitivity pneumonia;

poorly formed NON necrotizing granulomas with giant cells

Question 21

cobblestoned pleural surface with honeycomb cysts is a description of what pulmonary disease?

Answer 21

IPF / UIP

idiopathic pulmonary fibrosis / usual interstitial fibrosis

Question 22

because sarcoidosis is a systemic disease, other than the lungs, what are some other organs that can be affected?

Answer 22

• skin: erythema nodosum
• eyes/lacrimal glands: sicca syndrome (sjogren syndrome where you have dry eyes and mouth)
• parotid glands: bilateral partotidis
• spleen
• liver
• bone marrow

Question 23

what are Masson bodies and what lung pathology would you expect to see them?

Answer 23

Masson bodies are polypoid plugs of loose fibroblastic tissue filling the alveolar spaces
seen in cryptogenic organizing pneumonia (bronchiolitis obliterans organizing pneumonia)

Question 24

respiratory bronchiolitis is associated with _______ fibrosis

Answer 24

peribronchiolar

Question 25

restrictive lung diseases are restricted to the ______-

Answer 25

lung parenchyma (interstitial)

Question 26

macrophages accumulate in the alveoli in ___________

Answer 26

desquamative interstitial pneumonia

Question 27

treatment method for organizing pneumonia

Answer 27

corticosteroids

Question 28

lymphoid interstitial pneumonia occurs in association with ____, ____ or _____

Answer 28

connective tissue disorders, autoimmune diseases or HIV infections

Question 29

honeycomb change is seen in _______ characterized by:

Answer 29

IPF/UIP;

cystic spaced lined by type II pneumocystis or respiratory epithelium (not conducive to gas exchange)

Question 30

the interstitial fibrosis in restrict lung diseases will ____ compliance and elasticity

Answer 30

↓ → ↓ lung expansion during inspiration

Question 31

of the restrictive lung diseases, which ones have granulomas?
both of these are immunological mediated response to antigen. Which type of HS reaction is each?

Answer 31

sarcoidosis (type 4 HS reaction) and hypersensitivity pneumonia (type 3 HS reaction but becomes type 4 HS with chronic exposure of the extrinsic antigen)

Question 32

temporal and geographic heterogeneity in histology is seen in ______ lung disae

Answer 32

IPF and UIP

• can see mature and young fibroblast foci
• some areas of the lung are spared

Question 33

sarcoidosis causes ____ lung disease

Answer 33

restrictive

Question 34

what are the two variants of NSIP?

Answer 34

• cellular variant (infiltrate of lymphocytes) has better prognosis
• fibrosing variant

Question 35

sarcoidosis can cause peripheral depletion of _____ and as a result can expect to see ____ on skin tests such as PPD

Answer 35

CD4 T cells (accumulate in intravelolar and interstitlum)

ANERGY

Question 36

↑/ ↓ serum ACE levels are seen in sarcoidosis

Answer 36

↑

Question 37

cystic spaces in UIP / IPF are lined by type ___ pneumocytes

Answer 37

type II pneumocytes

Question 38

sarcoidosis is characterized by __________ in many tissues and organs

Answer 38

NON necrotizing/ non caseating granulomas in young adults

Question 39

hyper/hypocalcemia is seen in sarcoidosis

Answer 39

HYPERcalcemia because the epithelioid cells activate vitamin D

Question 40

what are the interstitial lung diseases associated with cigarette smoking?

Answer 40

• respiratory bronchiolitis
• desquamative interstitial pneumonia
• pulmonary langerhans cell histiocytosis (eosinophilic granuloma)

Question 41

______ granulomas are seen in sarcoidosis in a ____ distribution

Answer 41

non necrotizing epithelioid granuloma in a lymphangitic distribution