Diffuse Parenchymal Lung Diseases

Question 1

DPLD Pathophys

Answer 1

• Inflam and fibrosis of pulm interstitium

Question 2

DPLD Sx

Answer 2

• Dyspnea

- Cough

Question 3

DPLD Dx

Answer 3

• H&P
• CXR
• CT

Question 4

DPLD Tx

Answer 4

• Best supportive care: O2, resp rehab, steroids

Question 5

DPLD of known causes Etiology

Answer 5

• Drugs
• Connective tissue diseases
• Environmental exposure (birds, farming chemicals)
• Occupational exposures (coal, asbestosis)

Question 6

Pneumoconioses Epi

Answer 6

• Common
• Mining, agriculture
• “Black lung”

Question 7

Pneumoconioses Etiology

Answer 7

• Occupational lung disease caused by inhalation of particulate materials (usually minerals)

Question 8

Pneumoconioses Pathophys

Answer 8

• Inflam rxn in lungs leading to fibrosis and lung dysfx

Question 9

Pneumoconioses Sx

Answer 9

• Long latency period
• Dyspnea
• Cough

Question 10

Pneumoconioses Dx

Answer 10

• H&P: work hx, exposure hx, smoking, fam hx
• CXR (usually abnl)
• CT, PFT, bronchoscopy, biopsy

Question 11

Pneumoconioses Tx

Answer 11

• Lung transplant is only real option for severe
• Remove causative agent
• Supportive care: O2, bronchodilators, immunosuppressant

Question 12

Sarcoidosis Epi

Answer 12

• RF: young adults, AA

Question 13

Sarcoidosis Pathophys

Answer 13

• Idiopathic

- Multisystem disease–lungs are most frequent site

Question 14

Sarcoidosis Sx

Answer 14

• Dyspnea
• Cough
• Usually stable and self-limiting (non-harmful)

Question 15

Sarcoidosis Dx

Answer 15

• HRCT shows 1-5 mm lung nodules along bronchovascular bundles and subpleurally
• Need biopsy to r/o other diseases

Question 16

Sarcoidosis Tx

Answer 16

• Steroids

Question 17

LAM Epi

Answer 17

• Rare

- Young women

Question 18

LAM Etiology

Answer 18

• Idiopathic, diffuse, progressive

- Accelerated by preg and supplemental estrogen

Question 19

LAM Pathophys

Answer 19

• Proliferation of interstitial smooth muscle leading to cyst formation

Question 20

LAM Sx

Answer 20

• Dyspnea

- Pneumothorax

Question 21

LAM Dx

Answer 21

• HRCT

- Biopsy

Question 22

LAM Tx

Answer 22

• Lung transplant

Question 23

Idiopathic Pulm Fibrosis (IPF) Epi

Answer 23

• Common, progressive, fatal

- RF: M, >50, smoking, env exposure to particulate materials, viral inf, fam hx

Question 24

Idiopathic Pulm Fibrosis (IPF) Pathophys

Answer 24

• Small lung volume

Question 25

Idiopathic Pulm Fibrosis (IPF) Sx

Answer 25

- Exertional dyspnea - Nonproductive cough - Sx for many months - PE: fine insp crackles (Velcro rales) at bases

Question 26

Idiopathic Pulm Fibrosis (IPF) Dx

Answer 26

- HRCT: "honeycombing" - Appearance of unusual interstitial pneumonia - PFTs: restrictive pattern w/ low TLC, FEV1, DLCO - CXR: lower lung reticular opacities - Surg lung biopsy (gold standard)

Question 27

Idiopathic Pulm Fibrosis (IPF) Tx

Answer 27

- Steroids and immunosuppressant - O2 if needed - Pulm rehab - Lung transplant is only proven benefit