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Respiratory/Musculoskeletal > CM- Restrictive Lung Diseases > Flashcards

CM- Restrictive Lung Diseases Flashcards

1
Q

Describe the PFT findings for restrictive lung disease.

A

FEV1/FVC ratio is preserved because airway resistance is not markedly elevated and expiratory flow rates are maintained.
TLC is reduced- less air is held by the lungs so the FEV1 and FVC are both decreased (but proportionately

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2
Q

What happens to the following in restrictive lung disease:

  1. vital capacity
  2. FEV1
  3. FEV1/FVC
  4. FEF 25%-75%
  5. TLC
A
  1. decreased
  2. decreased
  3. normal (>80%)
  4. normal
  5. reduced
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3
Q

What happens to the following in obstructive lung disease?

  1. FVC
  2. FEV1
  3. FEV1/FVC
  4. FEF 25%-75%
  5. TLC
A
  1. decreased
  2. GREATLY decreased
  3. decreased (<80%)
  4. decreased
  5. increased
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4
Q

In order to confirm lung volume reduction (for restrictive disorders), what tests must be performed?

A

He dilution technique or

Plethysmography

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5
Q

What are the 5 major causes of restriction?

A
  1. Pleural disease (effusion, mesothelioma, fibrosis)
  2. Alveolar (hemorrhage, pulmonary edema, pneumonia, eosonphilic pneumonia, alveolar proteinosis)
  3. Interstitial (IPF, sarcoidosis, hypersensitivity pneumonitis)
  4. Neuromuscular (ALS, myasthenia, botulism)
  5. Thoracic cage (obesity, kyphoscoliosis, pregnancy, ascites)
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6
Q

What are the 5 major types of ILD?

A
  1. idiopathic interstitial pneumonia
  2. systemic disorders
  3. occupational/environmental
  4. connective tissue diseases
  5. drug-induced lung disease
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7
Q

What are the 2 major histological classifications for ILD?

A
  1. granulomatous reaction - sarcoidosis, hypersensitivity pneumonitis, berylliosis (environmental/occupational, systemic)
  2. inflammatory/fibrotic changes
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8
Q

What are the 2 most common ILD?

A
  1. Sarcoidosis- systemic

2. Interstitial Pulmonary Fibrosis (IPF)

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9
Q

Describe normal lung interstitium.

what is it composed of, thin/thick, cellular/acellular, etc

A

Thin, relatively acellular space made of:

  1. collagen (2:1 Type I:Type III)
  2. elastin
  3. proteogylcans
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10
Q

How does ILD change the composition of normal lung interstitium?
What are the main physiological results?

A
  1. infiltration of inflammatory cells
  2. deposition of excess collagen

This thickens the interstitium leading to:

  1. decreased compliance–> increased work of breathing, reduced lung volume
  2. Gas exchange is impaired due to loss of alveolar-capillary units–> VQ mismatch, hypoxemia
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11
Q

What is usually the primary complaint that patients with ILD present with?

A

Dyspnea on exertion WITHOUT:

  1. sputum
  2. wheezing
  3. orthopnea
  4. paroxsymal attacks
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12
Q

When a patient presents with:

  • dyspnea on exertion
  • no sputum, wheezing, othopnea, paroxysmal attacks
  • fever

What are the diagnoses we want to consider?

A

Infectious disease or sarcoidosis

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13
Q

A patient presents with dyspnea on exertion, crackles, clubbing and cyanosis.
What is a likely diagnosis?

A

ILD

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14
Q

A patient presents with dyspnea, tachycardia, chest pain, leg swelling, and syncope. What is the most likely cause?

A

PE

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15
Q

A patient presents with dyspnea, S3 gallop, bibasilar inspiratory crackles, paroxysmal nocturnal dyspnea, orthopnea. What is the most likely cause?

A

CHF

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16
Q

A patient presents with skeletal muscle weakness and dyspnea. What is the most likely cause?

A

Neuromuscular disorder (ALS, myasthenia, botulism)

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17
Q

A patient presents with dyspnea, pale conjunctiva, and tachycardia. What is the most likely cause?

A

Anemia

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18
Q

What are the 3 most prominent findings associated with ILD on physical exam?

A
  1. Crackles- late inspiratory, by sudden explosive opening of previously collapses airways
  2. Digital clubbing- esp. with IPF and caused by connective tissue proliferation in the distal phalanx
  3. Cyanosis- rapid shallow breathing, very obvious during exercise with RR >30bpm
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19
Q

What are lab findings suggestive of IPF and connective tissue disorders?

A

positive serological tests for ANA or rheumatoid factor

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20
Q

What does ABG data show for patients with ILD?

A
  1. pCO2 is normal
  2. paO2 is decreased
  3. A-a gradient is increased
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21
Q

If you suspect ILD, what is the first test you should perform?

A

Measure PaO2 during exercise.

Exercise hypoxia is a more sensitive test than spirometry early in the course of the disease

22
Q

DLCO measures the gas exchange capacity of the lung. Diseases which decrease the number of __________ or ____________ will lower the DLCO.

A
  1. functional alveoli

2. pulmonary capillaries

23
Q

Why don’t diseases like bronchitis and asthma change the DLCO?

A

They only affect conducting airways. To lower the DLCO, there needs to be reduced number of alveoli or reduced pulmonary capillaries

24
Q

What are the 6 diseases that affect DLCO?

