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Flashcards in Interstitial Lung Disease Deck (36):
1

What are interstitial lung diseases?

heterogeneous group of inflammatory and fibrotic disorders of the lower respiratory tract involving interstitial and occasionally alveolar and bronchial structures. They are classified together because of common clinical, x-ray, physiologic, and pathologic features.

2

What will you see on CXR with interstitial lung disease?

- bilateral streaky white opacities between the rib interspaces (too many lines and too many dots; reticulonodular).
- honeycombing
- volume loss
*looks like a spiderweb

3

Will a cat scan pick up interstitial lung diseases better tan CXR?

YES

4

What are the clinical features of interstitial lung disease?

- insidious onset of dyspnea with observed exertion and/or cough.
- spontaneous pneumothorax (especially eosinophilic granuloma).
- manifestations of extra-thoracic disease (sarcoidosis, collagen vascular).

5

**** What will you look for on physical exam of a pt with interstitial lung disease.

- bibasilar crackles
- clubbing
- signs of pulmonary HTN
- signs of systematic involvement

6

Is interstitial lung disease a RESTRICTIVE lung disease?

YES (decrease in all lung volumes)

7

What arterial blood gas abnormalities will you see with interstitial lung disease?

mild respiratory alkalosis due to mild drop in pCO2 and sharp drop in O2 sat with exercise.

8

What is the pathology of interstitial lung disease?

- interstitial pneumonitis (alveolitis)= usually mixed population of cells (mononuclear cell/polys) with mononuclear cell predominance.
- fibrosis
- bronchiectasis
- as disease progresses, may see sequence of: interstitial pneumonitis, fibrosis, honeycombing + bronchiectasis

9

How do we classify interstitial lung diseases?

- occupational (pneumoconioses)
- environmental
- drugs/poisons

10

What connective tissue diseases are associated with interstitial lung diseases?

RA, progressive systemic sclerosis, mixed connective tissue disease, polymyositis/dermatomyositis, SLE, Sjogrens, and ankylosing spondylitis

11

What are the main idiopathic causes of interstitial lung diseases?

- sarcoidosis
- vasculitis (wegener's, lymphomatoid granulomatosis, churg-strauss)
- histiocytosis X (eosinophilic granuloma)
- idiopathic pulmonary fibrosis
- idopathic pulmonary hemosiderosis
- chronic pulmonary alveolar proteinosis
- tuberous sclerosis
- familial pulmonary fibrosis

12

What are some other causes of interstitial lung diseases?

- infections
- lymphatic CA
- chronic aspiration
- uremia

13

** What is the general approach to a pt with interstitial lung disease?

- establish a diagnosis
- evaluate severity
- evaluate inflammatory activity (controversial)
- treatment

14

** How do we diagnose interstitial lung disease?

- H&P
- CXR
- CT chest (high resolution)
- PFTs
- arterial blood gas, exercise oximetry
- serology
- gallium scan
- bronchoscopy with bronchoalveolar lavage and transbronchial biopsy
- open lung biopsy (BEST)

15

** When looking at a CXR, what is the pneumonic for UPPER LOBE predominant causes of interstitial fibrosis?

A: ankylosing spondylitis
S: sarcoidosis
S: silicosis
E: eosinophilic granuloma
T: tuberculosis

16

**** If a young black female comes in with upper lobe interstitial lung disease and a skin rash, what is the most likely cause?

SARCOIDOSIS

17

**** If a young white male smoker comes in with upper lobe interstitial lung disease and a pneumothorax, what is the most likely cause?

EOSINOPHILIC GRANULOMA

18

** When looking at a CXR, what is the pneumonic for LOWER LOBE predominant causes of interstitial fibrosis?

B: bronchiectasis
A: asbestosis
D: DIP/UIP/IPF
B: breast shadows from implants
A: aspiration
S: sarcoidosis
S: scleroderma

19

**** If a pt who was in the navy and slept under pipes for 2 years and come in with interstitial disease with lower lobe thickening and calcification of plaques, what is the cause?

ASBESTOSIS

20

**** If a pt comes in with sclerodactyly (thickening and tightness of the skin of the fingers or toes) and lower lobe interstitial disease, what do they have?

SCLERODERMA

21

** When looking at a CXR, what is the pneumonic for MID_LUNG predominant causes of interstitial fibrosis?

S: sarcoidosis
U: uremia
C: CHF
G: goodpasture's
P: pulmonary alveolar proteinosis

22

What is a bronchoscopy?

insertion of a tube through the nose, down the trachea and into the lung in order to obtain a biopsy or for examination purposes.

23

What is a thoracoscopy?

surgical procedure used to biopsy the lung by making a small incision in the chest, performed by a CT surgeon.

24

For what can esophageal manometrics be sed?

scleroderma

25

For what would you look at sed rate, ANA, and rheumatoid factor?

SLE and RA

26

For what would you look at anti scl-70 and anti centromere antibody?

scleroderma

27

In what disease would you see ANCAs?

Wegener's granulomatosis

28

For what would you look at anti Jo, anti PM 1?

polymyositis= an idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness

29

In what disease would you see antiglomerular basement membrane antibodies?

Goodpasture's

30

In what disease would you see IgG precipiating antibodies?

acute hypersensitiviy pneumonitis

31

How do you decide if you are dealing with pure fibrosis or mixed inflammation on top of fibrosis causing symptoms?

indices vary from disease to disease
- for sarcoidosis= follow ACE level, and if it goes up over time you know the disease is worsening. You can also use a gallium scan, which is a radioactive tracer that gets actively taken up in areas where there is inflammation so it can be visualized.

32

If you don't see gallium in the lungs after doing a lung scan, are you most likely dealing with inflammation or fibrosis?

fibrosis

33

How do you treat asbestosis or eosinophilic granuloma?

nothing you can do

34

How do you treat idiopathic pulmonary fibrosis, sarcoidosis, or hypersensitivity pneumonitis?

steroids or new drugs that block fibrosis (pirfenidone or nintedanib)

35

How do you treat Wegener's?

cyclophosphamide w/wo steroids or azothioprine

36

How do we treat Goodpasture's?

cyclophosphamide + plasmapheresis