Restrictive lung diseases

Question 1

Definition of insterstitial lung disease

Answer 1

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

Question 2

General categories of causes of interstitial lung disease

Answer 2

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

Question 3

Six classes of idiopathic interstitial pneumonias

Answer 3

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

Question 4

Describe approach to diagnosis of ILD

Answer 4

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.

Question 5

Presentation of most ILD

Answer 5

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

Question 6

PFTs of most ILDs

Answer 6

Restrictive pattern (decreased lung volume) with reduced diffusing capacity

Question 7

Physical exam of ILD

Answer 7

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

Question 8

Treatment of ILD

Answer 8

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

Question 9

Idiopathic pulmonary fibrosis

Answer 9

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

Question 10

Idiopathic pulmonary fibrosis physical exam and survival

Answer 10

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

Question 11

IPF associations

Answer 11

tobacco use, disease of older patients

Question 12

IPF imaging

Answer 12

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

Question 13

IPF pathology

Answer 13

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

Question 14

IPF therapy

Answer 14

none

Question 15

Non specific interstitial pneumonia

Answer 15

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

Question 16

Nonspecific intersitial pneumonia therapy

Answer 16

anti-inflammatory therapy

Question 17

Nonspecific intersitial pneumonia radiograph

Answer 17

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

Question 18

Non specific interstitial pneumonia pathology

Answer 18

homogenous fibrosis with varying inflammation and fibrosis

Question 19

Smoking related interstitial lung diseases

Answer 19

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

Question 20

Organizing pneumonia

Answer 20

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

Question 21

Organizing pneumonia imaging and path

Answer 21

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

Question 22

Organizing pneumonia treatment

Answer 22

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

Question 23

Eosinophilic pneumonia

Answer 23

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

Question 24

Lymphangioleiomyomatosis (LAM)

Answer 24

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

Question 25

Sarcoidosis description

Answer 25

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

Question 26

Sarcoidosis diagnosis

Answer 26

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

Question 27

Sarcoidosis trtmt

Answer 27

steroids