Lung Fibrosis (OSCE Guide)

4/03/2020

Lung Fibrosis (OSCE Guide)

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.

LUNG FIBROSIS – EXAMINATION

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).

GENERAL EXAMINATION

  • Shortness of breath – due to fibrosis itself
  • Peripheral Cyanosis (in advanced disease)
  • Probable Aetiology (mainly try to find any evidence of underlying rheumatological disease)Rheumatoid Arthritis – Rheumatoid nodules, Rheumatoid arthropathy
    1. SLE – Petechial rash, Butterfly rash, Alopecia
    2. Systemic Sclerosis – Tight & shiny skin, Atrophic nails
    3. Psoriasis – Psoriatic arthropathy (Nail pitting, subungual onycholysis)
    4. Ankylosing Spondylitis – Loss of lumbar lordosis
    5. Tuberculosis – Cachectic, Lymphadenopathy, Apical flattening
    6. Drug (steroid) Side effects – Cushingoid features (Steroid purpura, Moonlike face, Buffalo hump)

Out of above mentioned ones, 1-3 causes predominantly “Basal fibrosis” and 4-6 causes “Apical fibrosis”.

Basal Fibrosis Vs Apical Fibrosis

RESPIRATORY EXAMINATION

INSPECTION
  • Decreased chest movements are seen bilaterally.
  • In case of asymmetrical involvement, movement reduction will be seen localized.
PALPATION
  • Symmetrical reduction in chest expansion usually in both lower zones.
  • Reduced vocal fremitus.
  • Trachea is not deviated in symmetrical bilateral involvement (but may be deviated to the affected side in cases fibrosis is localized “Unilateral apical fibrosis seen in TB”).
PERCUSSION
  • Dull.
AUSCULTATION
  • Reduced Vocal Resonance.
  • Reduced-intensity of breath sounds.
  • Fine-end inspiratory basal crepitations in both lung fields is the classical finding of IPF. You have to listen very carefully, as these crackles (crepitations) are “very fine” at times.
  • If you hear “Rhonchi”, think of an alternative diagnosis, since it is not usually not associated with Lung fibrosis.

EXTENDED EXAMINATION

  • Look for loud second heart sound in Pulmonic area (Indicative of Pulmonary Hypertension)
  • Elevated JVP
  • Right ventricular heave (Cor Pulmonale)
  • Proximal Myopathy – (if steroid overuse is suspected)

LUNG FIBROSIS – CASE PRESENTATION 01

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.

LUNG FIBROSIS – CASE PRESENTATION 02

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)


FREQUENTLY ASKED QUESTIONS

1. What are the causes of interstitial lung disease?

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

2. What is Cryptogenic Fibrosing Alveolitis?

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.

3. What are the subtypes of Idiopathic Lung Fibrosis (IPF)?

1. Acute Interstitial Pneumonia (AIP) 2. Usual Interstitial Pneumonitis (UIP) 3. Non-Specific Interstitial Pneumonia (NSIP)

4. What are the rheumatological diseases associated with interstitial lung disease?

1. SLE 2. Rheumatoid Arthritis 3. Systemic Sclerosis 4. Ankylosing Spondylitis 5. Psoriasis

5. What are the vasculitic conditions associated with interstitial lung disease?

1. Polyarteritis Nodosa 2. Wegner’s Granulomatosis 3. Chrug-Strauss Syndrome 4. Goodpasture’s Syndrome

6. What are the drugs associated with interstitial lung disease?

. Methotrexate (MTX) 2. Amiodarone 3. Gold 4. Nitrofurantoin

7. What are the conditions causing a predominant apical fibrosis?

1. Tuberculosis 2. Allergic Bronchopulmonary Aspergillosis (ABPA) 3. Ankylosing Spondylitis 4. Psoriasis 5. Sarcoidosis 6. Radiation 7. Langerhans Cell Histiocytosis

8. What are the conditions causing a predominant basal fibrosis?

1. Idiopathic Pulmonary Fibrosis (IPF) 2. Rheumatic Arthritis 3. Drugs 4. Asbestosis

9. What are the respiratory causes of clubbing?

1. Interstitial Lung Disease 2. Lung CA 3. Bronchiectasis 4. Mesothelioma 5. Lung Abscess 6. Cystic Fibrosis

10. What are the radiological features seen in Chest X-ray in idiopathic pulmonary fibrosis?

• Bi-basal reticulonodular infiltrates • “Honeycomb” appearance in advanced cases

11. How would you investigate a patient with lung fibrosis?

• FBC, CRP, ESR • Chest X-ray, HRCT, MRI • Arterial Blood Gas (ABG) • Rheumatoid Factor, ANA, ANCA • Immunoglobulin levels

12. What are the complications of interstitial lung disease?

1. Respiratory Failure 2. Recurrent Pneumonia 3. Pulmonary Hypertension 4. Cor pulmonale 5. Lung CA

13. What type of respiratory failure will it cause?

Type 1 Respiratory Failure.

14. What are the basic principles of management in this patient?

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)

15. What are the immunosuppressive agents commonly used in these patients?

1. Prednisolone 2. Azathioprine 3. Methotrexate 4. Cyclophosphamide

16. What are the antifibrotic agents available?

1. Colchicine 2. D-Penicillamine

17. What are the surgical options available?

Lung transplantation.

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