Clinical Interstitial Lung disease

Question 1

What is the pulmonary feature of SLE?

Answer 1

Pleural disease is the most common but with long standing SLE then interstitial lung disease (and UIP) can form

Question 2

What is used for every interstitial lung disease EXCEPT for IPF?

Answer 2

corticosteroids

Question 3

In what connective tissue disease is pulmonary fibrosis most common and most severe

Answer 3

Progressive systemic sclerosis (PSS)

Question 4

What are the Interstitial lung diseases associated with smoking?

Answer 4

• RB-ILD
• Desquamative Interstitial Pneumonia (DIP)
• Langerhans Cell histiocytosis
• IPF (also happens in non smokers but more common in smokers)

Question 5

Progressive systemic sclerosis can involve pulmonary arteries, leading to what?

Answer 5

Pulmonary HTN

Question 6

Drugs associated with interstitial lung disease

Answer 6

• Antiarrhythmics (amiodarone)
• Anti bacterial (nitrofurantoin, sulfonamides)
• Anti neoplastics (bleomycin, cyclophasphamide, methotrexate, nitrosoureas)
• anti rheumatics (gold salts, penicillamine)
• phenytoin

Question 7

Most common pulmonary feature with Rheumatoid Arthritis?

Answer 7

Pleurisy, pleural effusions, or both

Question 8

RB-ILD is found in only what type of people?

Answer 8

smokers

Question 9

Cause of interstitial Lung disease

Answer 9

• In most, no specific cause can be identified

- in remainder, medications and a variety of organic and inorganic dusts

Question 10

Common presentation features

Answer 10

• Dyspnea
• Late inspiratory crackles
• septal thickening and reticulonodular changes

Question 11

histopathologic patter in interstitial lung disease

Answer 11

usuall interstitial pneumonia (UIP)

Question 12

When can a diagnosis of idiopathic fibrosis be made with 90% confidence?

Answer 12

in patients over 65 who have

• idiopathic disease by history and who demonstrate inspiratory crackles on physical exam
• restrictive physiology on pulmonary function testing
• characteristic radiographic evidence of progressive fibrosis over several years
• and diffuse, pathcy fibrosis with pleural-based honeycombing on high resolution CT scan

Question 13

The 3 connective tissue disorders discussed that have pulmonary involvement and can show NSIP

Answer 13

• Rheumatoid arthritis
• SLE
• Progressive systemic sclerosis (PSS)

Question 14

NSIP is found in patients with what?

Answer 14

connective tissue diseases

Question 15

What is the characteristic of a pleural effusion in rhematoid arthritis?

Answer 15

tends to have the lowest glucose levels in the fluid

Question 16

Describe the 2 agents approved for treatment of IPF

Answer 16

DON’T improve survival or quality of life

-Expensive (100,000/year)

Question 17

What does biopsy of progressive systemic sclerosis show?

Answer 17

NSIP

Question 18

2 agents approved for treatment of IPF showing a significant reduction in the rate of decline in lung function

Answer 18

• Nintedanib

- Pirfenidone

Question 19

What is the only definite treatment for IPF and what is the survival rate?

Answer 19

Lung transplant

-50% 5 year survival rate