systemic scleroderma

Summary

Summary: 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.

Top Publications

  1. ncbi Bosentan therapy for pulmonary arterial hypertension
    Lewis J Rubin
    Division of Pulmonary and Critical Care Medicine, University of California at San Diego, La Jolla 92037 1330, USA
    N Engl J Med 346:896-903. 2002
  2. pmc Systemic sclerosis: a prototypic multisystem fibrotic disorder
    John Varga
    Devision of Rheumatology, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA
    J Clin Invest 117:557-67. 2007
  3. ncbi Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study
    Eric Hachulla
    Hopital Claude Huriez, Lille, France
    Arthritis Rheum 52:3792-800. 2005
  4. pmc Changes in causes of death in systemic sclerosis, 1972-2002
    Virginia D Steen
    Georgetown University, Washington, DC 20007, USA
    Ann Rheum Dis 66:940-4. 2007
  5. pmc Identification of a SmD3 epitope with a single symmetrical dimethylation of an arginine residue as a specific target of a subpopulation of anti-Sm antibodies
    Michael Mahler
    Dr Fooke Laboratorien GmbH, Neuss, Germany
    Arthritis Res Ther 7:R19-29. 2005
  6. ncbi Interstitial lung disease in systemic sclerosis: a simple staging system
    Nicole S L Goh
    Royal Brompton Hospital and National Heart and Lung Institute, London, United Kingdom
    Am J Respir Crit Care Med 177:1248-54. 2008
  7. pmc Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach
    D Mukerjee
    Department of Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Ann Rheum Dis 62:1088-93. 2003
  8. ncbi Autocrine TGF-beta signaling in the pathogenesis of systemic sclerosis
    Hironobu Ihn
    Department of Dermatology and Plastic and Reconstructive Surgery, Graduate School of Medical and Pharmaceutical Sciences, Kumamoto University, Kumamoto, Japan
    J Dermatol Sci 49:103-13. 2008
  9. ncbi Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival
    Lilian Scussel-Lonzetti
    Hopital Notre Dame, Centre Hospitalier de l Universite de Montreal, Montreal, Quebec, Canada
    Medicine (Baltimore) 81:154-67. 2002
  10. ncbi Dysregulation of transforming growth factor beta signaling in scleroderma: overexpression of endoglin in cutaneous scleroderma fibroblasts
    A Leask
    FibroGen, Inc, South San Francisco, California 94080, USA
    Arthritis Rheum 46:1857-65. 2002

