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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Respiratory Examination (OSCE Guide)

1/03/2020

Respiratory Examination (OSCE Guide)

Ideally, the patient should be examined in the sitting position. Position the patient before you begin your “Examination Proper”. Examination of respiratory system consists of all 4 conventional steps – namely inspection, palpation, percussion and auscultation preceded by relevant general examination.

But these 4 steps can (should) be done both anteriorly and posteriorly. Usually, the examiner will guide you at the exam from which side you may examine the patient due to time limitation. Most of the physical signs are easily detected when the examination is done from posterior aspect. Hence, unless the examiner specifically asked, always begin your examination from the posterior aspect and keep in mind to examine the patient anteriorly if the time permits.

Suspect an upper lobe pathology if an examiner commands you “You may examine anteriorly!”, he might be giving you a clue to the diagnosis.

Upper lobe pathologies are easier to detect when the patient is examined anteriorly. 

EXAMINATION PROPER

RESPIRATORY SYSTEM EXAMINATION – INSPECTION

1. CHEST DEFORMITIES

Carefully look for the shape of the chest and chest deformities. For this you have to inspect the patient from both anteriorly and posteriorly. Remember the normal chest is elliptical and bilaterally symmetrical in shape.

  • Barrel Chest – Seen in COPD
  • Harrison’s Sulcus – seen in Chronic Asthma
  • Pectus Carinatum
  • Pectus Excavatum
  • Scoliosis/ Kyphosis/ Kyphoscoliosis
2. SURGICAL SCARS

Look for surgical scars. Never miss it! If you detect the lobectomy or pneumonectomy scar, you have your case there.

3. RESPIRATORY PATTERN

Usual respiratory pattern in adults is thoracic. Abdominal type of breathing is seen in children. But when respiratory muscles are weak, adults may show predominant abdominal type of respiration.

4. RESPIRATORY RATE

Approximately try to count the respiratory rate. You will get it with experience. You are not going to count this for one minute, Not in your exam! The normal respiratory rate in an adult is 12-20 breaths per minute.

5. CHEST WALL MOVEMENTS

You can get an idea of chest wall movement (Chest expansion) in inspection. Look whether there is a reduction in chest movements in one side or both sides asking the patient to take a deep breath in and out. You can confirm your findings at the next step, “Palpation”.

RESPIRATORY SYSTEM EXAMINATION – PALPATION

1. CHEST EXPANSION

Chest expansion should be assessed in all three zones (Apex, Upper, Lower) of thorax both posteriorly (and anteriorly Ideally).

Have the patient seated erect with arms by the side. Stand directly behind the patient. First, gently grab the lower hemithorax on either side of the chest (with an equal amount of pressure) and bring your thumbs close together until they approximate each other in the midline. Have the patient slowly take a deep breath and expire. Assess the “Degree” of chest expansion & “Symmetry” of movement of each hemithorax simultaneously. Then repeat the technique over the upper chest and the apex.

Then if the time permits, repeat the whole process anteriorly, at least the apex. Expansion of the apex of the chest best felt anteriorly!

What is the Normal Chest Expansion?

Normal Chest Expansion is 2-5 inches and chest wall should move symmetrically. That is the distance between the two thumbs should be at least 5 cm and both thumbs should be equal distance apart from the midline.

What are Abnormal Findings?
  • Reduced Chest Expansion (if the distance between the two thumbs less than 5 cm)
  • Asymmetrical Chest Expansion (if one thumb remains close to the midline)

Half the times, your examination may be normal up to this point of the examination. But If you find unilateral reduction in chest expansion, then you know the abnormal side and you can significantly narrow down your possible diagnosis ie. Pleural effusion, Lung collapse, Pneumothorax, Unilateral Lung Fibrosis!

