Sarcoidosis is a multi-system disease with frequent pulmonary involvement. Development of sarcoidosis-associated pulmonary hypertension (SAPH), although relatively rare, is associated with significant morbidity and mortality. SAPH can be related to a number of different mechanisms including fibrotic lung disease, intrinsic vasculopathy, left heart disease, pulmonary veno-occlusive and thromboembolic disease, whilst rarer factors such as extrinsic pulmonary vascular compression, anaemia and liver disease can also play a role. The definitive diagnosis of SAPH requires right heart catheterisation (RHC), with prior screening for PH in this setting reliant on non-invasive investigations, each with certain advantages and drawbacks. Early diagnosis and careful phenotyping of SAPH is advisable and has important prognostic and therapeutic implications. Management for SAPH involves treatment of the underlying mechanism driving the PH including PAH drug therapy to target the vasculopathy (currently only based on evidence from small single-centred studies), optimisation of oxygen therapy, diuretics, immunosuppression and rarely interventional procedures. The onset of SAPH should prompt referral for lung transplantation. Urgent prospective randomised controlled trials on a multi-centre basis are needed to further understand the role of existing therapies and aim to improve the prognosis of this adverse complication of sarcoidosis.

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Pulmonary Hypertension Associated with Sarcoidosis

  • Alexander Liu,
  • Vasilis Kouranos,
  • Laura C. Price

摘要

Sarcoidosis is a multi-system disease with frequent pulmonary involvement. Development of sarcoidosis-associated pulmonary hypertension (SAPH), although relatively rare, is associated with significant morbidity and mortality. SAPH can be related to a number of different mechanisms including fibrotic lung disease, intrinsic vasculopathy, left heart disease, pulmonary veno-occlusive and thromboembolic disease, whilst rarer factors such as extrinsic pulmonary vascular compression, anaemia and liver disease can also play a role. The definitive diagnosis of SAPH requires right heart catheterisation (RHC), with prior screening for PH in this setting reliant on non-invasive investigations, each with certain advantages and drawbacks. Early diagnosis and careful phenotyping of SAPH is advisable and has important prognostic and therapeutic implications. Management for SAPH involves treatment of the underlying mechanism driving the PH including PAH drug therapy to target the vasculopathy (currently only based on evidence from small single-centred studies), optimisation of oxygen therapy, diuretics, immunosuppression and rarely interventional procedures. The onset of SAPH should prompt referral for lung transplantation. Urgent prospective randomised controlled trials on a multi-centre basis are needed to further understand the role of existing therapies and aim to improve the prognosis of this adverse complication of sarcoidosis.