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Respiratory > Interstitial lung disease > Flashcards

Interstitial lung disease Flashcards

1
Q

Three synonyms for interstitial lung disease

A
  • Infiltrative lung disease -Infiltration of cellular and non-cellular elements within alveolar septa and alveoli.
  • Diffuse parenchymal lung disease (DPLD)
  • Restrictive lung disease – Characterized by reduced total lung capacity in presence of a normal or reduced expiratory flow rate.
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2
Q

Infiltration of cellular and non-cellular
elements within alveolar septa and alveoli.

A

Infiltrative lung disease

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3
Q

Characterized by reduced total lung
capacity in presence of a normal or reduced expiratory flow rate

A

Restrictive lung disease

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4
Q

– Fibrosing lung disorders (pneumoconioses)
– Granulomatous disorders (sarcoidosis)
– Idiopathic interstitial pneumonias (IIPs)

these are all examples of

A

Chronic interstitial lung disease

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5
Q

“_______” – Damage to pneumocytes and
endothelial cells

A

Alveolitis

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6
Q

Pathogenesis of ILDS: Leads to leukocytes releasing cytokines which mediate and stimulate

A

interstitial fibrosis

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7
Q

Interstitial fibrosis –
–↓es lung _____ and _____
• ↓ed lung expansion during inspiration

A

compliance and elasticity

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8
Q

What are our modulating factors for patholgenesis of Interstitial lung disorders?

A

chronicity of lung exposure, effectiveness of lung defenses, extent of injury, intactness of basement membrane, individual susceptibility

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9
Q

What are clinical findings of interstitila lung disease?

what do you see on xray?

A

• Dry cough and dyspnea
• Late inspiratory crackles, bibasilar (Velcro
crackles)
• Cor pulmonale

-bilateral reticulonodular infiltrates

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10
Q

Non-neoplastic lung diseases in response
to inhalation of mineral dusts inhaled in the
workplace.

A

Pneumoconioses

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11
Q

include diseases induced by organic and inorganic particulate matter/chemical fumes /vapors.
• Coal dust, silica, asbestos, beryllium
• 25% cases of chronic interstitial lung disease

A

Pneumoconioses

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12
Q

Devo of pneumoconiosis depends on:

A

amount of dust retained in parenchyma and airways

size/shape/buoyace of particle

solubility of particle and physiochemical reactivity

possible additional effects of other irritants

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13
Q

1 – 5 μm particles will – reach bifurcation of
– < 0.5 μm – reach alveoli and are

A

respiratory bronchioles and alveolar ducts

phagocytosed by alveolar macrophages

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14
Q

Coals workers pneumocosis, silicosis, silicatosis, asbestosis are alls forms of

A

fibrogenic pnemoconiosis

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15
Q

Anthracotic pigment – coal mines, urban centers, tobacco smoke
Pulmonary anthracosis
Simple
Complicated

all examples of:

A

Coal workers pneumoconiosis = CWP

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16
Q

How does anthracosis present?

A

asmptomatic and anthracotic pigment in interstitial compartment and lymph nodes

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17
Q

Simple coal workers’ pneumoconiosis
• Fibrous opacities _____
•are seen in which lobes?
• Characterized by coal dust deposits adjacent
to _______

A

< 1 cm

Upper lobes and upper portions of lower lobes

respiratory bronchioles

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18
Q

Complicated coal workers’ pneumoconiosis
(progressive massive fibrosis)
• Fibrous opacities ____
• _________ central necrosis
• Massive fibrosis –

A

> 1 cm

With or without

crippling lung disease (Black
lung disease)

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19
Q

What other issues do we see with complicated coal workers pneumoconiosis?

