Interstitial lung diseases

Question 1

What is the definition of interstitial lung disease

Answer 1

Fibrosing and thickening of interstitial septum between arteriolar space and alveolus, interfering with gas exchange

Question 2

What are some specific causes of interstitial lung disease

Answer 2

1. ) Idiopathic mostly
2. ) Radiation
3. ) Drugs: Bleomycin, amiodarone, nitrofurantoin, cyclophosphamide
4. ) Pneumoconioses (asbestosis, berylliosis, coal worker’s, silicosis, etc)

Question 3

Interstitial lung disease can be separated based on whether or not its in the inflammatory stage or if its actually fibrosis. What does this mean

Answer 3

1. ) Inflammatory - WBC’s there, it is reversible so must use prednisone (berryolsis is the one that responds most because has granulomas)
2. ) Fibrosis - irreversible, no treatment

Question 4

What is the presentation of someone with interstitial lung disease from any cause

Answer 4

1. ) Dyspnea worse on exertion
2. ) Crackles on examination
3. ) Loud P2
4. ) Clubbing of fingers

Should not have wheezing

Question 5

What is the best initial test, more accurate test, and most accurate test for interstitial lung disease

Answer 5

Initial test: Chest X-Ray

More accurate: High resolution CT - will see honeycombing

Most accurate: Lung biopsy

Echo may show pulmonary HTN and right sided ventricular hypertrophy

Question 6

In interstitial lung disease, what will the PFT’s show

Answer 6

Restrictive pattern - FEV1, FVC, TLC, and residual volume all decreased, and DLCO is super decreased

However, FEV1/FVC ratio will remain the same

Question 7

What is the definition of sarcoidosis

Answer 7

Idiopathic inflammatory disorder that affects lungs and other parts of the body that most likely happens to young african women

Question 8

How would someone with sarcoidosis present on examination

Answer 8

Young African woman with shortness of breath and fine rales on exam, but no wheezing

If they have erythema nodosum or lymphadenopathy, this is most likely the answer

Other things they will have: Parotid gland enlargement, facial palsy, heart block and restrictive cardiomyopathy, CNS, and iritis and uveitis

Question 9

What is another hint that it is sarcoidosis on examination

Answer 9

A previously healthy african american woman with hilar adenopathy

Question 10

What is the order of diagnostic tests for someone with sarcoidosis

Answer 10

1. ) Best initial: Chest Xray showing hilar adenopathy
2. ) Most accurate: Lymph node biopsy showing granulomas that are non-caseating**
3. ) Can also do bronchoalveolar lavage which will show a lot of T4 helper cells

Other lab values: Elevated ACE most common, high calcium less common from vitamin D formation in granulomas

Pfts will show restrictive pattern (normal FEV1/FVC with everything decreased)

Question 11

What is the treatment for sarcoidosis

Answer 11

Like all restrictive diseases, prednisone and most people will respond since granulomas are present indicating active inflammation and not fibrosis

Question 12

Associate the job with the type of pneumoconiosis: Coal

Answer 12

Coal worker’s pneumoconiosis

Question 13

Associate the job with the type of pneumoconiosis: Sandblasting, tunneling, rock mining

Answer 13

Silicosis

Question 14

Associate the job with the type of pneumoconiosis: Shipyard workers, pipes, insulators

Answer 14

Asbestosis

Question 15

Associate the job with the type of pneumoconiosis: Electronic manufacture

Answer 15

Berylliosis