Interstitial Lung Disease - Goya

Question 1

What is Interstitial Lung Disease (ILD)?

Answer 1

comprised of a diverse group of lung infiltrates that cause disruption of the alveolar structures and have common clinical, radiographic and physiological consequences

scarring of lung and SOB and restrictive pattern on PFTs

incidence of ILD varies with diseases

• scleroderma 100%
• RA 33%
• sarcoidosis 33%
• idiopathic pulmonary fibrosis 30-40%

Question 2

What is interstitium?

Answer 2

the space between the basement membranes of the alveolar lining epithelium and the capillary endothelium

Question 3

What drugs can cause ILD?

Answer 3

Nitrofurantoin
Amiodarone**
Gold
Dilantin
Bleomycin (chemo)
Cytoxan (chemo)
Radiation

Question 4

What are the 2 idiopathic interstitial pneumonias that we have to know? What are their prognoses?

Answer 4

Usual Interstitial Pneumonia (UIP)
-poor prognosis –> more fibrosis and scarring

Nonspecific interstitial pneumonia (NSIP)

• treatable
• cellular level

Question 5

What are the 5 most common interstitial lung diseases?

Answer 5

Usual interstitial pneumonia

Nonspecific interstitial pneumonia

Connective tissue Disease

Hypersensitivity Pneumonitis

Sarcoidosis

Question 6

What is the common presentation of ILD?

Answer 6

Typical patients are middle aged presenting with progressive dyspnea & nonproductive cough

other symptoms depend on the underlying process

• connective tissues affect women > men (except RA)
• pneumoconiosises affect men more

long latency from exposure –> disease

Question 7

What are some PE findings associated with ILD?

Answer 7

• bibasilar rales
• clubbing of fingers (except in sarcoidosis)
• pulmonary HTN and cor pulmonale in advanced diseases

Question 8

What x-ray findings will be seen in ILD?

Answer 8

reticular or reticulonodular pattern is the most common

honey-combing is a late finding (seen better on CT)

*CXR goof for suggesting presence of ILD but not the stage

Question 9

What is a great test for suspected early ILD?

Answer 9

high resolution CT scan

start as sub pleural patch leading to diffuse fibrosis “honeycomb lung”

Question 10

What will PFTs for ILD show?

Answer 10

• decrease in TLC –> restrictive
• decrease in FVC
• increase in FEV1/FVC ratio

Question 11

How will the bronchoalveolar lavage differ in the causes of ILD? When is this done?

Answer 11

Lymphocytes-hypersensitivity pneumonitis & sarcoidosis

Neutrophils increased in IPF & asbestosis

*only done in suspected infectious causes of ILD

Question 12

When would a lung biopsy be used to diagnose ILD?

Answer 12

to exclude neoplastic and infectious processes

useful in diagnosing UIP, nonspecific interstitial pneumonia

Question 13

What may be the initial procedure of choice in ILD? What causes are good yield?

Answer 13

bronchoscopy

good yield:

• sarcoidosis
• hypersensitivity pneumonitis
• eosinophilic pneumonia
• lymphanngitic carcinomatosis

Question 14

What is the major goal of management of ILD?

Answer 14

identify and aggressively treat the inflammatory process (many causes are not responsive to treatment)

remove the offending agent

Question 15

What are some treatment options for ILD?

Answer 15

Immunosuppressants (Azathioprine, Cytoxan, Cyclosporin)

Corticosteroids* (suppress the alveolitis)

Question 16

When should a lung transplant be considered?

Answer 16

Failure to medical therapy

Worsening oxygen desaturation with minimal exercise

Resting hypoxemia

Worsening pulmonary function testing

normally only do a single lung transplant

Question 17

What is crucial to diagnosis of ILD?

Answer 17

HISTORY*