January 26, 2016 - Diagnosis and Management of ILD

Question 1

Type I Pneumocyte

Answer 1

Flattened region of gaseos transport/diffusion, which is vulnerable to injury.

Question 2

Type II Pneumocyte

Answer 2

Site of surfactant synthesis.

Can proliferate and re-form alveolar epithelial surface.

Question 3

Non-Pharmacologic Management of ILD

Answer 3

Smoking cessation

Infection prevention

Pulmonary rehabilitation

Oxygen supplementation

Manage co-morbidities (GERD, OSA (obstructive sleep apnea), CHF)

Question 4

Pharmacologic Management

Answer 4

1. Immuno-modulatory therapies
2. Anti-fibrotic therapies (IPF only)

Question 5

Sarcoidosis

Answer 5

A multisystem granulomatous disorder of unknown etiology.

Affects individuals worldwide.

Characterized histologically by the presence of non-caseating granulomas in the affected organs.

~50% is detected incidentally on CXR.

Question 6

Organ Involvement of Sarcoidosis

Answer 6

Lungs - 95%

Skin - 16%

Lymph nodes - 15%

Eye - 12%

Liver - 12%

Erythema Nodosum - 8%

Spleen - 7%

Question 7

Sarcoidosis - Histology

Answer 7

Granulomatous (non-caseating) inflammation.

Question 8

Indications for Treatment - Sarcoidosis

Answer 8

60-80% of asymptomatic patients have spontaneous remission.

Consider treatment if it is progressive symptomatic pulmonary, neurologic, ocular, hypercalcemia/renal, or cardiac

Question 9

Treatment - Pulmonary Sarcoidosis

Answer 9

Glucocorticoids (First line) - prednisone

Methotrexate

Azathioprine

Combination therapy

Lung transplantation

Question 10

Signs of Restrictive Spirometry

Answer 10

FEV1/FVC > 70%

Low FVC (<80%)

Low FEV1 (<80%)

Low DLCO

Question 11

Idiopathic Pulmonary Fibrosis (IPF)

Answer 11

The most common of the idiopathic ILD’s.

Chronic progressive parenchymal lung disease of unclear origin.

Median survival is 3-5 years from diagnosis.

Usually affects those aged >50

2 therapies now available; pirfenidone and nintedanib