Case 01
This 65-year-old male has a 4-month history of a dry cough and exertional breathlessness.
i. Describe the appearances on HRCT
ii. What clinical features might you expect to find on examination?
iii. What are the associations and complications of this condition?
iv. What is the treatment?
Case 02
This 50-year-old male presented with gradually progressive dyspnoea over the course of 6 months. His mother had become unwell at that time, and he had started visiting her daily to look after her. Her chest X-ray (2a) is shown.
i. Describe the appearances seen on the HRCT (2b) of the lungs.
Answer Case 01
i. The HRCT axial cut demonstrates reticular opacities, traction bronchiectasis, and honeycombing in a subpleural and basal distribution. Honeycombing is recognized by the presence of one or more rows of clustered cysts (<5 mm in diameter) and implies a poor prognosis. The features are characteristic of idiopathic pulmonary fibrosis. HRCT is a very reliable method of making the diagnosis, and lung biopsy (which would demonstrate usual interstitial pneumonitis) is not required in most cases.
ii. The examination features would include digital clubbing (25–50% of cases) and fine end-inspiratory ‘Velcro-like’ crackles at the bases. The pansystolic murmur of tricuspid regurgitation, a raised jugular venous pressure, and peripheral oedema may herald the development of pulmonary hypertension.
iii. No cause for idiopathic pulmonary fibrosis has been discovered. It may, however, be associated with connective tissue diseases (e.g. rheumatoid arthritis, scleroderma and mixed connective tissue diseases) and in this context has a better prognosis. The condition is linked to an increased incidence of lung cancer and is a cause of respiratory failure and secondary pulmonary hypertension. The 5-year survival is worse than for many extrathoracic cancers, at 20–40%.
iv. The treatment options are limited. A combination of N-acetylcysteine, prednisolone, and azathioprine is recommended to reduce the rate of decline of lung function, although it does not improve mortality. Lung transplant is rarely performed for patients with idiopathic pulmonary fibrosis, since most patients present over the age of 60 (the cut-off for transplantation in the UK).
Answer Case 02
ii. Ask about exposure to any pets or unusual dusts or chemicals. In this case, the patient had had a cockatoo that he had been feeding since his mother had become unwell. Budgerigars and pigeons are common causes of this, as are the classical irritants encountered in farmer’s lung, bark-stripper’s lung, etc.
iii. EAA or hypersensitivity pneumonitis may be caused by exposure to a variety of dusts and antigens, and may be confirmed by avian precipitins in the serum and removal of the source, i.e. the bird. A careful occupational history is essential.
iv. Patients must be instructed to avoid the allergen or precipitant. Oral prednisolone may be used during the initial few weeks until the patient feels better, although there is no definite evidence on optimal dose and duration.
v. The differential diagnosis includes sarcoidosis, pneumoconiosis, ankylosing spondylosis, and TB.
This 65-year-old male has a 4-month history of a dry cough and exertional breathlessness.
i. Describe the appearances on HRCT
ii. What clinical features might you expect to find on examination?
iii. What are the associations and complications of this condition?
iv. What is the treatment?
Case 02
This 50-year-old male presented with gradually progressive dyspnoea over the course of 6 months. His mother had become unwell at that time, and he had started visiting her daily to look after her. Her chest X-ray (2a) is shown.
i. Describe the appearances seen on the HRCT (2b) of the lungs.
ii. What important
history would you obtain?
iii. What is the
likely diagnosis?
iv. How would you
treat this condition?
v. What is the
differential diagnosis of bilateral upper lobe shadowing?Answer Case 01
i. The HRCT axial cut demonstrates reticular opacities, traction bronchiectasis, and honeycombing in a subpleural and basal distribution. Honeycombing is recognized by the presence of one or more rows of clustered cysts (<5 mm in diameter) and implies a poor prognosis. The features are characteristic of idiopathic pulmonary fibrosis. HRCT is a very reliable method of making the diagnosis, and lung biopsy (which would demonstrate usual interstitial pneumonitis) is not required in most cases.
ii. The examination features would include digital clubbing (25–50% of cases) and fine end-inspiratory ‘Velcro-like’ crackles at the bases. The pansystolic murmur of tricuspid regurgitation, a raised jugular venous pressure, and peripheral oedema may herald the development of pulmonary hypertension.
iii. No cause for idiopathic pulmonary fibrosis has been discovered. It may, however, be associated with connective tissue diseases (e.g. rheumatoid arthritis, scleroderma and mixed connective tissue diseases) and in this context has a better prognosis. The condition is linked to an increased incidence of lung cancer and is a cause of respiratory failure and secondary pulmonary hypertension. The 5-year survival is worse than for many extrathoracic cancers, at 20–40%.
iv. The treatment options are limited. A combination of N-acetylcysteine, prednisolone, and azathioprine is recommended to reduce the rate of decline of lung function, although it does not improve mortality. Lung transplant is rarely performed for patients with idiopathic pulmonary fibrosis, since most patients present over the age of 60 (the cut-off for transplantation in the UK).
Answer Case 02
i. The HRCT (2b) shows diffuse ground-glass
shadowing with some areas of centrilobular nodularity. The chest X-ray (2a) shows
a mainly upper lobe nodular infiltrate.
ii. Ask about exposure to any pets or unusual dusts or chemicals. In this case, the patient had had a cockatoo that he had been feeding since his mother had become unwell. Budgerigars and pigeons are common causes of this, as are the classical irritants encountered in farmer’s lung, bark-stripper’s lung, etc.
iii. EAA or hypersensitivity pneumonitis may be caused by exposure to a variety of dusts and antigens, and may be confirmed by avian precipitins in the serum and removal of the source, i.e. the bird. A careful occupational history is essential.
iv. Patients must be instructed to avoid the allergen or precipitant. Oral prednisolone may be used during the initial few weeks until the patient feels better, although there is no definite evidence on optimal dose and duration.
v. The differential diagnosis includes sarcoidosis, pneumoconiosis, ankylosing spondylosis, and TB.
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