Parenchymal Lung Disease (Gerke)

Question 1

type of _____ lung disease is determined by the type, amount, and location of cells that are present in the interstitium, bronchiole and alveolar air spaces.

Answer 1

interstitial

Question 2

All of the following are either causes or manifestations of interstitial lung disease EXCEPT:
A. Edema
B. Bronchiectasis
C. Malignancy
D. Infections/Inflammation
E. Fibrosis

Answer 2

B. Interstitial lung disease (ILD) refers to a group of lung diseases affecting the tissue and space around the air sacs of the lungs). It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues. It does not include disease of airway obstruction, such as bronchiectasis, asthma, or COPD.

Question 3

A 41 yo African American female has 2 months of cough, non-productive. She also notes new red, itchy eyes (uveitis) and low grade fevers at night. Normal physical exam except for tender red-purple bumps (erythema nodosum) on shins. Chest xray revealed noncaseating granulomas. What is the probable diagnosis?

Answer 3

Sarcoidosis

This is a diagnosis of exclusion - must first rule out infection, cancer, foreign bodies and inhalations

Question 4

what is the treatment for sarcoidosis?

Answer 4

corticosteroids or other immunosuppressants (ie, methotrexate, leflunomide, azathioprine, mycophenolate)

Question 5

A 65 year old male presents with shortness of breath upon exertion, upstairs. He reports it has been going on for 1-2 years and is accompanied by an occasional dry cough, which is not bothersome. Past medical history is significant for obstructive sleep apnea, osteoarthritis and allergies. “Velcro crackles” but no wheezing are heard on chest auscultation. He has mild clubbing of the fingernails. Imaging reveals reduced lung volume with honeycombing and fibrosis along the bases of the lung. What is the probable diagnosis?

Answer 5

idiopathic pulmonary fibrosis (aka, usual interstitial pneumonia)

Question 6

what percentage of interstitial pulmonary fibrosis patients have crackles on exam?
A. 20%
B. 50%
C. 80%
D. 100%

Answer 6

C. Additionally, up to 50% have clubbing.

Question 7

True or false: inflammation is though to play a role in idiopathic pulmonary fibrosis.

Answer 7

false. that was the old theory, but anti-inflammatory medication does not appear to help this condition at all, and it has been thrown out.

Question 8

treatment approved for IPF includes what two antifibrotics that were recently approved in October 2014?

Answer 8

pirfenidone and nintedanib