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Pulmonary vasculitis

In this rare group of diseases there is inflammation of blood vessels (vasculitis) with collections of cells called granulomata. Later scarring develops. In the diseases listed below it is the small blood vessels which are affected.

  • Wegener’s granulomatosis; this mainly occurs in the lung, upper and lower airways, sinuses, kidney and skin. It may present with a discharge from the nose, nose bleeds, nose deformity and cough. There may also be blood in the sputum (occasionally a very large bleed into the lungs), upper airways obstruction, breathlessness and chest pain
  • Churg-strauss syndrome; this is particularly rare in children. It is strongly associated with allergic nasal problems, fever and severe asthma. In addition, nasal polyps, diarrhoea, bleeding into the gut, various skin rashes, high blood pressure and inflammation of the coat around the heart (percarditis) all may present. A high eosinophil white blood count (blood cell involved in allergic processes) is typical of this condition.
  • Microscopic polyangitis; typically this affects lungs and the kidneys. It may present with general tiredness, weight loss and coughing up lots of blood. It has a poor outlook and usually results in chronic kidney failure

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