Restrictive (Interstitial) Lung Diseases Flashcards

1
Q

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

A
  • Schaumann bodies: laminated concretions of calcium and protein
  • Asteroid bodies: stellate inclusion in giant cells
    THESE ARE NOT PATHOGNOMONIC
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2
Q

like IPF, sarcoidosis is a diagnosed by ______

A

exclusion

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3
Q

what are 4 features of restrictive lung diseases?

A
  • fibrosis
  • dyspnea
  • V/Q mismatch
  • progression to respiratory failure with pulmonary HTN and cor pulmonale
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4
Q

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

A

NSIP (non specific interstitial pneumonia)

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5
Q

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

A

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

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6
Q

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

A

acute interstitial pneumonia

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7
Q

what are the 4 major categories of restrictive lung diseases

A
  • 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 )
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8
Q

organizing pneumonia can be seen following: (3)

A
  • infectious pneumonia
  • transplantation (lung and bone marrow)
  • collagen vascular diseases
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9
Q

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

A

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

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10
Q

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

A

there is NO HONEYCOMB appearance with non specific interstitial pneumonia

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11
Q

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

A

type 4 (driven by CD4 helper T cell)

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12
Q

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

A

subepithelial

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13
Q

the main feature of restrictive lung diseases is _____

A

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

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14
Q

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

A

respiratory bronchiolitis

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15
Q

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

A

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

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16
Q

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

A

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

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17
Q

what is a unique feature on histology of crytogenic organizing pneumonia

A
  • alveolar architecture is NOT destroyed
18
Q

what is the first step of restrictive lung disease

A

“alveolitis: damage to pneumocytes and endothelial cells

19
Q

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

A
  • bilateral hilar lymphadenopathy with or w/o parenchymal infiltrates
20
Q

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

A

hypersensitivity pneumonia;

poorly formed NON necrotizing granulomas with giant cells

21
Q

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

A

IPF / UIP

idiopathic pulmonary fibrosis / usual interstitial fibrosis

22
Q

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

A
  • 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
23
Q

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

A

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

24
Q

respiratory bronchiolitis is associated with _______ fibrosis

A

peribronchiolar

25
restrictive lung diseases are restricted to the ______-
lung parenchyma (interstitial)
26
macrophages accumulate in the alveoli in ___________
desquamative interstitial pneumonia
27
treatment method for organizing pneumonia
corticosteroids
28
lymphoid interstitial pneumonia occurs in association with ____, ____ or _____
connective tissue disorders, autoimmune diseases or HIV infections
29
honeycomb change is seen in _______ characterized by:
IPF/UIP; | cystic spaced lined by type II pneumocystis or respiratory epithelium (not conducive to gas exchange)
30
the interstitial fibrosis in restrict lung diseases will ____ compliance and elasticity
↓ → ↓ lung expansion during inspiration
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?
sarcoidosis (type 4 HS reaction) and hypersensitivity pneumonia (type 3 HS reaction but becomes type 4 HS with chronic exposure of the extrinsic antigen)
32
temporal and geographic heterogeneity in histology is seen in ______ lung disae
IPF and UIP - can see mature and young fibroblast foci - some areas of the lung are spared
33
sarcoidosis causes ____ lung disease
restrictive
34
what are the two variants of NSIP?
- cellular variant (infiltrate of lymphocytes) has better prognosis - fibrosing variant
35
sarcoidosis can cause peripheral depletion of _____ and as a result can expect to see ____ on skin tests such as PPD
CD4 T cells (accumulate in intravelolar and interstitlum) ANERGY
36
↑/ ↓ serum ACE levels are seen in sarcoidosis
37
cystic spaces in UIP / IPF are lined by type ___ pneumocytes
type II pneumocytes
38
sarcoidosis is characterized by __________ in many tissues and organs
NON necrotizing/ non caseating granulomas in young adults
39
hyper/hypocalcemia is seen in sarcoidosis
HYPERcalcemia because the epithelioid cells activate vitamin D
40
what are the interstitial lung diseases associated with cigarette smoking?
- respiratory bronchiolitis - desquamative interstitial pneumonia - pulmonary langerhans cell histiocytosis (eosinophilic granuloma)
41
______ granulomas are seen in sarcoidosis in a ____ distribution
non necrotizing epithelioid granuloma in a lymphangitic distribution