Pulmonary hypertension in sarcoidosis

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)

Reference:

• 1. Grutters JC et al. Chapter 9 - Sarcoidosis. Eur Respir Mon 2009;46:126-154
• 2. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357(21):2153-65