interstitial lung disease

Question 1

what is The term Interstitial lung disease (ILD) ?

Answer 1

refers to a broad group of

inflammatory lung disorders

Question 2

what is ILD charaterzied by?

Answer 2

by acute, sub acute, or chronic inflammatory infiltration of alveolar walls by cells, fluid, and connective tissue

Question 3

how is ILD grouped?

Answer 3

based on

• occupational or environmental exposure,
• disease associations, and specific pathology.

Question 4

what can untreated ILD lead to?

Answer 4

irreversible pulmonary firbosis

Question 5

what are the 3 main categories of IDL?

Answer 5

• interstitial inflammation with granulomas
• idiopathic
• interstitial inflammation without granulomas
• miscellaneous diffuse lung disease

Question 6

what is the defintion of IDL

Answer 6

***restrictive lung diseases!!
Definition: a group of diseases that cause
chronic interstitial inflammation of the lung
leading to

1) collagen deposits (scar
tissue) and

2) pulmonary fibrosis

Question 7

what is the Known Risk factors for ILD

Answer 7

- occupation
• environment
• exposure to dusts
• cigarette smoke
• gender
• age

Question 8

What is the pathophysiology of ILD?

Answer 8

1. abnormalities involve pulmonary interstitium
   - –between capillary and alveolar space
2. Injury to the lung caused by
   - chronic inflammation
   - accumulation of inflammatory cells within the interstitial space

3.Leads to scarring and fibrosis

Question 9

charateristic of IPF?

Answer 9

What:
** specific form of chronic progressive fibrosing interstitial pneumonia. affecting mainly the lower lobes and periphery of lungs

Cause
- unknown cause

Who
- occurs in older adult

Question 10

how many types of IPF and what are they?

Answer 10

2 types

1) DIP (desquamative interstitial
pneumonia

2)UIP (usual interstitial pneumonia

Question 11

What are the risk factors of IPF

Answer 11

Smoking
- strong association particularly
if history > 20 years, 75% are smokers
or ex-smokers

GERD
-micro-aspiration of gastric
contents pulmonary fibrosis in
susceptible individuals

Viruses:
- role in initiation, progression
and acute exacerbations

Question 12

What are the ILD disease in the interstitial inflammation without granulomas?

Answer 12

1. Hypersensitivity Pneumonitis
2. Sarcoidosis
3. Wegner’s granulomatosis
4. Pneumoconiosis

• ->Silicosis
• ->Coal Worker’s
• ->Asbestosis

Question 13

What are the ILD disease in the Interstitial

disorders without granulomas

Answer 13

1. Drug-induced
   Interstitial Disease

2.Radiation-induced
Interstitial Lung
Disease

3.Goodpasture’s
Syndrome

4.Bronchiolitis
Obliterans with
Organizing
Pneumonia

5.Alveolar
Proteinosis

6.Collagen
Vascular Disease 
-->Rheumatoid
Arthritis 
-->Systemic Lupus
Erythematosus

Question 14

What is the Anatomic Alterations of the Lungs in IDL?

Answer 14

1. Destruction of the alveoli and adjacent pulmonary
   capillaries
2. Fibrotic thickening of the respiratory bronchioles,
   alveolar ducts, and alveoli
3. granuloma
4. Honeycombing and cavity formation
5. bronchial secretion
6. bronchospasm

Question 15

What is the clinical manifestations that lead

to a diagnosis of ILD initially?

Answer 15

1. Non-productive cough (dry) >80% of patients,
2. progressive dyspnea –> often hypoxemia (tachypnea, ↑HR, ), rapid shallow breathing,
3. bibasilar late inspiratory velcro like crackles >90% cases,
4. dull percussion, increased tactile and vocal fremitus

Question 16

What are the manifestations in late stage ILD?

Answer 16

Non Respiratory: 
- heart disease
- pulmonary hypertension (RHF), 
- Rheumatoid: joint pain &
swelling

Question 17

the key diagnosis finding in ILD?

Answer 17

1 CXR/CT – bilateral reticulo-nodular
opacity
–>secretion, atelectasis

2 .PFT’s – volumes, decrease DLCO,decrease FEV1, decrease FVC,
same FEV1%

3.ABG’s  Hypoxemia at rest later on

Question 18

what are the exception to the expected decrease diffusion capacity?

Answer 18

• Goodpasture’s syndrome
• Idiopathic pulmonary hemosiderosis

– DLCo is elevated response to the
increased amount of blood retained in the
alveolar spaces that is associated with these two disorders.

Question 19

what are the chest Radiograph findings?

Answer 19

Bilateral reticulonodular pattern
Irregularly shaped opacities
Granulomas
Cavity formation
Honeycombing
Pleural effusion

Question 20

what are the Pharmacologic Therapy for ILD?

Answer 20

Pirfenidone Esbriet
–> antifibrotic agent with anti-inflammatory & antioxidant effects

Nintedanib OFEV
–> a tyrosine kinase inhibitor (TKI) inhibit vascular endothelial, fibroblast
& platelet derived growth factor receptors

Question 21

dose/frequency of Pirfenidone Esbriet

Answer 21

dose
- 267mg/pill

freq
- 3 tid

Question 22

dose/frequency of Nintedanib OFEV?

Answer 22

dose
150mg

freq
- BID

Question 23

what is the outcome for both tx?

Answer 23

Improves FVC & disease progression &

quality of life

Question 24

What is the goal of ILD treatment?

Answer 24

1. early dx to slow disease progression
2. manage co-morbidities
3. prevent exacerbations to optimize QofL and increase survival

Question 25

what are other non-pharmcological treatment

Answer 25

• Oxygen Therapy
• Broncho-pulmonary Hygiene Therapy
• Avoidance of inflammatory triggers (GERDPPinhibitors,
OSA-CPAP) decrease lung injury
• Pulmonary rehabilitation
• Transplant (end-stage) less than 55 y.o
• Vaccinations (preventative)
• Palliative care (home care LTOT)

Question 26

what are the top 5 occupational that cuase ILD

Answer 26

1. Printing (Metal Working Fluids)
2. Healthcare/Nursing
3. Wood Working
4. Agriculture
5. Cleaners

Question 27

what is Pneumoconiosis ?

Answer 27

Pneumoconiosis is a form of lung
fibrosis caused by inhalation of dusts
such as asbestos, coal and silica

Question 28

what is Mesothelioma?

Answer 28

Mesothelioma is a malignant tumour
of the pleura caused by inhalation of
asbestos 20 40 years previously