A
  1. ILD
  2. emphysema
  3. pulmonary emboli
  4. pulmonary hypertension
  5. lung resection
  6. anemia
25
Q

What are the typical ILD CXR findings?

Do they correlate with the severity of the disease?

A
  1. reduced lung volume
  2. diffuse reticulo-nodular markings

No- they correlate poorly with symptoms, physiologic abnormalities, and histology

26
Q

You are looking at a chest X-ray and see hilar lymph node enlargement. What is the likely cause?

A

Sarcoidosis

27
Q

You are looking at a chest X-ray and see small miliary nodules. What is your suspision?

A

TB

28
Q

You are looking at a CXR and see upper lobe predominance. What is the suspicion?

A

silicosis

29
Q

You are looking at a CXR and see pleural plaques and calcifications. What is the most possible disease?

A

Asbestosis

30
Q

What is seen in early ILD on HRCT (high resolution CT)? What does this suggest? Is it reversible?

A

Ground glass appearance- suggest alveolitis which can respond to inflammatory drug therapy and is reversible

31
Q

What is seen in late ILD on HRCT? What does this suggest? Is it reversible?

A

You see harsh, coarse, linear white markings and “honeycombing” suggestive of fibrosis. This is irreversible.

32
Q

What drugs would make you consider a drug-induced ILD?

A

Chemo drugs- bleomycin, busulfan, cyclophosphamide, methtrexate, nitroureas

Cardiac drugs- amiodarone, procainamide, hydralazine

Antibiotics- nitrofurantoin, isonaizid

Illicit- heroin, cocaine

33
Q

How would you be able to diagnose ILD associated with connective tissue disorders?
What are some of the disorders?

A

ILD usually occurs in the setting of long-standing disease so it is generally not the initial presentation. It may also present with pleural involvement (serositis, effusion)

  1. SLE
  2. RA
  3. CREST
  4. Sjogrens
  5. dermatomyositis/polymyositis
34
Q

A 20-40 year old African American presents to you with non-productive cough, dyspnea, and occassional fever. You do histology and see non-caseating granulomas in multiple organs (skin, lymph nodes, eyes, CNS, bone). CXR shows hilar lymph node enlargement. What are you suspicious of?

A

Sarcoidosis

35
Q

How are the non-caseating granulomas associated with sarcoidosis formed?

A

TH1 cytokines (IL12, TNF, IL2) are secreted by alveolar macrophages and T cells

36
Q

How is sarcoidosis diagnosed?

A
  1. compatible clinical picture
  2. CXR with enlarged hilar lymph nodes
  3. non-caseating granulomas on biopsy
37
Q

What would blood tests of someone with sarcoidosis show?

A
  1. polyclonal hypergammaglobulinemia
  2. anergy to specific antigens
  3. hypercalcemia
38
Q

What is the difference between hypersensitivity pneumonitis and pneumoconiosis?

A

Hypersensitivity pneumonitis - organic materials from fungi, bacteria, animals, plants. GRANULOMAS
Pneumoconiosis- mineral dusts, NO GRANULOMAS

39
Q

What are the diff diagnoses for hypersensitivity pneumonitis?

A

pneumonia (acutely)

ILD (chronically)

40
Q

What would you see on histology of the lung for hypersensitivity pneumonitis?
How can it be differentiated from sarcoidosis?

A

You would see granulomas but they will ONLY be in the lung vs. multiple systems

41
Q

What are the 4 most common causes of pneumoconioses?

A
  1. Asbestos
  2. Beryllium
  3. Talc
  4. silica
42
Q

A 55 year old man that worked as a builder comes to you with dyspnea. You take xrays and note pleural plaques. What is your suspicion?
What other features are you going to see in his lungs?

A

Asbestosis- the fibers penetrate the visceral pleura and cause effusion, pleural thickening, and mesothelioma in addition to pulm. fibrosis

43
Q

Which pneumoconiosis mimics sarcoidosis?

A

Berylliosis

44
Q

What is the presentation of someone with idiopathic pulmonary fibrosis (clinical symptoms, physiology, histology)?

A

Chronic progressive onset of dyspnea and cough, restrictive lung physiology, and histopathologic pattern of UIP (usual interstitial pneumonia)

45
Q

Why is it important to distinguish IPF from the other ILDs?

A

IPF is more lethal (50% die in 3 years)

Other ILDs can be treated with corticosteroids and immunosuppressants to improve prognosis.

46
Q

It was previously believed that IPF was the common final pathway of the other ILDs. What is the current theory?

A

IPF is not characterized by inflammation, but rather fibroproliferation due to abnormal lung tissue healing

47
Q

How is the diagnosis of IPF made?

A

chronic, progressive fibrosing interstitial pneumonia with the other causes of ILD ruled out.
Mostly in people older than 50 (usually white, male smokers)

48
Q

Describe the presentation of someone with familial IPF.
What is the incidence?
How is it inherited?

A

It is 0.5% to 3.5% of all cases of IPF characterized by early onset of disease.
It is autosomal dominant with incomplete penetrance.
It presents the same as non-familial IPF but at an earlier age.

49
Q

What genes are thought to be mutated to cause familial IPF?

Describe the process.

A
  1. heterozygous mutations in telomerase
  2. Surfactant protein A2 and C

SPA2 and SPC mutations lead to lung injury. Repair occurs with mutated telomerase which leads to excessive fibrosis.

50
Q

What is the treatment for IPF?

A

Lung transplant in individuals 70 or younger

Respiratory/Musculoskeletal (36 decks)

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