Types of Interstitial Lung Diseases

Question 1

What is the primary pulmonary manifestation associated with the conditions discussed?

Answer 1

Interstitial Lung Disease (ILD)

ILD can also include pleural diseases, airway complications, pulmonary vascular disease, opportunistic infections, and drug toxicity.

Question 2

Define Interstitial Lung Disease (ILD).

Answer 2

Group of respiratory diseases that cause progressive fibrosis (scarring) of the interstitium and space around alveoli

ILD can be classified by its underlying cause.

Question 3

How many main types of ILD classifications are there?

Answer 3

3 main types

The types include exposure-related ILDs, systemic disease ILDs, and drug-induced ILDs.

Question 4

What are the three main types of ILD classifications?

Answer 4

• Exposure-related ILDs
• Systemic disease ILDs
• Drug-induced ILDs

Question 5

What are exposure-related ILDs caused by?

Answer 5

Occupational exposure, environmental exposure, intentional exposure, iatrogenic exposures

These exposures lead to various forms of lung disease.

Question 6

What is the most common cause of systemic disease ILDs?

Answer 6

Connective diseases (CDs), sarcoidosis, and vasculitis

Connective diseases often lead to ILD.

Question 7

What characterizes ILD associated with rheumatoid arthritis (ILD-RA)?

Answer 7

• More common in males than females
• Presents as idiopathic pulmonary fibrosis or nonspecific interstitial pneumonia
• High titres of rheumatoid factor increase risk
• No relationship between joint involvement and ILD progression
• RA treatments can cause pulmonary toxicity

Question 8

What is the most common form of ILD in Sjogren’s syndrome?

Answer 8

Nonspecific interstitial pneumonia (NSIP)

It can also present as idiopathic pulmonary fibrosis (IPF), cryptogenic organizing pneumonia (COP), or lymphocytic interstitial pneumonia (LIP).

Question 9

What are typical respiratory symptoms in ILD-Sjogren’s syndrome?

Answer 9

Cough and dyspnea

These symptoms are commonly associated with the disease.

Question 10

What are the clinical features of ILD in systemic sclerosis?

Answer 10

• More common in diffuse cutaneous systemic sclerosis than limited cutaneous systemic sclerosis
• Dyspnea and cough that progress slowly
• Most commonly presents as nonspecific interstitial pneumonia

Question 11

True or False: There is a relationship between skin involvement in systemic sclerosis and the progression of ILD.

Answer 11

False

No relationship exists between skin involvement and ILD progression.

Question 12

What can repeated flare ups of acute lupus pneumonitis cause in ILD-SLE?

Answer 12

Interstitial fibrosis

This indicates the chronic impact of lupus pneumonitis on lung health.

Question 13

What percentage of cases in ILD-Dermatomyositis see ILD precede skin or muscle involvement?

Answer 13

33%

This highlights the varied presentation of ILD in dermatomyositis.

Question 14

What are common presentations of ILD-Dermatomyositis?

Answer 14

• Acute interstitial pneumonia (AIP)
• Idiopathic pulmonary fibrosis (IPF)
• Cryptogenic organizing pneumonia (COP)
• Abnormal radiological lung findings without respiratory symptoms

Question 15

What are the main respiratory symptoms of ILD?

Answer 15

Dyspnea

Dyspnea can also be influenced by cardiac involvement in conditions like dermatomyositis.

Question 16

What systemic drug can cause interstitial lung disease (ILD)?

Answer 16

Methotrexate: Pulmonary toxicity side effect can present as methotrexate interstitial pneumonitis.

Typically subacute onset of cough, dyspnea and fever. Incidence increases as MTX dose increases.

Question 17

What is another systemic drug that can cause pneumonitis?

Answer 17

Leflunomide: Leflunomide alone or in conjunction with methotrexate can cause pneumonitis.

Question 18

What is the risk associated with anti-TNF biologics?

Answer 18

Anti-TNF biologics: Increase risk of ILD and can cause fatal exacerbations in patients with pre-existing ILD.

Question 19

What is Idiopathic Pulmonary Fibrosis (IPF)?

Answer 19

Chronic, patchy fibrosing interstitial pneumonitis of unknown cause that is limited to lungs.

Typical age onset over 50 yrs old and is more common in males than females.

Question 20

What are the clinical features of IPF?

Answer 20

Slowly-worsening dyspnea that is worse on exertion and non-productive cough. Typically no extrapulmonary involvement.

Question 21

What are the radiological findings for IPF?

Answer 21

Lower lung zone predominant, subpleural reticular opacities with areas of honeycombing.

Question 22

What is Nonspecific Interstitial Pneumonia (NSIP)?

Answer 22

Chronic diffuse, uniform infiltration of interstitium with varying amounts of chronic inflammation and fibrosis.

Typical age onset 40-50 yrs old, and is equally common in males and females.

Question 23

What are the clinical features of NSIP?

Answer 23

Progressive dyspnea, cough and fatigue.

Question 24

What are the radiological findings for NSIP?

Answer 24

Diffuse bilateral reticular and ground-glass opacities and no honeycombing.

Question 25

What is Cryptogenic Organising Pneumonia (COP)?

Answer 25

Acute/subacute inflammation and fibrosis of alveoli with/without bronchioles.

Typical age onset at 55 yrs old, and is equally common in males and females. 2x common in non-smokers than smokers.

Question 26

What are the clinical features of COP?

Answer 26

Subacute onset of dyspnea and cough, and frequent LRTIs.

Question 27

What are the radiological findings for COP?

Answer 27

Bilateral and multifocal consolidation that can extend into bronchioles.

Question 28

What is Acute Interstitial Pneumonia (AIP)?

Answer 28

Also known as Hamman-Rich syndrome.

Typical age onset is 50 yrs old, and is equally common in males and females.

Question 29

What are the clinical features of AIP?

Answer 29

Rapid onset of dyspnea and hypoxemia with sudden deterioration to respiratory failure.

Question 30

What is Lymphocytic Interstitial Pneumonia (LIP)?

Answer 30

Lymphocytic infiltration of the alveolar interstitium and air spaces.

Question 31

What is the typical age of onset for Lymphocytic Interstitial Pneumonia?

Answer 31

50 years old.

Question 32

Is Lymphocytic Interstitial Pneumonia more common in males or females?

Answer 32

It is more common in females than males.

Question 33

What condition is strongly associated with Lymphocytic Interstitial Pneumonia?

Answer 33

Sjogren’s syndrome.

Question 34

What are the clinical features of Lymphocytic Interstitial Pneumonia?

Answer 34

Progressive cough and dyspnea over 3 years.