Abstract
Pulmonary arterial hypertension (PAH) is a severe vascular complication of connective tissue diseases. In the context of systemic sclerosis (SSc), PAH is a devastating disease with a dramatic impact on prognosis and survival. Despite advances in early diagnosis and the development of new targeted treatments, SSc-related pulmonary arterial hypertension (SScPAH) represents the leading cause of death in SSc patients with reported poorer response in therapy and worse prognosis compared with idiopathic PAH. Recent findings indicate that factors accounting for these differences may include cardiac involvement, pronounced autoimmune and inflammatory response and pulmonary venous vasculature remodeling. Deeper understanding of the underlying pathogenic mechanisms of pulmonary vascular disorders in SScPAH may lead to novel therapeutic strategies which are currently under investigation and may improve the outcome of these patients, for whom our therapeutic armamentarium is not effective enough. In this article we attempt to critically analyze the factors contributing to the unique phenotype of SScPAH focusing on future challenges for the design of novel targeted treatments which may alter the natural history of the disease.
Keywords: Systemic sclerosis, pulmonary hypertension, drug treatments, vasodilators, antineoplastic drugs, connective tissue diseases, pulmonary vascular resistance, inflammatory infiltration, pulmonary interstitial fibrosis, neurohormones
Current Pharmaceutical Design
Title: Systemic Sclerosis-Related Pulmonary Hypertension: Unique Characteristics and Future Treatment Targets
Volume: 18 Issue: 11
Author(s): Theodoros Dimitroulas, Georgios Giannakoulas, Haralambos Karvounis, Lukas Settas and George D. Kitas
Affiliation:
Keywords: Systemic sclerosis, pulmonary hypertension, drug treatments, vasodilators, antineoplastic drugs, connective tissue diseases, pulmonary vascular resistance, inflammatory infiltration, pulmonary interstitial fibrosis, neurohormones
Abstract: Pulmonary arterial hypertension (PAH) is a severe vascular complication of connective tissue diseases. In the context of systemic sclerosis (SSc), PAH is a devastating disease with a dramatic impact on prognosis and survival. Despite advances in early diagnosis and the development of new targeted treatments, SSc-related pulmonary arterial hypertension (SScPAH) represents the leading cause of death in SSc patients with reported poorer response in therapy and worse prognosis compared with idiopathic PAH. Recent findings indicate that factors accounting for these differences may include cardiac involvement, pronounced autoimmune and inflammatory response and pulmonary venous vasculature remodeling. Deeper understanding of the underlying pathogenic mechanisms of pulmonary vascular disorders in SScPAH may lead to novel therapeutic strategies which are currently under investigation and may improve the outcome of these patients, for whom our therapeutic armamentarium is not effective enough. In this article we attempt to critically analyze the factors contributing to the unique phenotype of SScPAH focusing on future challenges for the design of novel targeted treatments which may alter the natural history of the disease.
