Lung fibrosis (Interstitial Lung Disease) commonly appears at undergraduate examinations, as it is a relatively straightforward case. But just diagnosing it won’t help you to acquire full marks allocated for the case.
You have to diagnose it as Lung fibrosis, find the possible aetiology & complications and also try to find outside effects of the current treatment if you really want to impress your examiner. Quite often they will be the cases of Idiopathic Pulmonary Fibrosis (IPF) or Rheumatic Arthritis (RA) associated lung fibrosis.
Suspect lung fibrosis in any patient with finger clubbing. But remember, clubbing may be absent in some patients with lung fibrosis.
If your clinical findings are compatible with Lung Fibrosis, try to find the possible aetiology in your general examination.
If you found “bilateral basal fibrosis” with “no evidence of” Rheumatological disease (RA, SLE mainly) you can safely mention “Idiopathic Pulmonary Fibrosis (IPF)” as your first differential diagnosis at undergraduate level (although there are many other causes except in rheumatological origin – refer FAQs).
Out of above mentioned ones, 1-3 causes predominantly “Basal fibrosis” and 4-6 causes “Apical fibrosis”.
Basal Fibrosis Vs Apical Fibrosis
The patient is not breathless at rest and no evidence of peripheral cyanosis. He has finger clubbing, Swan neck deformity & Rheumatic nodules. No lymphadenopathy. There are cushingoid features like moonlike face and steroid purpura.
Chest expansion is equal but symmetrically reduced bilaterally. Percussion note is dull and vocal fremitus and resonance are reduced. Breath sounds are of reduced intensity and there are Fine-end inspiratory basal crepitations in base of the both lung fields.
Pulmonary component of the second heart sound is loud in intensity and there is parasternal heave.
My, probable diagnosis is bilateral basal lung fibrosis secondary to Rheumatoid Arthritis complicated with pulmonary hypertension and right heart failure. The presence of cushingoid features are suggestive of patient is on a high dose of steroids. I would like to extend my examination to look for tender hepatomegaly and proximal myopathy.
The patient is not breathless at rest and no evidence of peripheral cyanosis. He has finger clubbing, No lymphadenopathy. There are no features suggestive of underlying rheumatological disease like rheumatoid arthritis, SLE.
Chest expansion is equal but symmetrically reduced bilaterally. Percussion note is dull and vocal fremitus and resonance are reduced. Breath sounds are of reduced intensity and there are Fine-end inspiratory basal crepitations in base of the both lung fields.
Pulmonary component of the second heart sound is of normal intensity and there is no parasternal heave.
My, probable diagnosis is bilateral basal lung fibrosis secondary probably due to Idiopathic Lung Fibrosis (IPF)
1. Idiopathic Pulmonary Fibrosis (IPF) 2. Rheumatological diseases (RA, SLE, AS) 3. Infections – TB, Aspergillosis 4. Inhaled Agents – Asbestosis, Silicosis 5. Drugs – Methotrexate, Amiodarone 6. Vasculitis – Chrug-Strauss, Goodpasture’s
It is the other name for Idiopathic Pulmonary Fibrosis (IPF), a chronic progressive lung disease of unknown aetiology which is characterized by inflammation and fibrosis of lung parenchyma. It is diagnosed only when the other causes of lung fibrosis are excluded.
1. Acute Interstitial Pneumonia (AIP) 2. Usual Interstitial Pneumonitis (UIP) 3. Non-Specific Interstitial Pneumonia (NSIP)
1. SLE 2. Rheumatoid Arthritis 3. Systemic Sclerosis 4. Ankylosing Spondylitis 5. Psoriasis
1. Polyarteritis Nodosa 2. Wegner’s Granulomatosis 3. Chrug-Strauss Syndrome 4. Goodpasture’s Syndrome
. Methotrexate (MTX) 2. Amiodarone 3. Gold 4. Nitrofurantoin
1. Tuberculosis 2. Allergic Bronchopulmonary Aspergillosis (ABPA) 3. Ankylosing Spondylitis 4. Psoriasis 5. Sarcoidosis 6. Radiation 7. Langerhans Cell Histiocytosis
1. Idiopathic Pulmonary Fibrosis (IPF) 2. Rheumatic Arthritis 3. Drugs 4. Asbestosis
1. Interstitial Lung Disease 2. Lung CA 3. Bronchiectasis 4. Mesothelioma 5. Lung Abscess 6. Cystic Fibrosis
• Bi-basal reticulonodular infiltrates • “Honeycomb” appearance in advanced cases
• FBC, CRP, ESR • Chest X-ray, HRCT, MRI • Arterial Blood Gas (ABG) • Rheumatoid Factor, ANA, ANCA • Immunoglobulin levels
1. Respiratory Failure 2. Recurrent Pneumonia 3. Pulmonary Hypertension 4. Cor pulmonale 5. Lung CA
Type 1 Respiratory Failure.
1. Identify the aetiology and treat/prevent 2. Aggressive treatment of chest infections 3. Immunosuppressive therapy 4. Antifibrotic therapy 5. Long term oxygen therapy (LTOT)
1. Prednisolone 2. Azathioprine 3. Methotrexate 4. Cyclophosphamide
1. Colchicine 2. D-Penicillamine
Lung transplantation.