Showing posts with label Interstitial lung disease. Show all posts
Showing posts with label Interstitial lung disease. Show all posts

Sunday, September 20, 2015

Lung infection Case Collection 03-04

Case  03
Shown here is a swollen right ankle  and  diffuse  macular  rash  in  a young female with cystic fibrosis.

i. What is the diagnosis?
ii. What  is  the  medical  treatment  and prognosis?


Case  04
 17 This is the chest X-ray of a 16- year-old male who presented with a 3- week history of a dry cough and fevers.

i. What does the chest X-ray show?
ii. What is the likely diagnosis?
iii. What  contact  tracing  procedures should be instigated?

Answer Case 03

i.The illustration shows a diffuse vasculitis involving the skin and synovium. Vasculitis of the skin may also be nodular or purpuric (without thrombocytopenia). It is associated with severe pulmonary disease and chronic Pseudomonas aeruginosa infection. It has been attributed to an overspill into the systemic circulation of immune complexes resulting from the hyperimmune stimulation associated with chronic pulmonary disease.

ii. Medical  treatment  consists  of  short-term,  high-dose  oral  steroids.  This  will usually produce complete resolution. Immunosuppressive agents have been used, but experience with them is limited.

Answer Case 04
i. Unilateral enlarged paratracheal lymph nodes.
ii. Primary pulmonary TB. The initial infection with Mycobacterium tuberculosis in children with no prior history of exposure to the disease leads to a small primary focus of infection, usually in the middle to lower zones on the chest X-ray when visible, and associated with enlarged mediastinal nodes on the affected side. The mycobacteria disseminate into the blood from the enlarged nodes and can therefore be seeded throughout the body, but they have a predilection for areas of relatively high oxygen tension such as the apices of the lungs, the CNS, and the metaphyses of the long bones.

Primary disease causes symptoms in only a minority of patients (less than 10%), usually resolves spontaneously (often leaving a tuberculoma), and is associated with conversion to a positive tuberculin skin test after 6 weeks. However, primary disease can  be  complicated  by  progressive  local  disease  within  the  lungs,  bronchial obstruction by enlarged nodes (especially the middle lobe, leading to bronchiectasis), bronchogenic TB due to the rupture of a caseating node into the bronchial tree, pleural or pericardial effusions, miliary TB, and tuberculous meningitis.

iii. Contact tracing is initiated to identify (a) cases who have caught TB from the index case, (b) the potential source of infection for the index case, and (c) other patients  who  may  have  been  infected  by  this  source.  Recommendations  vary between countries but usually suggest the screening of all subjects who live with the index case and other close contacts such as school classmates.

Screening requires a clinical history, chest X-ray, and tuberculin skin testing (at least in children or those who have not been given the BCG vaccine). The new gamma-interferon blood tests for TB identify whether the patient has circulating lymphocytes that recognize an antigen specific for M. tuberculosis, and may provide a more specific and sensitive test for identifying infected patients than tuberculin skin testing.

Interstitial lung disease Case 01-02

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.
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.