Sunday, 21 October 2007

Lung Fibrosis

Upper lung fibrosis:

1-S silicosis/sarcoidosis

2-C cool-worker pneumoconiosis

3-H histocytosis

4-A ankylosing spondylitis/ABBA

5-T TB

6-E extrinsic allergic alveolitis

Lower lung fibrosis:

1-B bronchiectasis

2-R rheumatoid arthritis

3-A asbestosis

4-S scleroderma

5-I idoipathic pul fibrosis/intrinsic fibrosing alveolitis

6-O others: drug, irradiation


look for clues:RA,ankylosing spondylitis,systemic sclerosis,drug history

look for signs of pulmonary hypertension ('a' wave in JVP,left parasternal heave,loud P2)

present the case as:interstitial lung disease as evidant by...,the most like eatiology is...

remember...Cryptogenic fibrosing alveolitis:

Disease of unknown cause, characterized by inflammatory cell infiltrate and pulmonary fibrosis.
Eventually lead to respiratory failure and cor pulmonale from pulmonary hypertension.
Associated with other autoimmune diseases.

Clinical features
Dry cough,
Exertional dyspnoea,
Malaise, Wt loss,
Finger clubbing,
Fine end inspiratory crackles.


  1. ABG; low Po2, high pco2
  2. High ESR.
  3. Autoimmune serology:ANA +ve in 30%, RF +ve on 10%
  4. CXR; bilateral lower zone reticulonidular shadowing, honeycomb lung.
  5. PFT; restrictive, reduce lung volumes and transfer factor
  6. Bronchalveolar lavage: (not required for diagnosis; however, findings help exclude other diseases such as infection, alveolar proteinosis, eosinophilic pneumonia, and malignancy.) ↑ lymphocytes (good response/prognosis) ↑ neutrophills/esinophills (poor response/prognosis)
  7. HRCT
  8. Lung Bx (ideo-assisted thoracoscopic lung biopsy)

Alternative; Cyclophosphamide
Prognosis; 50% 5 years survival

read more


  1. common question in this station:
    1-what occupations you would ask about
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    3-causes of basal fibrosis
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    5-findings in CT/spirometry

  2. Cyanosis
    This is a blue discoloration of the skin caused by 5 g or more of reduced haemoglobin per 100 ml of blood in the capillaries.
    Cyanosis may be central or peripheral

    Central cyanosis:
    Only occurs when the oxygen saturation of arterial blood is less than 85%.
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    1.Decreased PO2 of inspired air - high altitude
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    4.Right to left cardiac shunt - congenital cyanotic heart disease.

    Central cyanosis may be simulated by methaemoglobulinaemia and sulphaemoglobulinaemia. Also, a patient with Polycythemia may present with central cyanosis.

    Peripheral cyanosis:
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    2.Low cardiac output e.g. heart failure
    3.Vasoconstriction e.g. due to low ambient temperature, Raynaud's phenomenon
    4.Arterial obstruction e.g. atheroma
    5.Venous obstruction

  3. signs of pulmonary hypertension:
    1-raised JVP with a wave
    2-left parasternal heave
    3-right ventricular gallop rythm
    4-loud P2 and loud ejection click
    5-pansystolic murmur of TR
    6-early diastolic murmur in pul area-Graham steell
    look for hepatomegaly and pedal oedema.

  4. this patinet has interstsitail lung disease most likely crypogenic fibrosing alveolitis,there is no evidance of cor pulmonale.
    o/e,his respiratory rate is 20/min,
    there is clubbing but no evidance of cyanosis.
    the JVP is not elevated and there is no paplable cervical lymphadenopathy.
    the trachea appear to be central.
    chest wall expansion is reduced bilaterally and percussion notes were normal.
    auscultation reveals fine late inspiratory crakle at the bases which do not clear with coughing.

  5. According to the 2000 ATS/ERS international consensus statement, a correct clinical diagnosis of idiopathic pulmonary fibrosis must include all of the major criteria and at least 3 of the 4 minor criteria.

    Major criteria
    1.Exclusion of other known causes of intersitial lung diseases (eg, drug toxicities, environmental exposures, connective-tissue diseases)
    2.Abnormal pulmonary function study results that include evidence of restriction (reduced vital capacity often with an increased ratio of forced expiratory volume in 1 second to forced vital capacity) and impaired gas exchange
    3.Bibasilar reticular abnormalities with minimal ground-glass opacities on HRCT scan images
    4.Transbronchial lung biopsy or bronchoalveolar lavage findings showing no features to support an alternative diagnosis

    Minor criteria
    1.Patient older than 50 years
    2.Insidious onset of otherwise unexplained dyspnea upon exertion
    3.Duration of illness longer than 3 months
    4.Bibasilar, inspiratory crackles


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