Question of the Week # 328

328) A 66 year old man is seen in your office for progressive shortness of breath on exertion for the past one year. He also reports dry cough. He denies any fever, hemoptysis or weight loss. He has no history of infections. He denies smoking. He has no other medical problems and has never been hospitalized.  On examination, he is afebrile, blood pressure 120/80 mm Hg, RR 18/min and Pulse 82/min. There are no palpable chest-wall masses or lymphadenopathy. On auscultation, crackles are heard all over the lung fields, more pronounced at lung bases. Extremities show mild digital clubbing.

A chest x-ray is shown below:

 

Which of the following is expected to be seen with this disease?

A) Young age at onset

B) Rapid progression

C) High Resolution CT scans showing ill-defined cysts and pleural plaques

D) Poor or no response to steroids

E) Obstructive pattern on Pulmonary function tests

16 Responses

  1. A

  2. D) Poor or no response to steroids

  3. c

  4. C and there is subpleural cyst at rul and also
    Numerous pleural plaques

  5. cccccccccccccccc

  6. c

  7. E

  8. answer is c

  9. e

  10. E ??

  11. C

  12. The answers should be C

  13. ccc

  14. D
    Idiopathic pulmonary fibrosis
    IPF affects both genders and is usually encountered in patients greater than 50 years of age. However there is a notable reported case of a young adult has been diagnosed with IPF.[8][9] There are many different statements about average survival time following first diagnosis. Symptoms are gradual in onset. The most common are progressive dyspnea (difficulty breathing), but also include dry cough, clubbing (a disfigurement of the fingers), and rales (a crackling sound in the lungs during inhalation, heard with a stethoscope).[6] It should be noted that these features are not specific for IPF and can occur in a wide variety of other pulmonary disorders.

    Diagnosis requires clinical findings compatible with interstitial lung disease in combination with either characteristic radiologic findings or a pathologic diagnosis of UIP on surgical lung biopsy. Generally, lung biopsy is only undertaken when its risks are outweighed by the potential benefits of identifying an alternative, treatable disease process. Establishing the diagnosis of IPF without a lung biopsy has been shown to be reliable when expert clinicians and radiologists concur that the presenting features are typical of IPF.[10] Based on this evidence, the 2002 ATS/ERS Multidisciplinary Consensus Statement on the Idiopathic Interstitial Pneumonias proposed the following criteria for establishing the diagnosis of IPF without a lung biopsy:[6]

    Major criteria (all 4 required):

    Exclusion of other known causes of interstitial lung disease (drugs, exposures, connective tissue diseases)
    Abnormal pulmonary function tests with evidence of restriction (reduced vital capacity) and impaired gas exchange (pO2, p(A-a)O2, DLCO)
    Bibasilar reticular abnormalities with minimal ground glass on high-resolution CT scans
    Transbronchial lung biopsy or bronchoalveolar lavage (BAL) showing no features to support an alternative diagnosis
    Minor criteria (3 of 4 required):

    Age > 50
    Insidious onset of otherwise unexplained exertional dyspnea
    Duration of illness > 3 months
    Bibasilar inspiratory crackles

    • Plain chest x-rays reveal decreased lung volumes, typically with prominent reticular interstitial markings near the lung bases. Honeycombing, a pattern of lung fibrosis characterized by multiple cystic spaces located at the bases of the lungs, is frequently seen in advanced cases. In less severe cases, these changes may not be evident on a plain chest film.

      High-resolution CT scans of the chest demonstrate fibrotic changes in both lungs, with a predilection for the bases and the periphery. The most charactersitic radiologic feature of IPF is honeycombing, often described as traction bronchiectasis. There may be ground glass opacities of the lungs but these changes are relatively minor in comparison with the fibrotic changes

  15. And also the cxr look like apical paraseptal emphysema

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