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aa Step 2CK: Internal & Peds > Pulmonary- Restrictive/ Interstitial > Flashcards

Pulmonary- Restrictive/ Interstitial Flashcards

1
Q

Define restrictive lung disease

A

Restricted lung expansion causes decreased lung volumes with preserved FEV1/FVC ratio.

Causes:
-Mechanical
 	Muscle weakness: polio, MG, GBS
	Structural: Scoliosis, obesity
-Interstitial
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2
Q

What are the causes of interstitial lung disease

A 
Fibrosis (Idiopathic/ Pneumoconiosis/ Drugs)
Sarcoidosis
Hypersensitivity Pneumonitis 
Pulmonary Hypertension 
BOOP/COP
Vasculitis
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3
Q

What drugs can cause interstitial lung disease?

A 
Bleomycin
Busulfan
Amiodarone
Methysergide
Nitrofurantoin
Methotrexate
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4
Q

What is pulmonary fibrosis?

A

thickening of the interstitial septum of the lung between the arteriolar space and the alveolus.
Interferes with gas exchange in both directions

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5
Q

Characteristics of coal workers pneumoconiosis:

  • Exposure
  • X-ray findings
  • Location
  • Risks
A
  • Exposure: Coal
  • CXR: fine nodular opacifications
  • Location: upper lobes
  • Risk: Caplan syndrome
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6
Q

Characteristics of silicosis:

  • Exposure
  • X-ray findings
  • Location
  • Risks
A
  • Exposure: sandblasting, rock mining, tunneling, foundries
  • CXR: eggshell calcifications & opacities
  • Location: upper lobes
  • Risk: TB, Caplan Syndrome
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7
Q

Characteristics of Asbestosis:

  • Exposure
  • X-ray findings
  • Location
  • Risks
A
  • Exposure: shipyard, plumbing (pipes), insulators, roofing
  • CXR: diffuse bilateral infiltrates; calcified supradiaphragmatic & pleural plaques.
  • Location: Lower lobes
  • Risk: mesothelioma, bronchogenic carcinoma (worse with smoking), pleural effusions
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8
Q

Characteristics of Byssinosis:

  • Exposure
  • X-ray findings
  • Location
  • Risks
A
  • exposure: cotton
  • CXR: diffuse haziness
  • Location: lower lobes
  • Risk: n/a
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9
Q

Characteristics of Berylliosis:

  • Exposure
  • X-ray findings
  • Location
  • Risks
A
  • exposure: electronic manufacturing, aerospace manufacturing
  • CXR: reticular interstitial pattern (similar to sarcoidosis)
  • Location: upper lobes
  • Risks: cor pulmonale, cancer, TB, Caplan syndrome
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10
Q

Characteristics of Bagassosis:

  • Exposure
  • X-ray findings
  • Location
  • Risks
A
  • Exposure: moldy sugar cane
  • CXR: diffuse infiltrates, ground glass honeycomb (advanced)
  • Location: Mid to upper lobes
  • Risk: cor pulmonale
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11
Q

Signs and symptoms of pulmonary fibrosis:

A 
Dyspnea (worse w/ exertion) 
Short shallow breaths
Fine rales or crackles
Loud P2 heart sounds
clubbing of the fingers
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12
Q

Diagnostic tests for pulmonary fibrosis

A

BIT: CXR or high resolution CT (more accurate)
Most accurate: lung biopsy

PFT: decreased DLCO, increased A-a gradient
Echo: pulmonary HTN, right ventricular hypertrophy

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13
Q

What is Caplan Syndrome?

A

Pneumoconiosis in combinations with Rheumatoid Arthritis and intrapulmonary nodules

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14
Q

Tx pulmonary fibrosis

A 
Prednisone or Steroids (berylliosis most likely to respond)
Azithioprine (long term alt. to steroids)
Cyclophosphamime s(no response to steroids)
Pirfenidone/ Nintedanib (slow fibrosis)
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15
Q

How do pirfenidone and nintedanib work?

