Pulmonary Fibrosis

Question 1

Idiopathic Pulmonary fibrosis : Definition

Answer 1

Dysregulation of the repair process in the lungs due to an unknown cause results in fibrosis of the lung tissue

Question 2

Idiopathic Pulmonary fibrosis : Pathophysiology

Answer 2

• **Injury **to the alveolar line trigger an inflammatory response
• Fibroblasts are stimulated to proliferate into my fibroblasts which are fibroblasts with smooth muscle properties
• Myfibroblasts secrete collagen in response to help heal injury

Dysregulation of the normal repair process

1. • Due to unknown cause - over proliferation of fibroblasts and myofibroblasts
2. • Destroys alveoli structure
3. • Excess collagen accumulates within the interstitial space causing fibrosis
4. • Thicken the interstitial layer between the alveoli and capillaries
5. • Loss of elasticity of the lungs
6. • Reducing gas exchange } progressive dyspnea

Question 3

Idiopathic Pulmonary fibrosis : Risk factors

Answer 3

• Family history
• Cigarette smoking
• Advanced age
• Male sex

Question 4

Idiopathic Pulmonary fibrosis : Clinical features

Answer 4

1. Dyspnea on exertion
   * Most disabling and prominent symptoms
   * Progressive
   * Present > 6 months
2. Chronic dry cough
3. Weightloss , fatigue, malaise } common systemic symptoms
4. Clubbing } due to chronic hypoxia

Question 5

Idiopathic Pulmonary fibrosis : Clinical signs

Answer 5

End - expiratory bibasal crackles
* dry and velcro in quality
* Almost present 100% of the tie

Question 6

Idiopathic Pulmonary fibrosis : Investigation

Answer 6

1. High resolution CT - diagnostic
   * Reticulation, honey combing and bronchiectasis
2. Pulmonary function test
   * FEV1/FVC > 0.7 : Restrictive lung disease

Question 7

Idiopathic Pulmonary fibrosis : Management

Answer 7

1. First line :
   * Antifibrinolytic therapy Perfenidone or Nintedanib

2 . Smoking cessation + Pulmonary rehabilitation

3 . Home oxygen therapy; - Severely hypoxic Sat< 89% at rest or with exertion

Question 8

Idiopathic Pulmonary fibrosis : Acute exacerbation features

Answer 8

• Worsening dyspnea within < 30 days
• Chest CT - new ground-glass opacities or consolidation
  Without evidence of infection or other cause of lung injury

Question 9

Idiopathic Pulmonary fibrosis : Acute exacerbation : Management

Answer 9

High dose corticosteroid therapy for 4 weeks