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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
Q

What is the NIP pattern that differs from UIP in terms of temporal heterogeneity?

A

Fibrosing pattern
without honeycombing or fibroblastic foci

26
Q

Which pattern of NIP features mild to moderate chronic interstitial inflammation?

A

Cellular pattern

27
Q

Where in the lungs is the typical imaging appearance of NIP characterized by bilateral symmetric reticular opacities?

A

Predominantly in the lower lobes

28
Q

What disease is characterized by the presence of Masson bodies within the alveolar ducts alveoli and bronchioles?

A

Cryptogenic Organizing Pneumonia (COP)

29
Q

What is the hallmark feature seen in the lungs of patients with COP?

A

Masson bodies (polypoid plugs of loose connective tissue)

30
Q

What chronic fibrosing lung disease results from inhaling coal dust?

A

Coal Workers’ Pneumoconiosis (CWP)

31
Q

What is the accumulation of carbon particles within macrophages in the lungs and lymph nodes in CWP?

A

Anthracosis

32
Q

What are the coal macules and coal nodules in simple CWP typically found in the upper lobes?

A

Coal macules (carbon-laden macrophages)
coal nodules (collagen fibers)

33
Q

What advanced form of CWP is marked by the development of large blackened fibrotic scars?

A

Progressive Massive Fibrosis (PMF)

34
Q

What is the fibrogenic occupational hazard caused by the inhalation of silica dust?

A

Silicosis

35
Q

Which form of silica is more fibrogenic and can lead to silicosis?

A

Crystalline silica (quartz cristobalite tridymite)

36
Q

What inflammatory response is triggered by phagocytosis of silica crystals?

A

Activation of the inflammasome
release of IL-1, TNF, and fibrogenic cytokines

37
Q

What is the appearance of silicosis in early stages in the upper zones of the lungs?

A

Pale to blackened nodules

38
Q

What microscopic feature is used to identify silica particles in nodules in silicosis?

A

Birefringent silica particles under polarized microscopy

39
Q

What is the term for diseases caused by asbestos exposure including asbestosis pleural plaques and mesothelioma?

A

Asbestos-Related Diseases

40
Q

What type of asbestos fibers are more pathogenic in causing mesothelioma?

A

Amphibole asbestos fibers

41
Q

What disease is characterized by diffuse pulmonary interstitial fibrosis and the presence of asbestos bodies?

A

Asbestosis

42
Q

What is the hallmark appearance of asbestos bodies in the lungs?

A

Golden brown rod-shaped structures coated with an iron-containing protein

43
Q

Where does fibrosis typically begin in asbestosis?

A

In the lower lobes and subpleurally

44
Q

What is the most common manifestation of asbestos exposure characterized by dense collagen plaques?

A

Pleural plaques

45
Q

What is the hallmark feature of sarcoidosis with respect to tissue involvement?

A

Noncaseating granulomas in various tissues including the lungs

46
Q

What immune cells accumulate in sarcoidosis leading to granuloma formation?

A

CD4+ T cells

47
Q

What condition involves an exaggerated immune response to inhaled organic antigens such as fungi and animal proteins?

A

Hypersensitivity Pneumonitis

48
Q

What is a key histological feature of hypersensitivity pneumonitis?

A

Interstitial pneumonitis with noncaseating granulomas

49
Q

What is the term for a condition involving increased eosinophils in the lungs potentially due to parasitic infections or drug allergies?

A

Pulmonary Eosinophilia

50
Q

What interstitial lung disease is associated with smokers’ macrophages containing brown pigment from tobacco smoke?

A

Desquamative Interstitial Pneumonia (DIP)

51
Q

What type of lung disease is associated with pigmented macrophages in the respiratory bronchioles and peribronchiolar fibrosis?

A

Respiratory Bronchiolitis–Associated Interstitial Lung Disease

52
Q

What rare disease involves the accumulation of surfactant in the alveoli caused by defects in GM-CSF?

A

Pulmonary Alveolar Proteinosis (PAP)

53
Q

What is the autoimmune cause of Acquired PAP?

A

Antibodies against GM-CSF

54
Q

What rare form of PAP is caused by mutations in genes encoding GM-CSF or its receptor?

A

Hereditary PAP

55
Q

What form of PAP is associated with various underlying conditions affecting GM-CSF signaling or macrophage function?

A

Secondary PAP

Respi LE3 (9 decks)

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