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Respiratory system > Restrictive diseases > Flashcards

Restrictive diseases Flashcards

1
Q

What is the change that occurs in the interstitium in restrictive diseases?

A

Normally majority is elastic fibres, in the pathological interstitium the amount of elastic fibres is reduced, there is more collagen, this makes the interstitium less stretchable, lungs volume is reduced

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2
Q

What is the effect on the compliance and gas transfer?

A

The compliance and gas transfer are reduced, lower FEV1, FVC, but the ratio is normal the lung capacity is reduced but the respiration is not affected, expires the air that was inspired in, the diffusion is also reduced as there is bigger barrier, low Tco and Kco

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3
Q

What is the ned result of thickened interstitium?

A

ventilation/perfusion mismatch

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4
Q

What are the symptoms of interstitial lung diseases?

A

breathlessness on exertion, later in rest

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5
Q

What are the signs of interstitial lung diseases?

A

Abnormal chest x ray, respiratory failure type I

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6
Q

What is the acute response to parenchymal injury?

A

diffuse alveolar damage DADs or adult respiratory distress syndrome

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7
Q

What are the causes of DADs?

A

major trauma, chemical injury, circulatory shock, drugs, infection, autoimmune diseases, radiation, idiopathic

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8
Q

What is the pathology of DADs?

A

Accumulation of exudate with lots of protein (such as fibrin) and other macromolecules , epithelium is more leaky due to injury, formation of hyaline membrane starts (precipitation of the macromolecules), denuded membranes that lost layer, fibrosis follows, trying to repair proliferation takes place, increasing the bulk mass and thickening walls, fibroblasts also proliferates causing fibrosis

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9
Q

What is the results of DADs?

A

leads to respiratory failure, almost always lethal outcome

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10
Q

How many chronic responses are there and what are they?

A

3, granulomatous, usual interstitial pneumonitis, other patters

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11
Q

What is sarcoidosis?

A

Granulomatous disorder with unknown aetiology, affects multiple systems

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12
Q

What is the characteristic histology of sarcoidosis?

A

Little lymphoid infiltrate, variable fibrosis, epithelioid cells and giant cells granulomas, necrosis is very unusual, granuloma after located in scar tissue

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13
Q

What are the sites of sarcoidosis?

A

Common are lungs, lymph nodes, other spleen, liver, skins, eyes, muscles, salivary glands

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14
Q

What are the signs?

A

bilateral enlargement of hilar nodes, erythema nodosum, abnormal chest X ray, young adult with acute arthralgia, SOB, cough, anterior uveitis (red eye)

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15
Q

What is the treatment of sarcoidosis ?

A

Corticosteroids

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16
Q

What is Kveim test?

A

sample of a spleen from a patient with sarcoidosis is injected to a patient with suspected sarcoidosis, if non-caesating granulomas are found 4-6 weeks after the test is positive

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17
Q

What is hypersensitivity pneumonitis ?

A

Granulomatous disorder, inflammation in the centriacinar region of the lungs as a result to inhaled micromolecular organic antigens or organisms, body overreacts to them, can be acute or chronic

18
Q

What are examples of antigens that cause the hypersensitivity pneumonitis ?

A

thermophylic actinomycetes, birds or animal protein on faeces, aspergillum fungi, chemicals

19
Q

What types of hypersensitivity reactions are involved?

A

type III and IV

20
Q

What are the signs and symptoms of acute hypersensitivity pneumonitis?

A

fever, cough, myalgia, chills, crackles, tachypnoea, wheeze

21
Q

What are the signs and symptoms of chronic presentation?

A

Malaise, SOB, low grade illness, crackers, wheeze

22
Q

What is the histology of hypersensitivity pneumonitis

A

combination of granulomatous and lymphoid filtrate, so the fibrosis from the filtrate is more likely

23
Q

What is usual interstitial pneumonitis?

A

chronic interstitial disease, cryptogenic

24
Q

What are the causes of UIP?

A

connective tissue disorders -rheumatoid arthritis, exposure to some drugs, asbestos exposure, viral infection, other lung pathologies, idiopathic

25
Q

What is the histology of UIP?

A

Type 2 pneumocyte hyperplasia, proliferation of smooth muscle and vascular cells trying to repair, temporal and spatial heterogeneity, proliferating fibroblastic force

26
Q

What are the sings and symptoms of UIP?

A

cough, SOB, basal crackles, cyanosis, clubbing, on X ray cysts, diffuse infiltratesm posterior and basal fibrosis, scared honeycombed lung

27
Q

What is the outcome out UIP?

A

Respiratory failure does not respond to treatment, high death rate

28
Q

What are the other patterns?

A

nonspecific, asbestosis, silicosis, smoking related fibrosis, BOOP bronchiolitis obliterans organising pneumonia

29
Q

How is restriction defined?

A

Vital capacity less than 80%

30
Q

What are the lung causes of restrictive disease?

A

Sarcoidosis, hypersensitivity pneumonitis, idiopathic pulmonary fibrosis

31
Q

What are pleural causes of restrictive disease?

A

pleural effusion, pneumothorax, pleural thickening

32
Q

What are skeletal causes of restrictive disease?

A

kyphoscoloiosis, rib fracture, thoracoplasty, ankylosing spondylitis

33
Q

What are muscles causes of restrictive disease?

A

amyotrophic lateral sclerosis

34
Q

What are the sub-diaphragmatic causes?

A

obesity, pregnancy

35
Q

What are the investigations for sarcoidosis?

A

pulmonary function test, chest X ray, bloods, urinalysis, ECG, TB skin test, eye exam, also CT, mediastinoscopy, video assisted thoracoscopic biopsy

36
Q

What is the treatment for mild sarcoidosis ?

A

no major organs involvement, normal lung function - no treatment

37
Q

What is the treatment of sarcoidosis with erythema nodosum, arthralgia ?

A

NSAIDs

38
Q

What is the treatment for skin lesions, anterior uveitis, cough ?

A

topical steroids

39
Q

What is the treatment of cardiac, neurological, eye uveitis, hypercalcaemia?

A

systemic steroids

40
Q

What is the prognosis of sarcoidosis ?

A

Good unless some other somplications such as progressive fibrosis, pneumothorax …

Respiratory system (22 decks)

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