Pathology of Interstitial Lung Disease & Fibrosis

Question 1

What histologic finds are characteristic of all restrictive lung disease?

Answer 1

Honeycomb lung and ground glass appearance

Question 2

What cancer is most associated with asbestosis?

Answer 2

Bronchogenic carcinoma

Question 3

A patient is diagnosed with asbestosis. What findings present on histologic analysis helped with the diagnosis?

Answer 3

Asbestos bodies/Ferrugenous bodies

Question 4

What is anthracosis?

Answer 4

Carbon pigment deposition in the lungs that does not impair function

Question 5

True/False. Necrotic granulomas present in patients with sarcoidosis.

Answer 5

False - while granulomas are present, they are non-caseating

Question 6

“Angel wings” is used to describe an x-ray finding on a patient with sarcoidosis. What causes this finding?

Answer 6

bilateral hilar lymphadenopathy

Question 7

What lab findings are typical in patients with sarcoidosis?

Answer 7

Hypercalcemia, elevated ACE, polyclonal hypergammaglobulinemia

Question 8

Sarcoidosis is most associated with what demographic?

Answer 8

African Americans and Caucasians of Irish descent

Question 9

Patchy interstitial fibrosis and fibroblastic foci are histologic findings most associated with what restrictive lung disease?

Answer 9

Idiopathic Pulmonary Fibrosis (IPF)

Question 10

True/False. Idiopathic pulmonary fibrosis is well-treated with immunosuppressors.

Answer 10

False - they are non-responsive to immunosuppressors. The only definitive treatment is lung transplant

Question 11

Pulmonary alveolar proteinosis presents with an accumulation of surfactant in intra-alveolar spaces. Why does this occur?

Answer 11

Macrophage defect in GM-CSF signaling, which regulates macrophage clearance of surfactant

Question 12

A patient is diagnosed with rheumatoid arthritis and pneumoconiosis. What name is given when these disorders present together?

Answer 12

Caplan syndrome

Question 13

This pneumoconiosis is associated with an increased risk of lung cancer and TB.

Answer 13

Silicosis

Question 14

Acute eosinophilic pneumonia with respiratory failure is a type of pulmonary eosinophilia. What is another name for this disorder?

Answer 14

Loeffler Syndrome

Question 15

A male with 10-year pack history presents to the office with respiratory problems. Histologic analysis finds “dusty brown” macrophages, lamellar bodies, and PAS+ granules. What is the diagnosis?

Answer 15

Desquamative Interstitial Pneumonia

Question 16

True/False. In cases of restrictive lung disease, FEV1/FVC is approximately 1.

Answer 16

True - this is because both FEV1 & FVC are reduced. In obstructive lung disease, the ratio is <0.70

Question 17

Hypersensitivity pneumonitis is caused by a (Type III/Type IV) hypersensitivity reaction.

Answer 17

May be either or both Type III (vasculitis) & IV hypersensitivity (granulomas)