A

cor pulmonale

see w/ Caplan syndrome: CWP with rheumatoid nodules in lung

NO increased incidence of TB or Cancer

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20
Q

• Most common occupational disease worldwide
• Crystalline silicon dioxide (quartz)
– Foundries (metal casting), sandblasting, silica mines

A

Silicosis

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21
Q

Pathogenesis of silicosis

A

• Quartz activates alveolar macrophages after engulfment → cytokine release → fibrogenesis

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22
Q

Describe what you see on HE and grossly due to chronic exposure to quartz leading to silicosis

A

Gross we see Polarizable quartz particles and “Egg-shell” calcification in hilar lymph nodes

HE: Nodular opacities with concentric layers of collagen

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23
Q

What are complications of silicosis?

A

cor pulmonale; association with Caplan
syndrome with increased risk for TB (silicotuberculosis) and cancer

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24
Q

Two Asbestos forms
–______ (e.g. – chrysotile) – curly and flexible
–______ (e.g. – crocidolite) – straight and rigid

A

Serpentine

Amphibole

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25
Q

deposition sites of asbestos:

Source of asbestos:

A

– respiratory bronchioles, alveolar ducts and alveoli

Sources:Insulation around pipes in old naval ship, Roofing material used over 20 years ago, Demolition of old buildings

26
Q

What are ferruginous bodies?

A

macrophages phagocytose asbestos fibers and
coat them with ferritin (iron and protein

27
Q

What are our asbestos releated diseases?

A

Benign pleural plaques (not precursor of mesothelioma)

diffuse interstitial fibrosis, bronchogenic carcinoma, mosothelioma, NO incraesed risk for TB, cor pulmonale and Caplan syndrome

28
Q

What do we see on HE and gross for asbestos?

A

increase in collagen, much more thickened

Gross you will see pleural plaques–little nobs all over

29
Q

Berylliosis

  • Beryllium – where from?
  • _______ inflammation – TB and sarcoidosis (differential diagnosis)
  • Complications – _____ and _____
A

Nuclear and airspace industry

Granulomatous

cor pulmonale and lung cancer

30
Q

• Multisystem granulomatous disease of unknown
etiology
• 25% cases of chronic interstitial lung disease

A

Sarcoidosis

31
Q

Where and what population do we see infected with sarcoidosis?

A

highest in scandanavia, AA, F, and 70% less then 40 years old

**non-smokers

32
Q

Sarcoidosis

• Disorder of immune regulation
• Unknown antigen → Interaction with ______
cells → cytokine release → recruitment of
_________ → non-necrotizing granuloma formation

A

CD4 TH

monocytes/histiocytes

33
Q

Sarcoidosis is a diagnosis of

A

exclusion

34
Q

What systems are involved in sarcoidosis?

A

Skin; nodular granulomatosis

Eyes

Liver; granulomatous hepatitis

salivary and lacrimal glands, diabetes insipidus, bone marrow and splenic invovlement

35
Q

Sarcoidosis

↑ed ________ levels as a marker of disease activity and response to steroids

A

angiotensin converting enzyme (ACE)

36
Q

– Hypercalcemia (5% cases)
– Polyclonal gammopathy
– Cutaneous anergy (lack of response to common skin antigens (candida) due to consumption of CD4 THcells

A

Sarcoidosis

37
Q

What do you see on chest xray in sarcoidosis

A

bilateral hilar adenopathy adn reiculonodular shadows in lungs

38
Q

On HE of lung with sarcoidosis, what do you see?

A

interstitial granulomas

has a lymphatic flow distinct pattern in the interstitium and see they are around vascular bundles

39
Q

In this bronchiole is it surrounded by a granuloma full of ______ .

Characteristic of what disease?

A

histocytes

sarcoidosis

40
Q

What kind of cell is in the picture?

