Literature DB >> 19145444

[Systemic sclerosis. Objectives for the treatment].

B Maurer1, M Walder, R E Gay, S Gay, O Distler.   

Abstract

Systemic sclerosis (SSc) is a multi-systemic fibrotic disorder with worldwide distribution and high morbidity and mortality. Characteristic features of this disease are widespread vasculopathy, inflammation, autoimmunity, and fibrosis. The better clinical outcome in recent years is mainly due to better management of organ complications. To date, there is no approved specific therapy to prevent or slow down the overall progression of the disease. So far, conventional disease-modifying antirheumatic drugs (DMARDs) have had no major impact on the disease course and have not prolonged survival. Based on recent studies of molecular pathomechanisms and various animal models, key molecules of fibrogenesis and vasculopathy in SSc could be identified. Therefore, to date, we have to reconsider and redefine the objectives of our treatment strategies. In this article, we discuss current and future therapeutic concepts as well as the objectives of new treatment strategies and of the evaluation of diagnostic tools with respect to pulmonary arterial hypertension, lung fibrosis and skin/systemic fibrosis.

Entities:  

Mesh:

Year:  2009        PMID: 19145444     DOI: 10.1007/s00393-008-0362-x

Source DB:  PubMed          Journal:  Z Rheumatol        ISSN: 0340-1855            Impact factor:   1.372


  30 in total

1.  Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study.

Authors:  Eric Hachulla; Virginie Gressin; Loïc Guillevin; Patrick Carpentier; Elisabeth Diot; Jean Sibilia; André Kahan; Jean Cabane; Camille Francès; David Launay; Luc Mouthon; Yannick Allanore; Kiet Phong Tiev; Pierre Clerson; Pascal de Groote; Marc Humbert
Journal:  Arthritis Rheum       Date:  2005-12

2.  Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions.

Authors:  C P Denton; M Humbert; L Rubin; C M Black
Journal:  Ann Rheum Dis       Date:  2006-06-22       Impact factor: 19.103

3.  Successful treatment of resistant scleroderma-associated interstitial lung disease with rituximab.

Authors:  D McGonagle; A L Tan; J Madden; A C Rawstron; A Rehman; P Emery; S Thomas
Journal:  Rheumatology (Oxford)       Date:  2008-02-15       Impact factor: 7.580

Review 4.  The controversial role of tumor necrosis factor alpha in fibrotic diseases.

Authors:  Jörg H W Distler; Georg Schett; Steffen Gay; Oliver Distler
Journal:  Arthritis Rheum       Date:  2008-08

5.  Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension.

Authors:  Lionel Schachna; Thomas A Medsger; James H Dauber; Fredrick M Wigley; Neil A Braunstein; Barbara White; Virginia D Steen; John V Conte; Stephen C Yang; Kenneth R McCurry; Marvin C Borja; David E Plaskon; Jonathan B Orens; Allan C Gelber
Journal:  Arthritis Rheum       Date:  2006-12

6.  A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis.

Authors:  O Nadashkevich; P Davis; M Fritzler; W Kovalenko
Journal:  Clin Rheumatol       Date:  2005-10-14       Impact factor: 2.980

7.  Sitaxsentan for the treatment of pulmonary arterial hypertension: a 1-year, prospective, open-label observation of outcome and survival.

Authors:  Raymond L Benza; Robyn J Barst; Nazzareno Galie; Adaani Frost; Reda E Girgis; Kristin B Highland; Charlie Strange; Carol M Black; David B Badesch; Lewis Rubin; Thomas R Fleming; Robert Naeije
Journal:  Chest       Date:  2008-07-14       Impact factor: 9.410

8.  Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial.

Authors:  N Galiè; Lj Rubin; Mm Hoeper; P Jansa; H Al-Hiti; Gmb Meyer; E Chiossi; A Kusic-Pajic; G Simonneau
Journal:  Lancet       Date:  2008-06-21       Impact factor: 79.321

9.  Therapy for severe interstitial lung disease in systemic sclerosis. A retrospective study.

Authors:  V D Steen; J K Lanz; C Conte; G R Owens; T A Medsger
Journal:  Arthritis Rheum       Date:  1994-09

10.  Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis.

Authors:  A M Stupi; V D Steen; G R Owens; E L Barnes; G P Rodnan; T A Medsger
Journal:  Arthritis Rheum       Date:  1986-04
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