Pulmonary hypertension (PH) has gained more and more attention in the last years given the increased mortality and morbidity rate and in particular pulmonary arterial hypertension associated with autoimmune diseases (a specific subgroup of PH), entities with an increasing incidence and prevalence globally. Among these, systemic sclerosis is of particular interest, being the most common among connective tissue diseases (CTDs) with PH as a major complication. Other CTDs such as mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), primary Sjogren's syndrome, and dermatomyositis may also develop PH as a complication of these severe and complex conditions, which can have a major impact on prognosis and quality of life. PH has also been reported in rheumatoid arthritis and ankylosing spondylitis but further studies are necessary. Several studies have shown that the 3-year survival rate for patients with systemic sclerosis-associated with pulmonary hypertension (SSc-PH) is between 31% and 52%, while the 5-year survival rate is below <50%. Therefore, an early and accurate diagnosis as well as subsequent management of PH in these conditions is mandatory, since survival remains suboptimal despite treatment advances. The aim of this article is to provide an overview of different autoimmune diseases associated with PH and to provide an easy reference source on current best practices.
Auto-immune rheumatic diseases are characterised by high levels of inflammation that accelerate the development of atherosclerosis, increasing the cardiovascular risk. Direct evaluation of arteries such as of intima media thickness measurement is a simple test that asesses the cerebral and cardiovascular risk. In the initial stages, an increase of the intimal media thickness can be observed. Later on, atherosclerosis (plaques), stenosis and occlusion of the arteries can be identified. Indirect evaluation of the cardiovascular risk can be performed using serological markers - fibrinogen, C reactive protein, estimated sedimentation rate, total cholesterol, low density lipoproteins and high density lipoproteins, homocysteine levels etc. The presence of certain lifestyle factors that increase the risk, such as smoking, sedentarism and associated pathologies such as obesity, diabetes, hypertension were assesed in patients with rheumatoid arthritis. The aim of the study is to prove the relationship between anti-tumor necrosis factor alpha therapy effect on the cardiovascular risk in rheumatoid arthritis patients.
