Novel Treatment Approaches to Fibrosis in Scleroderma
Department of Internal Medicine III and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Krankenhausstrasse 12, 91054 Erlangen, Germany.Rheumatic Disease Clinics of North America (Impact Factor: 2.69). 03/2008; 34(1):145-59; vii. DOI: 10.1016/j.rdc.2007.12.003
The molecular mechanisms leading to tissue fibrosis were only poorly understood in the past, and even today the cause or trigger of systemic sclerosis is still unknown. Remarkable breakthrough findings have been obtained regarding the identification of key molecules, key cellular mechanisms, and key intracellular signaling cascades, which mediate the perpetuation of fibrosis rather than trigger it. These findings have true translational implications, because modifiers of these key mediators and key mechanisms are often in clinical use in other disease indications, such as cancer. This article summarizes the clinical and preclinical evidence of examples of these novel antifibrotic treatment approaches in systemic sclerosis, including stem cell transplantation, modifiers of transforming growth factor-beta1 signaling, intravenous immunoglobulins, tyrosine kinase inhibitors, and histone deacetylase inhibitors.
Article: Sklerodermie[Show abstract] [Hide abstract]
ABSTRACT: Scleroderma (synonyms: systemic sclerosis, systemic scleroderma) is a systemic disease which affects the skin as well as internal organs such as the lungs, gastrointestinal tract, kidneys, and the heart. Pathogenetically a distinction should be made between uncontrolled formation of extracellular matrix proteins (fibrosis) and vasculopathy. In addition to organ fibrosis, this leads to a clinical picture of vascular manifestations. These include fingertip ulcers, pulmonary arterial hypertension, and acute renal crisis. Localized forms of scleroderma, such as morphea, which do not involve organ complications, should be differentiated from systemic sclerosis. Due to its clinical heterogeneity and high rate of morbidity and mortality, systemic sclerosis poses an enormous diagnostic and therapeutic challenge in everyday clinical practice. This review article summarizes the current status of classification and epidemiology, pathogenesis, and the most important clinical manifestations such as interstitial fibrosis, pulmonary arterial hypertension, acute renal crisis, and peripheral vasculopathy and provides an overview of current and future treatment options.Der Internist 01/2010; 51(1):30-38. DOI:10.1007/s00108-009-2405-z · 0.31 Impact Factor
Article: SCLERODERMA.[Show abstract] [Hide abstract]
ABSTRACT: Scleroderma is a descriptive construct that defines a large family of diseases characterized clinically by thickening of the skin. Systemic sclerosis (SSc) is a complex disease affecting multiple organ systems and is the most representative and prevalent of this spectrum of disorders. Generalized subcutaneous morphea (GSM, morphea profunda) is a form of localized scleroderma that tends to spare the internal organs. We are presenting two cases illustrating the aforementioned clinical entities.Journal of the Indian Medical Association 06/1965; 44:484-6. DOI:10.1007/978-1-60761-296-4_18
- [Show abstract] [Hide abstract]
ABSTRACT: The pathogenesis of systemic sclerosis (SSc) is complex, and the final story is yet to be elucidated. The clinical heterogeneity of the disease, its various autoimmune and antibody profiles, its long course and tendency for spontaneous cure makes the design of clinical trials difficult. The overwhelming need in this disease is to diagnose it early and identify those patients who will benefit most from early, aggressive treatment. We attempt to review data from recent clinical trials and the lessons derived.Indian journal of dermatology, venereology and leprology 09/2008; 74(5):436-46. DOI:10.4103/0378-6323.44298 · 1.39 Impact Factor
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.