You can further narrow down the possibilities by next step of palpation (Tracheal deviation & Mediastinal Shift) which is explained below.
2. TRACHEAL DEVIATION

Have the patient seated and position yourself directly in front of the patient and look for any deviation of the trachea. Keep your index and ring fingers of the right hand on the sternal heads of each sternocleidomastoid and then gently palpate the trachea above downwards with your middle finger along tracheal rings feeling its direction.

Then compare the empty space on both sides of the trachea. If the empty space is more on one side, it means the trachea is deviated to the opposite side. Normally there is a slight deviation of the trachea to the right side.

What are Abnormal Findings?
  • Trachea is shifted towards the side of pathology in Lung collapse, Lung Fibrosis.
  • Trachea is shifted away from the side of the pathology in Pleural effusion, Pneumothorax.
  • Trachea is not deviated in Lung consolidation.

Trail’s Sign – It is the prominence of clavicular head of sternocleidomastoid muscle of the side in which trachea is deviated.

Try to identify tracheal deviation before you even touch it!
3. APEX BEAT

Look for deviation of the apex beat indicative of a “mediastinal shift” when there is a tracheal deviation.

4. VOCAL FREMITUS

It is the palpation of the vibrations transmitted on to the chest wall (from larynx through the lungs).
To look for vocal fremitus palpate each side of the chest wall using the ulnar border of your hand at least at three levels (Upper, Middle, Lower zones).

Ask the patient to say “ninety-nine” and feel the vibrations on the chest wall. Always compare both sides. Ideally, the sequence should be repeated anteriorly if the time permits.

What are Abnormal Findings?
  • Vocal Fremitus is increased in Lung consolidation (Pneumonia, Pulmonary infarct) and Lung Fibrosis.
  • Vocal Fremitus is decreased in Pleural effusion, COPD, Chronic Asthma, Pneumothorax and thick pleura.

You can confirm your findings of vocal fremitus at the auscultation when you do “Vocal Resonance”.

Vocal Resonance is the Better of the Two

RESPIRATORY SYSTEM EXAMINATION – PERCUSSION

1. PERCUSSION TECHNIQUE

Have the patient seated and approach from behind. Keep your middle finger of the left hand firmly over the intercostal spaces parallel to the ribs, with other four fingers lifted above, not touching the thoracic wall.

Then percuss (strike) the centre of the middle phalanx of the middle finger perpendicularly. Striking movement should be at the wrist joint, not the elbow. Striking finger should be taken off immediately to prevent dampening of the percussion note. Clavicles are percussed directly on the bone.

Always percuss bilaterally, comparing one side with the other. Always percuss from resonant area to dull (above downwards). Remember to percuss all three zones of bilateral chest wall, and ideally the whole process should be repeated anteriorly if time permits.

“Striking a surface over an air-filled cavity will produce a resonant sound, whereas striking a surface over a fluid / tissue filled cavity will produce a dull sound” That’s it!

The Physcis Behind the Percussion

2. PERCUSSION NOTES
  • Hyper-resonant – Pneumothorax
  • Resonant – Normal Lung
  • Dull – Consolidation, Lung Fibrosis
  • Stony Dull – Pleural Effusion
3. TIDAL PERCUSSION

This is done to “exclude elevated hemidiaphragm causing basal dullness” from other causes like Lung Fibrosis, Basal Pneumonia, Lung Collapse or even Pleural effusion.

Percuss along the midclavicular line from the 2nd intercostal space downwards. Normally upper level of the liver dullness is met at 5th intercostal space in right side. If you encounter basal dullness, ask the patient to take a deep breath in, and percuss again. If the percussion note becomes resonant (being dull previously), it is due to elevated hemidiaphragm.

RESPIRATORY SYSTEM EXAMINATION – AUSCULTATION

1. HOW TO AUSCULTATE?

Usually with the diaphragm of the stethoscope firmly placed over the chest wall. Examine all three zones, apices in both lung fields. Make sure the patient is breathing in and out (preferably through the mouth). Auscultate a bit laterally (avoid the medial 3cm from midline).

