Biomaterial Database

Pulmonary arterial hypertension in systemic sclerosis. 2010

Sevdalina Lambova, and Ulf Müller-Ladner

Department for Propaedeutics of Internal Medicine, Clinic of Rheumatology, Medical University, Plovdiv, Bulgaria. sevdalina_n@abv.bg

Pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) is a complex clinical situation resulting from restricted flow through the pulmonary arterial circulation ending in increased pulmonary vascular resistance and right heart failure. PAH is a common and life-threatening complication in connective tissue diseases, specifically in SSc if not treated rapidly and adequately. Based on the emerging knowledge in SSc epidemiology by large scale patient cohorts such as EUSTAR, of PAH pathophysiology and advances in cardiopulmonary diagnostic techniques, several novel treatment approaches have been examined and have proceeded to licensing and daily use in the clinical practice. Amongst them are different endothelin receptor antagonists and PDE-5 inhibitors, but several other ideas are being currently pursued to improve the long-term outcome of the affected patients.

UI	MeSH Term	Description	Entries
D006973	Hypertension	Persistently high systemic arterial BLOOD PRESSURE. Based on multiple readings (BLOOD PRESSURE DETERMINATION), hypertension is currently defined as when SYSTOLIC PRESSURE is consistently greater than 140 mm Hg or when DIASTOLIC PRESSURE is consistently 90 mm Hg or more.	Blood Pressure, High,Blood Pressures, High,High Blood Pressure,High Blood Pressures
D006976	Hypertension, Pulmonary	Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES.	Pulmonary Hypertension
D011652	Pulmonary Circulation	The circulation of the BLOOD through the LUNGS.	Pulmonary Blood Flow,Respiratory Circulation,Circulation, Pulmonary,Circulation, Respiratory,Blood Flow, Pulmonary,Flow, Pulmonary Blood,Pulmonary Blood Flows
D011658	Pulmonary Fibrosis	A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.	Alveolitis, Fibrosing,Idiopathic Diffuse Interstitial Pulmonary Fibrosis,Fibroses, Pulmonary,Fibrosis, Pulmonary,Pulmonary Fibroses,Alveolitides, Fibrosing,Fibrosing Alveolitides,Fibrosing Alveolitis
D001783	Blood Flow Velocity	A value equal to the total volume flow divided by the cross-sectional area of the vascular bed.	Blood Flow Velocities,Flow Velocities, Blood,Flow Velocity, Blood,Velocities, Blood Flow,Velocity, Blood Flow
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001327	Autoimmune Diseases	Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides.	Autoimmune Disease,Disease, Autoimmune,Diseases, Autoimmune
D012595	Scleroderma, Systemic	A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.	Sclerosis, Systemic,Systemic Scleroderma,Systemic Sclerosis
D014655	Vascular Resistance	The force that opposes the flow of BLOOD through a vascular bed. It is equal to the difference in BLOOD PRESSURE across the vascular bed divided by the CARDIAC OUTPUT.	Peripheral Resistance,Total Peripheral Resistance,Pulmonary Vascular Resistance,Systemic Vascular Resistance,Peripheral Resistance, Total,Resistance, Peripheral,Resistance, Pulmonary Vascular,Resistance, Systemic Vascular,Resistance, Total Peripheral,Resistance, Vascular,Vascular Resistance, Pulmonary,Vascular Resistance, Systemic
D065627	Familial Primary Pulmonary Hypertension	Familial or idiopathic hypertension in the PULMONARY CIRCULATION which is not secondary to other disease.	Heritable Pulmonary Arterial Hypertension,Idiopathic Pulmonary Arterial Hypertension,Idiopathic Pulmonary Hypertension,Pph1 With Hht,Primary Pulmonary Hypertension,Pulmonary Hypertension, Primary, 1,Pulmonary Hypertension, Primary, 1, With Hereditary Hemorrhagic Telangiectasia,Pulmonary Hypertension, Primary, Dexfenfluramine-Associated,Pulmonary Hypertension, Primary, Fenfluramine-Associated,Hypertension, Idiopathic Pulmonary,Hypertension, Primary Pulmonary,Pulmonary Hypertension, Idiopathic,Pulmonary Hypertension, Primary