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Flashcards in Restrictive lung diseases Deck (27):
1

Definition of insterstitial lung disease

Hetergenous group of disorders with a combo of inflammation or scarring.

2

General categories of causes of interstitial lung disease

idiopathic (sarcoidosis or idiopathic interstitial pneumonias), 1. autoimmune, 2. Exposure to inorganic dusts (typically occupational dusts such as silica or asbestos) 3. Exposure to organic molecules that result in hypersensitivity pneumonitis 4. Drug effect

3

Six classes of idiopathic interstitial pneumonias

Idiopathic pulmonary fibrosis, idiopathic nonspecific interstitial pneumonia, Respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia, cryptogenic organized pneumonia, acute interstitial pneumonia

4

Describe approach to diagnosis of ILD

clinical (history and physical) look for evidence of known causes (ie. Autoimmune). Radiographic patterns on high resolution CT. Pathologic pattern from lung biopsy. Etiology alone is not enough, must also know pattern.

5

Presentation of most ILD

Dyspnea on exertion. Nonproductive cough is common. Rarely, the disease may be sudden in onset. Decreased DLCO

6

PFTs of most ILDs

Restrictive pattern (decreased lung volume) with reduced diffusing capacity

7

Physical exam of ILD

end-inspiratory crackles (velcro like), digital clubbing if bad, joint/skin exam to identify collagen vascular diseases

8

Treatment of ILD

remove exposure, immunosuppressive therapy (steroids), oxygen, transplant, research trial for IPF

9

Idiopathic pulmonary fibrosis

Idiopathic scarring lung disease with pattern of injury of usual insterstitial pneumonia (UIP).

10

Idiopathic pulmonary fibrosis physical exam and survival

Cough, DOE, fatigue, basilar predominant velcro crackles, digital clubbing if bad. Survival is 2-3 yrs.

11

IPF associations

tobacco use, disease of older patients

12

IPF imaging

Peripheral and basilar predominant reticulation, traction bronchiectasis, honeycombing and ground glass infiltrate

13

IPF pathology

Heterogenous fibrosis with fibroblast foci and juxtaposition of fibrotic and normal lung

14

IPF therapy

none

15

Non specific interstitial pneumonia

Presents similar to other ILD. May be idiopathic, collagen vascular disease related (RA), fibrotic, or drug related. More likely to be female and younger, with better prognosis than IPF

16

Nonspecific intersitial pneumonia therapy

anti-inflammatory therapy

17

Nonspecific intersitial pneumonia radiograph

basilar predominant reticular abnormality with volume loss, bronchiectasis, ground glass, no honeycombing

18

Non specific interstitial pneumonia pathology

homogenous fibrosis with varying inflammation and fibrosis

19

Smoking related interstitial lung diseases

Respiratory bronchiolitis-ILD, Desquamative interstitial pneumonia (DIP) and pulmonary langerhans cell histiocytosis. RBILD and DIP are both on a continuum. Langerhans is seen in young smokers and it results in cysts with high risk for pneumothorax. Treatment for all is smoking cessation

20

Organizing pneumonia

noninfectious pneumonia with alveolar filling. May be idiopathic, secondary to cardio disease, drugs, aspiration of stomach acid.

21

Organizing pneumonia imaging and path

imaging shows ground glass and consolidation. Path shows organizing pneumonia pattern with alveolar filling

22

Organizing pneumonia treatment

steroids- requires 6-12 months, and can have relapse

23

Eosinophilic pneumonia

Acute often results in acute respiratory failure, mimics ARDS and shows eosinophils. Chronic is associated with asthma. Both are treated with steroids

24

Lymphangioleiomyomatosis (LAM)

Cystic lung disease in young women- Has an obstructive pattern on PFTs. Treated with Sirolimus (mTOR inhibitor)

25

Sarcoidosis description

systemic granulomatous disorder of unknown etiology. Noncaseating granulomas that affect many organs. May have obstructive, restrictive or mixed PFTs. Cough, chest pain, wheezing. Younger patients

26

Sarcoidosis diagnosis

clinical finding swith pathology- granulomas. Must exclude other causes of granulomas

27

Sarcoidosis trtmt

steroids