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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the causes of interstitial lung disease

A
Fibrosis (Idiopathic/ Pneumoconiosis/ Drugs)
Sarcoidosis
Hypersensitivity Pneumonitis 
Pulmonary Hypertension 
BOOP/COP
Vasculitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What drugs can cause interstitial lung disease?

A
Bleomycin
Busulfan
Amiodarone
Methysergide
Nitrofurantoin
Methotrexate
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Characteristics of Byssinosis:

  • Exposure
  • X-ray findings
  • Location
  • Risks
A
  • exposure: cotton
  • CXR: diffuse haziness
  • Location: lower lobes
  • Risk: n/a
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is Caplan Syndrome?

A

Pneumoconiosis in combinations with Rheumatoid Arthritis and intrapulmonary nodules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How do pirfenidone and nintedanib work?

A

Pirfenidone: inhibits collagen synthesis

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
What drugs are prostacyclin analogue vasodilators?
``` Epoprostenol Treprostinil Iloprost Beraprost Selexipag ```
26
Define BOOP/COP
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
Diagnostic test and Tx of BOOP/COP
Most accurate test: lung biopsy Tx: glucocorticoids
28
What is hypersensitivity pneumonitis?
An exaggerated immunological response to repeat exposure to antigens such as actinomycetes, fungi, molds and bird droppings. (Farmers Lung)
29
S&S acute hypersensitivity pneumonitis
``` Sudden fever, cough, dyspnea Fine crackles Leukocytosis Recurrent episodes Symptoms decrease after expose (e.g.when traveling) ```
30
Diagnosis tests
CXR: (Acute) scattered micro nodular interstitial opacities BAL: high relative lymphocyte count Lung biopsy: lymphocytic infiltrate
31
What is eosinophilic pneumonia?
Pneumonia that presents with 1-2wks of fever, cough, SOB and processes to respiratory failure. Hx: cancer Drugs, parasitic infections
32
What medications can precipitate eosinophilic pneumonia
``` Amiodarone NSAIDs Nitrofurantoin Phenytoin DAptomycin ```
33
What parasites can precipitate eosinophilic pneumonia
Strongyloidiasis Ascariasis Trichinellosis Schistosomiasis
34
Diagnostic test eosinophilic pneumonia
Most accurate test: BAL or biopsy (eosinophilic presence)
35
Common causes of increased A-a gradient
Diffusion limitation (emphysema, fibrosis) V/Q mistmatch (PE, lobar pneumonia) Intrapulmonary shunt (p. edema) Dead-space ventilation (PE, R--> L cardio shunt)
36
Tx hypersensitivity pneumonitis
glucocorticoids if persistent after antigen exposure removal. azathioprine (alt. chronic steroids) Acute HP: self resolving
37
S&S chromic hypersensitivity pneumonitis
``` Progressive cough Dyspnea Fatigue, weight loss Hypoxemia worse with exertion CXR: diffuse reticular interstitial opacities ```
38
Late phase reaction asthma
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
Tx late phase asthma reaction
systemic glucocorticoids