Export Options
About this article
Cite this article as:
Dimitroulas Theodoros, Giannakoulas Georgios, Karvounis Haralambos, Settas Lukas and D. Kitas George, Systemic Sclerosis-Related Pulmonary Hypertension: Unique Characteristics and Future Treatment Targets, Current Pharmaceutical Design 2012; 18 (11) . https://dx.doi.org/10.2174/138161212799504704
DOI https://dx.doi.org/10.2174/138161212799504704 |
Print ISSN 1381-6128 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-4286 |
Call for Papers in Thematic Issues
"Tuberculosis Prevention, Diagnosis and Drug Discovery"
The Nobel Prize-winning discoveries of Mycobacterium tuberculosis and streptomycin have enabled an appropriate diagnosis and an effective treatment of tuberculosis (TB). Since then, many newer diagnosis methods and drugs have been saving millions of lives. Despite advances in the past, TB is still a leading cause of infectious disease mortality ...read more
Current Pharmaceutical challenges in the treatment and diagnosis of neurological dysfunctions
Neurological dysfunctions (MND, ALS, MS, PD, AD, HD, ALS, Autism, OCD etc..) present significant challenges in both diagnosis and treatment, often necessitating innovative approaches and therapeutic interventions. This thematic issue aims to explore the current pharmaceutical landscape surrounding neurological disorders, shedding light on the challenges faced by researchers, clinicians, and ...read more
Emerging and re-emerging diseases
Faced with a possible endemic situation of COVID-19, the world has experienced two important phenomena, the emergence of new infectious diseases and/or the resurgence of previously eradicated infectious diseases. Furthermore, the geographic distribution of such diseases has also undergone changes. This context, in turn, may have a strong relationship with ...read more
Melanoma and Non-Melanoma Skin Cancer Treatment: Standard of Care and Recent Advances
In this thematic issue, we aim to provide a standard of care of the diagnosis and treatment of melanoma and non-melanoma skin cancer. The editor will invite authors from different countries who will write review articles of melanoma and non-melanoma skin cancers. The Diagnosis, Staging, Surgical Treatment, Non-Surgical Treatment all ...read more
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
- Announcements
Related Articles
-
Dynamics of Diabetes and Obesity: An Alarming Situation in the Developing Countries in Asia
Mini-Reviews in Medicinal Chemistry Adipokines and Myokines: A Pivotal Role in Metabolic and Cardiovascular Disorders
Current Medicinal Chemistry Female Infertility and Cardiovascular Risk - A Hype or an Underestimated Reality?
Current Pharmaceutical Design Antiangiogenic Agents in the Treatment of HER2-Negative Metastatic breast Cancer: A Brief Review
Current Angiogenesis (Discontinued) Genetic Variability of Matrix Metalloproteinase Genes in Cardiovascular Disease
Current Topics in Medicinal Chemistry Heart Failure in Acute Ischemic Stroke
Current Cardiology Reviews A Cyclooxygenase-2 Inhibitor Reduces Vascular Wall Thickness and Ameliorates Cognitive Impairment in a Cerebral Small Vessel Diseases Rat Model
Current Alzheimer Research Editorial (Thematic Issue: Enzymatic Studies in Medicinal Chemistry of Neglected Diseases)
Current Protein & Peptide Science A Hopeful Prospect of Riociguat as a Soluble Guanylate Cyclase Stimulator for Management of Pressure Ulcers
Current Drug Discovery Technologies Subject index to volume 3
Current Molecular Medicine Treatment of Hypertension to Prevent Atrial Fibrillation
Current Pharmaceutical Design Mast Cells and Basophils: Trojan Horses of Conventional Lin- Stem/Progenitor Cell Isolates
Current Pharmaceutical Design Design of a Genetically Modified Soybean Protein Preventing Hypertension Based on an Anti-Hypertensive Peptide Derived from Ovalbumin
Current Medicinal Chemistry - Cardiovascular & Hematological Agents Dimethyl Sulfoxide as an Excitatory Modulator and its Possible Role in Cancer Pain Management
Inflammation & Allergy - Drug Targets (Discontinued) Pharmacokinetics of Topical Ocular Drug Delivery: Potential Uses for the Treatment of Diseases of the Posterior Segment and Beyond
Current Drug Metabolism Serelaxin: New Investigational Treatment in Acute Heart Failure
Cardiovascular & Hematological Agents in Medicinal Chemistry Oxidative Stress Targeting Amyloid Beta Accumulation and Clearance in Alzheimer’s Disease: Insight into Pathological Mechanisms and Therapeutic Strategies
Current Psychopharmacology Sex-Genetic Interaction in the Risk for Cerebrovascular Disease
Current Medicinal Chemistry Cerebrovascular Damage as a Cause for Alzheimers Disease
Current Neurovascular Research Peripheral Mononuclear Cell Rejuvenation for Senescence Surveillance in Alzheimer Disease
Current Pharmaceutical Design