pulmonary hypertension in sarcoidosis

Last reviewed 01/2018

Pulmonary hypertension is a well described complication associated with an increased risk of mortality (1).

  • the incidence in a large Japanese study has been estimated to be around 5% (1)
  • it is elevated in 6 to 23% of patients at rest and in 43% with exertion (2)

The mechanism for pulmonary hypertension can be due to

  • fibrosis - the most common mechanism which results in obliteration of the pulmonary vessels
  • external compression from the enlarged lymphnodes
  • granulomatous infiltration of the pulmonary arterioles

Sildenafil has been reported to significantly improve the haemodynamic parameters but the effectiveness of other pulmonary vasodilator therapy is currently unknown (1)

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