Interstitial Lung Disease

Question 1

This is the most common cause of interstitial lung disease?

Answer 1

Idiopathetic pulmonary fibrosis

Question 2

Pathophysiology of ILD?

Answer 2

Lung sustains an injury. Due to exhausted resources or imperfect repair system, the injury is retained. ILDs can involve excess collagen deposition into the interstitium and surrounding tissues. This leads to fibrotic tissue deposition in the lung and impaired capillary gas exchange.

Question 3

Clinical presentation of ILD?

Answer 3

Dyspnea on exertion that is progressive, non productive dry cough, pleuritic chest pain

Question 4

Physical exam findings of ILD?

Answer 4

Coarse crackles, wheezing IS NOT common, clubbing of finger nails if in severe stage, cyanosis

Question 5

Diagnostic testing for ILD?

Answer 5

CXR, high resolution CT scan (HRCT), pulmonary function testing (PFT)

Question 6

This will be present on PFT of ILD?

Answer 6

Restrictive patterns, decrease in TLC.

Question 7

Pathogensis of idiopathic pulmonary fibrosis?

Answer 7

Fibrosis of parenchyma of lungs. Thought to be a result of aberrant promotion of fibroblast and subsequent collagen deposition of epithelial surfaces. Fibrosis spreads from alveoli to alveolar lumen and adjacent lung parenchyma

Question 8

Physical exam findings for IPF?

Answer 8

MAY reveal crackles, clubbing, cyanosis, pulm HTN, pedal edema, holosystolic tricuspid regurg murmur

Question 9

Findings on CXR? Finding on CT

Answer 9

CXR: opacities and densities, especially lower lobs with loss of volume. CT: Honeycombing

Question 10

These values would indicate oxygen therapy?

Answer 10

SpO2

Question 11

Sarcoidosis is a systemic disease that involves?

Answer 11

formation of granulomas, found commonly in the tissues of lungs and lymph nodes of throacic region

Question 12

Having a first degree relative with sarcoidosis increases your risk this much?

Answer 12

5 fold. Genetics likely play a role

Question 13

Clinical manifestation of sarcoidosis?

Answer 13

Dyspnea, cough, chest pain. Fatigue, night sweats, weigh loss can occur

Question 14

Sarcoidosis can eventually progress to?

Answer 14

Pulmonary fibrosis

Question 15

ENT presentation of sarcoidosis

Answer 15

anosmia, erosion of cartilage nasal septum, deformity, stridor, dysphonia, dysphagia and cough

Question 16

Dermatologic presentation of sarcoidosis?

Answer 16

Maculopapular lesions w/ red/violaceous appearance

Question 17

Why would you test serum ACE levels?

Answer 17

sarcoid granulomas produce angiotensin converting enzyme but only elevated in 60% of sarcoid patients.

Question 18

Most conditions of sarcoidosis are treated with?

Answer 18

oral prednisone

Question 19

Unlike other ILD, fibrotic lesions are reversible in this disease?

Answer 19

Cryptogenic organizing pneumonia (COP)

Question 20

Clinical presentation of crypgenic organizing pneumonia

Answer 20

cough, dyspnea, malaise, fever. onset is typically weeks to months, weight loss of greater than 10lbs in over 50%

Question 21

CXR findings

Answer 21

bilateral consolidation that can be patchy or diffuse

Question 22

CT scan on COP

Answer 22

Patchy opacities are more frequently found in periphery of lung fields in lower portions of lungs

Question 23

These are higher in COP than IPF?

Answer 23

Lymphocytes, neutrophils, eosinophiles

Question 24

Treatment of COP depends on?

Answer 24

Severity

Question 25

Sever or progressing COP treatment?

Answer 25

If patient failed steroids, cyclophosphamide. May also need macrolide antibiotic

Question 26

Mild to moderate COP treatment?

Answer 26

Macrolide therapy: Clarithromycin 250-500mg BID 3-6 months

Question 27

Sever COP treatment?

Answer 27

4-8 wks PO steroids (0.75-1mg/kg/day). taper over 4-6 weeks.