Station 1: Pulmonary fibrosis

Clinical findings:

- breathless, oxygen support ie LTOT
- cyanosis
- bilateral clubbing
- purpura, moon fancies (from steroid)
- lung: symmetrical reduced expansion, dullness on percussion, fine inspiratory crackles
- look out for underlying aetiology:

• RA: symmetrical deforming polyarthropathy, rheumatoid nodule
• SLE: malas rash, alopecia
• polymyositis, dermatomyositis: tender muscle, proximal muscle weakness, heliotrope rashes gottron papules
• ankylosing spondylitis: stooped ? mark posture
• scleroderma: tight shiny face, sclerodactyly, telangiectasia

- assess for complications:

• pulmonary HTN: palpable P2,  left parasternal heave
• cardiac failure: raised JVP, LL oedema, bibasal crepitations

Aetiolgy:

1. Idiopathic - most common is UIP
2. Autoimmune causes: as above, acute causes like pulmonary vasculitis (Churg- Strauss, wegeners)
3. Sarcoidosis
4. Extrinsic allergic alveolitis
5. Drug induced: bleomycin, nitrofurantoin, amiodarone, MTX, cyclophosphamide
6. Radiation fibrosis
7. Occupational/environmetal exposure: asbestosis, silica, coal, beryllium
8. Allergic bronchopulmonary aspergillosis ( ABPA)

Investigation:

- FBC, renal panel, LFT, inflammatory markers,  serum calcium, ACE, ABG
- CXR: bilateral reticulonodular and interstitial shadowing
- ECG: RVH
- HRCT: traction bronchiectasis, basal honey combing, ground class changes
- Lung function test; restrictive defect, reduced lung volume, impaired diffusion capacity
-  serology: ANA, ENA profile, C3/C4, RF and anti- CCP, ANCA (c- ANCA for WG, p- ANCA for  churg- strauss)
- serum precipitins
- Histology
- BAL, VATS
- 2d echo, cardiac cath: for pulmonary hypertension
- 6 minute walk test (to assess functional status)

Management:

- multi-disciplinary approach (respi. rheumato, cardio, physio, OT)
- pharmacological:

• Oral steroid
• Immunosuppressant: cyclosporin, MMF, Azathioprine
• Anti-fibrinolytic: Pirphenidone, Nintedanib (inhibit VEGF)
• treatment of pulmonary HTN: prostanoids (Epoprostenol, Ilioprost), endothelin antagonist (Bosentan), phosphodiestarase-inhibitors (Sildenafil, Taladafil)

- vaccination (pneumococcal, flu)

- LTOT

- pulmonary rehab

- lung transplant 

The end