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Flashcards in Restrictive lung diseases Deck (27)
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1
Q

Definition of insterstitial lung disease

A

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

2
Q

General categories of causes of interstitial lung disease

A

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
Q

Six classes of idiopathic interstitial pneumonias

A

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

4
Q

Describe approach to diagnosis of ILD

A

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
Q

Presentation of most ILD

A

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

6
Q

PFTs of most ILDs

A

Restrictive pattern (decreased lung volume) with reduced diffusing capacity

7
Q

Physical exam of ILD

A

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

8
Q

Treatment of ILD

A

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

9
Q

Idiopathic pulmonary fibrosis

A

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

10
Q

Idiopathic pulmonary fibrosis physical exam and survival

A

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

11
Q

IPF associations

A

tobacco use, disease of older patients

12
Q

IPF imaging

A

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

13
Q

IPF pathology

A

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

14
Q

IPF therapy

A

none

15
Q

Non specific interstitial pneumonia

A

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
Q

Nonspecific intersitial pneumonia therapy

A

anti-inflammatory therapy

17
Q

Nonspecific intersitial pneumonia radiograph

A

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

18
Q

Non specific interstitial pneumonia pathology

A

homogenous fibrosis with varying inflammation and fibrosis

19
Q

Smoking related interstitial lung diseases

A

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
Q

Organizing pneumonia

A

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

21
Q

Organizing pneumonia imaging and path

A

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

22
Q

Organizing pneumonia treatment

A

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

23
Q

Eosinophilic pneumonia

A

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

24
Q

Lymphangioleiomyomatosis (LAM)

A

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

25
Q

Sarcoidosis description

A

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
Q

Sarcoidosis diagnosis

A

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

27
Q

Sarcoidosis trtmt

A

steroids