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
-
3-(1H-Pyrrol-2-yl)-2-oxazolidinones as Novel Monoamine Oxidase Type A Inhibitors
Medicinal Chemistry TRAIL as Biomarker and Potential Therapeutic Tool for Cardiovascular Diseases
Current Drug Targets The Therapeutic Role of Renin-Angiotensin System Blockers in Obesity- Related Renal Disorders
Current Clinical Pharmacology Identification of Genes for a Complex Trait: Examples from Hypertension
Current Pharmaceutical Biotechnology The Role of the Immune System in the Pathogenesis of Hypertension
Current Hypertension Reviews Endothelial Dysfunction: Methods of Assessment and Application to Hypertension
Current Pharmaceutical Design Ageing as a Trait de Union Between Diabetes and Dementia for Frailty
CNS & Neurological Disorders - Drug Targets HHT: A Rare Disease with A Broad Spectrum of Clinical Aspects
Current Pharmaceutical Design Psychosocial Risk Factors Related to Ischemic Heart Disease in Women
Current Pharmaceutical Design Monocyte Cyclooxygenase-2 Activity: A New Therapeutic Target for Atherosclerosis?
Current Drug Targets - Cardiovascular & Hematological Disorders Biology, Epidemiology, Clinical Aspects of Hepatocellular Carcinoma and the Role of Sorafenib
Current Drug Targets Vasopressin Antagonists as Anxiolytics and Antidepressants: Recent Developments
Recent Patents on CNS Drug Discovery (Discontinued) Multiplex Analysis of Cardiac Hypertrophic Signaling: Reduced in vivo Phosphorylation of Glycogen Synthase Kinase-3β and Proline-Rich Akt Substrate (PRAS40)
Current Signal Transduction Therapy Targeting Vascular Redox Biology Through Antioxidant Gene Delivery: A Historical View and Current Perspectives
Recent Patents on Cardiovascular Drug Discovery Editorial [Hot Topic: Recent Progress in the Development of Anti-Diabetic Drugs (Guest Editor: J. Eckel)]
Current Medicinal Chemistry - Immunology, Endocrine & Metabolic Agents Neuroimmune Aspects of Sjogren`s Syndrome: Role of VIP/VPAC System in Immune and Salivary Gland Epithelial Cell Function
Current Pharmaceutical Design Predementia and Dementia Syndromes: Possible Role of Lipoprotein Metabolism
Vascular Disease Prevention (Discontinued) The Impact of Pharmaceutical Care Practice on the Practitioner and the Patient in the Ambulatory Practice Setting: Twenty-five Years of Experience
Current Pharmaceutical Design The Vascular Compartments of Neovascularization: Spotlight on the Microcirculation
Current Pharmaceutical Biotechnology Physical Function and Exercise in Older Patients with Cardiovascular and Respiratory Conditions
Current Pharmaceutical Design