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Respi LE3 > Restrictive Pulmonary Diseases > Flashcards

Restrictive Pulmonary Diseases Flashcards

1
Q

What lung condition shows patchy interstitial fibrosis fibroblastic foci and honeycombing often associated with idiopathic pulmonary fibrosis?

A

Usual Interstitial Pneumonia (UIP)

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2
Q

What are clusters of proliferating fibroblasts seen in UIP indicating areas of active fibrosis?

A

Fibroblastic Foci

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3
Q

Which term describes the cystic lung appearance in advanced fibrosis with thick fibrous walls?

A

Honeycomb Lung

In idiopathic pulmonary fibrosis and granulomatous hypersensitivity pneumonitis

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4
Q

What condition refers to large blackened lung scars seen in advanced stages of coal workers’ pneumoconiosis?

A

Progressive Massive Fibrosis (PMF)

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5
Q

What occupational lung disease results from inhaling crystalline silica dust leading to nodule formation?

A

Silicosis

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6
Q

What are rod-shaped structures coated with iron-containing material found in asbestos-exposed lungs?

A

Asbestos Bodies

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7
Q

Which aggressive cancer is primarily caused by asbestos exposure affecting the mesothelial lining of the lungs or peritoneum?

A

Mesothelioma

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8
Q

What are immune cell clusters found in sarcoidosis that lack central necrosis?

A

Noncaseating Granulomas

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9
Q

What is the lung condition caused by an immune reaction to inhaled organic particles like mold or bird droppings?

A

Hypersensitivity Pneumonitis

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10
Q

What condition involves an excess of eosinophils in the lungs linked to asthma or drug reactions?

A

Pulmonary Eosinophilia

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11
Q

What are pigment-laden macrophages found in the lungs of smokers associated with smoking-related diseases?

A

Smokers’ Macrophages

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12
Q

What rare disease involves the accumulation of surfactant in the alveoli due to genetic or autoimmune factors?

A

Pulmonary Alveolar Proteinosis (PAP)

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13
Q

Which protein is critical for alveolar macrophage function and is linked to PAP when defective?

A

Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF)

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14
Q

What lung condition is characterized by reduced lung compliance and reduced FVC with a normal FEV1/FVC ratio?

A

Restrictive Lung Diseases

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15
Q

Which disease is a clinicopathologic syndrome associated with UIP and characterized by patchy interstitial fibrosis?

A

Idiopathic Pulmonary Fibrosis (IPF)

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16
Q

What pattern of fibrosis is seen in IPF characterized by fibroblastic foci and honeycomb lung?

A

Usual Interstitial Pneumonia (UIP)

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17
Q

What environmental factors contribute to alveolar epithelial cell injury in IPF?

A

Smoking air pollution occupational exposures

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18
Q

What genetic mutations increase the risk of developing IPF?

A

Mutations in TERT TERC PARN RTEL1 and surfactant genes

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19
Q

At what age is IPF most likely to develop?

A

After the age of 50

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20
Q

Which protein plays a key role in driving the fibrotic process in IPF by promoting fibroblast proliferation?

A

TGF-β1

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21
Q

What is decreased in IPF that normally inhibits fibrosis and limits TGF-β1 activity?

A

Caveolin-1

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22
Q

What is the typical gross appearance of the lungs in IPF particularly in the lower lobes?

A

Cobblestoned pleural surfaces firm rubbery white fibrosis

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23
Q

What is the typical microscopic feature of IPF with varying degrees of intensity?

A

Patchy interstitial fibrosis with fibroblastic foci

24
Q

How long is the median survival after diagnosis of IPF?

A

About 3.8 years

25
What is the NIP pattern that differs from UIP in terms of temporal heterogeneity?
**Fibrosing pattern** without honeycombing or fibroblastic foci
26
Which pattern of NIP features mild to moderate chronic interstitial inflammation?
Cellular pattern
27
Where in the lungs is the typical imaging appearance of **NIP** characterized by **bilateral symmetric reticular opacities?**
Predominantly in the lower lobes
28
What disease is characterized by the presence of Masson bodies within the alveolar ducts alveoli and bronchioles?
Cryptogenic Organizing Pneumonia (COP)
29
What is the hallmark feature seen in the lungs of patients with COP?
Masson bodies (polypoid plugs of loose connective tissue)
30
What chronic fibrosing lung disease results from inhaling coal dust?
Coal Workers' Pneumoconiosis (CWP)
31
What is the accumulation of carbon particles within macrophages in the lungs and lymph nodes in CWP?
Anthracosis
32
What are the coal macules and coal nodules in **simple CWP** typically found in the **upper lobes?**
Coal macules (carbon-laden macrophages) coal nodules (collagen fibers)
33
What advanced form of CWP is marked by the development of large blackened fibrotic scars?
Progressive Massive Fibrosis (PMF)
34
What is the fibrogenic occupational hazard caused by the inhalation of silica dust?
Silicosis
35
Which form of silica is more fibrogenic and can lead to silicosis?
Crystalline silica (quartz cristobalite tridymite)
36
What inflammatory response is triggered by phagocytosis of silica crystals?
Activation of the inflammasome release of IL-1, TNF, and fibrogenic cytokines
37
What is the appearance of silicosis in early stages in the upper zones of the lungs?
Pale to blackened nodules
38
What microscopic feature is used to identify silica particles in nodules in silicosis?
Birefringent silica particles under polarized microscopy
39
What is the term for diseases caused by asbestos exposure including asbestosis pleural plaques and mesothelioma?
Asbestos-Related Diseases
40
What type of asbestos fibers are more pathogenic in causing mesothelioma?
Amphibole asbestos fibers
41
What disease is characterized by **diffuse pulmonary interstitial fibrosis** and the presence of **asbestos bodies**?
Asbestosis
42
What is the hallmark appearance of asbestos bodies in the lungs?
Golden brown rod-shaped structures coated with an iron-containing protein
43
Where does fibrosis typically begin in asbestosis?
In the lower lobes and subpleurally
44
What is the most common manifestation of asbestos exposure characterized by dense collagen plaques?
Pleural plaques
45
What is the hallmark feature of sarcoidosis with respect to tissue involvement?
**Noncaseating granulomas** in various tissues including the lungs
46
What immune cells accumulate in sarcoidosis leading to granuloma formation?
CD4+ T cells
47
What condition involves an exaggerated immune response to inhaled organic antigens such as fungi and animal proteins?
Hypersensitivity Pneumonitis
48
What is a key histological feature of hypersensitivity pneumonitis?
Interstitial pneumonitis with noncaseating granulomas
49
What is the term for a condition involving increased eosinophils in the lungs potentially due to parasitic infections or drug allergies?
Pulmonary Eosinophilia
50
What interstitial lung disease is associated with smokers' macrophages containing brown pigment from tobacco smoke?
Desquamative Interstitial Pneumonia (DIP)
51
What type of lung disease is associated with **pigmented macrophages** in the respiratory bronchioles and **peribronchiolar fibrosis**?
Respiratory Bronchiolitis–Associated Interstitial Lung Disease
52
What rare disease involves the accumulation of surfactant in the alveoli caused by defects in GM-CSF?
Pulmonary Alveolar Proteinosis (PAP)
53
What is the autoimmune cause of Acquired PAP?
Antibodies against GM-CSF
54
What rare form of PAP is caused by mutations in genes encoding GM-CSF or its receptor?
Hereditary PAP
55
What form of PAP is associated with various underlying conditions affecting GM-CSF signaling or macrophage function?
Secondary PAP

Respi LE3 (9 decks)

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