A

Langhans giant cell, seen in sarcoidosis

41
Q

Prognosis of sarcoidosis

A

do fairly well, you tx with steroids and pts will respond

See variable responses from spontaneous remission and relapse to progressie interstitial fibrosis and cor pulmonale and death in 10-15%

42
Q

– Farmers’ lung
– Silo fillers’ disease
– Byssinosis
are all examples of:

A

Hypesensitivity pneumonitis

43
Q 
Inhaled antigen (known or unknown) producing 
granulomatous interstitial pneumonitis (extrinsic 
allergic alveolitis)
A

Hypersensitivity pneumonitis

44
Q

Hypersensitivity pneumonitis:

Type ____ hypersensitivity reaction

First exposures we see _____ in serum

Second exposure we see:

A

Type III hypersensitivity rxn

First exposure: IgG antibodies in serum

Second exposure: antibodies combine w/ inhaled antiG to form inmume complexes–> inflammatory response in lung

45
Q

What happens during the chonic phase of hypersensitivity pneumonitis

A

Granuloma fomration (Type IV hypersentivitiy response)

46
Q

Causes of farmers lung;

Silo fillers disease

Byssinosis

A

farmers lung = moldy hay- thermophilic actinomycetes bacteria

silo fillers = inhalation of gases from plant material (oxides of nitrogen)

Byssinosis = cotton/linen/hemp and textile factory workers and monday morning blues

47
Q

Interstitial and alveolar infiltrates of inflammatory
cells, peri-bronchiolar accentuation, ill-defined
granulomas

A

Interstitial lung disease: HSP

48
Q

How do you diagnose Interstitial lung disease, specifically HSP?

A

Clinico-pathologic diagnosis – symptoms and physical findings, x-ray abnormalities, PFTs,
immunologic features (Abs to suspected Ags); lung
biopsy may be needed

49
Q

What do we see on HE for interstitial lung disease

A

Granuloma, but not as well circumscribed as sarcoidosis

50
Q

___of chronic interstitial lung disease are idiopathic and usually ______pneumonias are idiopathic

A

15%

usual interstitial pneumonia is idopathic

51
Q

Idiopathic pulmonary fibrosis is more:

male or female

occurs at what age

duration of symptoms

A

M>F

40-70 yrs

duration of syptoms 18-24 months

52
Q

Clinical manifestations of idiopathc pulmonary fibrosis:

A

– Dyspnea; non-specific constitutional symptoms
such as fever, weight loss, fatigue, arthralgias; cough

53
Q

Pulmonary fibrosis

• Repeated injury to the lung → alveolitis → cytokine release → _________

A

interstitial fibrosis

54
Q

Idiopathic pulmonary fibrosis:

Interstitial fibrosis → irregular dilatation of adjacent airways →

A

honeycomb lung (end stage interstitial fibrosis)

55
Q

Honeycomb cysts are typically seen in:

A

Idiopathic pulmonary fibrosis

56
Q

Course of idiopathic pulmonary fibrosis

A

progressive disease–end-stage lung; cor pulmonale

57
Q

– Interstitial lung disease in 50% of patients
– Wide spectrum of pulmonary changes; common manifestation – pleural effusion (unexplained pleural effusion in a young woman likely to be this).

A

Systemic lupus erythematosus (SLE)

58
Q

Wide spectrum of pulmonary changes
• Rheumatoid nodules (when associated with pneumoconiosis – Caplan syndrome)
• Interstitial fibrosis
• Pleural effusions

A

Rheumatoid arthritis (RA)

59
Q

– Interstitial fibrosis with pulmonary vascular hypertrophy
– Commonest cause of death

A

Systemic sclerosis (scleroderma)

60
Q

A 66-year-old man has had increasing dyspnea
for about a year. He is a smoker. He is retired
from the construction business. There are
some rales auscultated in both lungs on
physical examination. A chest radiograph
reveals bilateral diaphragmatic pleural plaques
as well as diffuse interstitial lung disease. A
sputum cytology shows no atypical cells. These findings are most likely to suggest prior exposure to which environmental agents?

A

Asbestos fibers

61
Q

A 50-year-old African American woman presents
with increasing shortness of breath, fever, weight
loss, and night sweats for the past 4 months. She
is a non-smoker. On examination, there are fine
rales auscultated in all lung fields. A chest
radiograph reveals hilar lymphadenopathy and a
reticulonodular pattern in all lung fields. A
transbronchial biopsy is performed and
microscopically demonstrates numerous small
pulmonary interstitial non-caseating granulomas. Which of the following is most likely?

A

Sarcoidosis

Respiratory (15 decks)

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