A

Pirfenidone: inhibits collagen synthesis

ninetedanib: tyrosine kinase inhibitor, blocks ffibrogenic growth factors and inhibits fibroblast

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16
Q

Define sarcoidosis

A

an idiopathic inflammatory disorder predominantly of the lungs but can affect whole body. Widespread noncaseating granulomas

17
Q

Signs and symptoms of sarcoidosis

A 
Young African American women 
SOB with fine rales
Erythema nodosum 
Lymphadenopathy
Parotid gland enlargement 
Facial palsy
Heart block and restrictive cardiomyopathy
CNS involvement
Iritis and Uveitis
18
Q

Diagnostic tests sarcoidosis

A

BIT: CSR (hilar adenopathy)
Most accurate: lymphadenopathy biopsy (noncaseating granuloma)

Hypercalcemia
Elevated ACE
BAL: increased helper cells

19
Q

Tx sarcoidosis

A

Prednisone

20
Q

Define pulmonary hypertension (PHTN)

A

Pulmonary systolic BP >20mmHg.

Causes:

  • Pulmonary arterial hypertension (idiopathic)
  • Hypoxemia (COPD, Fibrosis, chronic lung disease)
21
Q

Mechanism of hypoxia causing PHTN

A

Hypoxemia causes vasoconstriction of the pulmonary circulation. Shunting blood away from areas of the lung with poor oxygen. causing full system constriction resulting in more hypoxemia (feedback)

22
Q

Signs and symptoms of PHTN

A 
Dyspnea
Fatigue
Syncope 
Chest pain
Wide splitting of S2 w/ loud P2
23
Q

Diagnostic tests for PHTN

A

BIT: CXR (dilation of proximal pulmonary arteries, narrowing “pruning” distal vessels)
Most accurate: Swan Ganz catheter

EKG/Echo: right hypertrophy
Polycythemia

24
Q

Tx PHTN

A 
Correct underlying disease.
Endothelin antagonist (Bosnian)
Phosphodiesterase inhibitors (sildenafil)
Prostacyclin analogue vasodilators
CCB

Lung transplant (curative for idiopathic)

25
Q

What drugs are prostacyclin analogue vasodilators?

A 
Epoprostenol
Treprostinil
Iloprost
Beraprost
Selexipag
26
Q

Define BOOP/COP

A

Bronchiolitis obliterans organized pneumonia (old name)
Cryptogenic Organized Pneumonia (new name)

Patchy process with proliferation of granulation tissue in small airways and ducts. Presents similar to CAP.

27
Q

Diagnostic test and Tx of BOOP/COP

A

Most accurate test: lung biopsy

Tx: glucocorticoids

28
Q

What is hypersensitivity pneumonitis?

A

An exaggerated immunological response to repeat exposure to antigens such as actinomycetes, fungi, molds and bird droppings. (Farmers Lung)

29
Q

S&S acute hypersensitivity pneumonitis

A 
Sudden fever, cough, dyspnea
Fine crackles
Leukocytosis 
Recurrent episodes
Symptoms decrease after expose (e.g.when traveling)
30
Q

Diagnosis tests

A

CXR: (Acute) scattered micro nodular interstitial opacities
BAL: high relative lymphocyte count
Lung biopsy: lymphocytic infiltrate

31
Q

What is eosinophilic pneumonia?

A

Pneumonia that presents with 1-2wks of fever, cough, SOB and processes to respiratory failure.
Hx: cancer Drugs, parasitic infections

32
Q

What medications can precipitate eosinophilic pneumonia

A 
Amiodarone
NSAIDs
Nitrofurantoin
Phenytoin
DAptomycin
33
Q

What parasites can precipitate eosinophilic pneumonia

A

Strongyloidiasis
Ascariasis
Trichinellosis
Schistosomiasis

34
Q

Diagnostic test eosinophilic pneumonia

A

Most accurate test: BAL or biopsy (eosinophilic presence)

35
Q

Common causes of increased A-a gradient

A

Diffusion limitation (emphysema, fibrosis)
V/Q mistmatch (PE, lobar pneumonia)
Intrapulmonary shunt (p. edema)
Dead-space ventilation (PE, R–> L cardio shunt)

36
Q

Tx hypersensitivity pneumonitis

A

glucocorticoids if persistent after antigen exposure removal.
azathioprine (alt. chronic steroids)

Acute HP: self resolving

37
Q

S&S chromic hypersensitivity pneumonitis

A 
Progressive cough
Dyspnea
Fatigue, weight loss
Hypoxemia worse with exertion 
CXR: diffuse reticular interstitial opacities
38
Q

Late phase reaction asthma

A

Hours after immediate response in asthma exacerbation . Characterized by proinflamatory gene expression, de novo synthesis of Th2 cytokines, further recruitment of leukocytes. Worsening bronchospasm

39
Q

Tx late phase asthma reaction

A

systemic glucocorticoids

aa Step 2CK: Internal & Peds (71 decks)

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