Interstitial lung disease and idiopathic fibrosis

Question 1

What is Interstitial lung disease?

Answer 1

Interstitial lung disease describes a large group of disorders, most of which cause progressive scarring of lung tissue.

The scarring associated with interstitial lung disease eventually affects your ability to breathe and get enough oxygen into your bloodstream

Question 2

What causes interstitial lung disease?

Answer 2

1. Can be idiopathic (Idiopathic pulmonary fibrosis)
2. Secondary to autoimmune disease such as RA
3. Inhaled substances
4. Medication

Question 3

What are the symptoms of interstitial lung disease?

Answer 3

The primary signs and symptoms of interstitial lung disease are:

• Shortness of breath at rest or aggravated by exertion
• Dry cough

By the time symptoms appear, irreversible lung damage has often already occurred

Question 4

What are risk factors for interstitial lung disease?

Answer 4

1. Age
2. Exposure to occupational and environmental toxins
3. Gastroesophageal reflux disease
4. Smoking
5. Radiation and chemotherapy

Question 5

How is interstitial lung disease managed?

Answer 5

ILD is not a single disease but encompasses many different pathological processes. Hence treatment is different for each disease. If a specific occupational exposure cause is found, the person should avoid that environment. If a drug cause is suspected, that drug should be discontinued.

Question 6

What is Idiopathic pulmonary fibrosis?

Answer 6

Idiopathic pulmonary fibrosis (IPF) is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue.

Chronic lung scarring disease, associated with progressive and irreversible decline in lung function

Question 7

What are the symptoms of Idiopathic pulmonary fibrosis?

Answer 7

The main features are progressive breathlessness, a non- productive cough and cyanosis, which eventually lead to respiratory failure, pulmonary hypertension and cor pulmonale

Bilateral inspiratory crackles on auscultation of the chest. Clubbing is common

Age over 45 years.

Question 8

What are risk factors for Idiopathic pulmonary fibrosis?

Answer 8

Risk factors include cigarette smoking, acid reflux disease (GERD) certain viral infections, and genetic predisposition

Question 9

How would you manage Idiopathic pulmonary fibrosis?

Answer 9

Assessments for pulmonary rehabilitation should be made at the time of diagnosis and repeated at 6-month or 12-month intervals

Pirfenidone treatment, ONLY if:

1. The person has a forced vital capacity (FVC) between 50% and 80% predicted.
2. Treatment is stopped if there is evidence of disease progression
3. The agreed pricing structure is followed.

Question 10

What is the prognosis of Idiopathic pulmonary fibrosis?

Answer 10

The median survival for people with IPF in the UK is approximately 2.5 years from the time of diagnosis

Question 11

What are pathological hallmarks of pulmonary fibrosis?

Answer 11

1. Interstitial lung damage
2. Fibrosis
3. Loss of elasticity

Question 12

What are the types of pulmonary fibrosis?

Answer 12

Replacement fibrosis secondary to lung damage - eg, infarction, tuberculosis and pneumonia.

Focal fibrosis in response to irritants - eg, coal dust and silica.

Diffuse parenchymal lung disease (DPLD), which occurs in idiopathic pulmonary fibrosis (IPF) and hypersensitivity pneumonitis.

Question 13

What aer key pathological features in Idiopathic pulmonary fibrosis?

Answer 13

1. Patchy fibrosis of the interstitium
2. Subpleural and paraseptal changes
3. Minimal or absent inflammation
4. Acute fibroblastic proliferation
5. Collagen depositions and honeycombing

Question 14

What does a respiratory function test in Idiopathic pulmonary fibrosis show?

And blood gas?

Answer 14

restrictive ventilatory defect

ABG shoes hypoxia

Question 15

What is the median survival time for idiopathic pulmonary fibrosis?

Answer 15

5 years