2. WHAT TO AUSCULTATE?

Carefully try to assess one by one, concentrate only on the specific component you are looking for.

  1. Vocal Resonance
  2. Breath sounds
    • Character
    • Intensity
  3. Added Sounds
    • Crepitations – Fine or Coarse, Phase of respiration (Biphasic, Early- Inspiratory, End Inspiratory etc)
    • Ronchi
    • Pleural rub
    • Stridor (rare in the exam!)
  4. Pulmonary component of second heart sound (P2)
3. VOCAL RESONANCE

Keep the diaphragm of your stethoscope on chest wall and ask the patient to say “ninety nine”. Repeat this process in all three lung zones bilaterally, both anteriorly and posteriorly. The findings and interpretations are similar to vocal fremitus, but this is more sensitive. (Hence some examiners might ask you to skip Vocal Fremitus in percussion).

4. BREATH SOUNDS & ADDED SOUNDS
What is Normal?

Normal breath sounds are low pitch vesicular in nature. There should be no added sounds. P2 should be of normal intensity.

What is Abnormal?
1. Breath Sounds – Abnormal Character
  • Bronchial Breathing – High pitched sound and inspiration & expiration duration are almost of the same duration. Three types of bronchial breathing are,
    • Tubular Bronchial Breathing – (Pneumonic Consolidation)
    • Cavernous Bronchial Breathing – (Lung Cavity)
    • Amphoric Bronchial Breathing – (Bronchopulmonary Fistula)

Whishpering Pectoriloquy – Useful techninque to confirm Lung Consolidation if you heard a patch of bronchial breathing.

You can confirm or exclude the presence of consolidation on dull patches you found during the Percussion
2. Breath Sounds – Abnormal Intensity
  • Absent Breath sounds – Pleural Effusion, Pneumothorax, Lung Collapse
  • Reduced Intensity – COPD, Asthma, Pleural Thickening
3. Crepitations (Crackles) – Added Sounds
  • Non-musical explosive interrupted sounds. Results from the collapse of peripheral airways on expiration.
  • Seen in Pulmonary Oedema, Bronchiectasis, Interstitial Lung Disease. It can be Fine or Corse in nature.
  • It can be Inspiratory (Early inspiratory, Mid inspiratory, End inspiratory), Expiratory or Biphasic in timing.
4. Ronchi – Added Sounds
  • Continous low picthed musical sounds. Occurs due to small airway obstruction.
  • Seen in Asthma, COPD, Bronchiectasis, Pneumonia.
  • It can be further subdivided to Monophonic & Polyphonic ronchi.
5. Pleural Rub – Added Sounds
  • Creaking or grating sounds. Occurs due to frictional resistance between two layers of inflamed pleura

If you are not sure, ask the patient to cough; crepitations & ronchi may disappear, but not the pleural rub!

Confirming a Pleural Rub
5. SECOND HEART SOUND (P2)

Most of the time students do miss this! Always exclude pulmonary hypertension secondary to chronic lung disease. To do this you just need to put your stethoscope over the pulmonary area and listen whether the second heart sound is louder or not. Be smart! Show the examiner that you are looking for possible complications of lung pathology.

If the second heart sound is loud, you may further extend your examination to look for signs of right heart failure to impress the examiner. If so, look for parasternal heave and tender hepatomegaly if time permits.

SAMPLE PRESENTATION – RESPIRATORY EXAMINATION

This average build patient is not breathless at rest. He is not plethoric or cyanosed and he has no finger clubbing, tar stains or lymphadenopathy. There is no ankle oedema.

There are no chest deformities or surgical scars. Chest expansion is normal bilaterally in all three zones. Trachea is not deviated and percussion note is resonant throughout both lung fields. Vocal fremitus and vocal resonance are normal. The breath sounds were heard in normal intensity and there were no added sounds over both lung fields. The pulmonary component of the second heart is not loud.

So, examination of the respiratory system is unremarkable in this patient.

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