Detail Information

Publications360 found, 100 shown here

  1. ncbi Bosentan therapy for pulmonary arterial hypertension
    Lewis J Rubin
    Division of Pulmonary and Critical Care Medicine, University of California at San Diego, La Jolla 92037 1330, USA
    N Engl J Med 346:896-903. 2002
    ..The present trial investigated the effect of bosentan on exercise capacity in a larger number of patients and compared two doses...
  2. pmc Systemic sclerosis: a prototypic multisystem fibrotic disorder
    John Varga
    Devision of Rheumatology, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA
    J Clin Invest 117:557-67. 2007
    ....
  3. ncbi Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study
    Eric Hachulla
    Hopital Claude Huriez, Lille, France
    Arthritis Rheum 52:3792-800. 2005
    ....
  4. pmc Changes in causes of death in systemic sclerosis, 1972-2002
    Virginia D Steen
    Georgetown University, Washington, DC 20007, USA
    Ann Rheum Dis 66:940-4. 2007
    ..Survival of scleroderma has changed since the renal crisis treatment has become possible...
  5. pmc Identification of a SmD3 epitope with a single symmetrical dimethylation of an arginine residue as a specific target of a subpopulation of anti-Sm antibodies
    Michael Mahler
    Dr Fooke Laboratorien GmbH, Neuss, Germany
    Arthritis Res Ther 7:R19-29. 2005
    ..Thus, anti-SMP detection using ELISA represents a new serological marker with which to diagnose and discriminate between systemic autoimmune disorders...
  6. ncbi Interstitial lung disease in systemic sclerosis: a simple staging system
    Nicole S L Goh
    Royal Brompton Hospital and National Heart and Lung Institute, London, United Kingdom
    Am J Respir Crit Care Med 177:1248-54. 2008
    ..In interstitial lung disease complicating systemic sclerosis (SSc-ILD), the optimal prognostic use of baseline pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) is uncertain...
  7. pmc Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach
    D Mukerjee
    Department of Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Ann Rheum Dis 62:1088-93. 2003
    ..To determine the prevalence of systemic sclerosis associated pulmonary arterial hypertension (SScPAH), evaluate outcome, and identify predictors of mortality in a large patient cohort...
  8. ncbi Autocrine TGF-beta signaling in the pathogenesis of systemic sclerosis
    Hironobu Ihn
    Department of Dermatology and Plastic and Reconstructive Surgery, Graduate School of Medical and Pharmaceutical Sciences, Kumamoto University, Kumamoto, Japan
    J Dermatol Sci 49:103-13. 2008
    ..This review focuses on the possible role of autocrine TGF-beta signaling in the pathogenesis of SSc...
  9. ncbi Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival
    Lilian Scussel-Lonzetti
    Hopital Notre Dame, Centre Hospitalier de l Universite de Montreal, Montreal, Quebec, Canada
    Medicine (Baltimore) 81:154-67. 2002
  10. ncbi Dysregulation of transforming growth factor beta signaling in scleroderma: overexpression of endoglin in cutaneous scleroderma fibroblasts
    A Leask
    FibroGen, Inc, South San Francisco, California 94080, USA
    Arthritis Rheum 46:1857-65. 2002
    ....
  11. ncbi Autoantibodies in systemic sclerosis
    Virginia D Steen
    Department of Medicine, Georgetown University, Washington, DC 2007, USA
    Semin Arthritis Rheum 35:35-42. 2005
    ..To describe the clinical, laboratory, and prognostic features associated with the scleroderma-specific autoantibodies...
  12. pmc Serum TIMP-1, TIMP-2, and MMP-1 in patients with systemic sclerosis, primary Raynaud's phenomenon, and in normal controls
    S A Young-Min
    Department of Rheumatology, University of Newcastle upon Tyne, UK
    Ann Rheum Dis 60:846-51. 2001
    ..It is suggested that decreased serum levels of matrix metalloproteinases (MMPs) or increased levels of tissue inhibitors of matrix metalloproteinases (TIMPs) may account for this matrix accumulation...
  13. pmc Predictors of end stage lung disease in systemic sclerosis
    V Steen
    Georgetown University, Washington DC, USA
    Ann Rheum Dis 62:97-9. 2003
  14. pmc Persistent down-regulation of Fli1, a suppressor of collagen transcription, in fibrotic scleroderma skin
    Masahide Kubo
    Division of Rheumatology and Immunology and the Laboratory of Cancer Genomics, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    Am J Pathol 163:571-81. 2003
    ..Persistent down-regulation of Fli1 in scleroderma fibroblasts in vivo may directly contribute to uncontrolled matrix deposition in scleroderma skin...
  15. ncbi Presence of CD4+CD8+ double-positive T cells with very high interleukin-4 production potential in lesional skin of patients with systemic sclerosis
    Yann Parel
    Geneva University Hospital, and Geneva University School of Medicine, Geneva, Switzerland
    Arthritis Rheum 56:3459-67. 2007
    ..Since no direct comparison with T cells in normal skin has been performed previously, this study was undertaken to functionally characterize T cells in the skin of patients with early active SSc and in normal skin...
  16. pmc Constitutive thrombospondin-1 overexpression contributes to autocrine transforming growth factor-beta signaling in cultured scleroderma fibroblasts
    Yoshihiro Mimura
    Department of Dermatology, Faculty of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Am J Pathol 166:1451-63. 2005
    ..These results indicate that the constitutive overexpression of TSP-1 may play an important role in autocrine TGF-beta signaling and accumulation of ECM in scleroderma fibroblasts...
  17. ncbi Oxidative stress in scleroderma: maintenance of scleroderma fibroblast phenotype by the constitutive up-regulation of reactive oxygen species generation through the NADPH oxidase complex pathway
    P Sambo
    , Italy
    Arthritis Rheum 44:2653-64. 2001
    ..Our results also exclude O2-, H2O2, IL-1beta, TGFbeta1, PDGF-BB, IL-4, IL-6, TNFalpha, or CTGF as mediators of a positive, autocrine feedback mechanism of ROS generation...
  18. ncbi Identification of PLOD2 as telopeptide lysyl hydroxylase, an important enzyme in fibrosis
    Annemarie J van der Slot
    Department Tissue Repair, P O Box 2215, 2301 CE Leiden, The Netherlands
    J Biol Chem 278:40967-72. 2003
    ..These data underscore the significance of PLOD2 in fibrotic processes...
  19. ncbi Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome
    Demosthenes Bouros
    Royal Brompton Hospital, London, United Kingdom
    Am J Respir Crit Care Med 165:1581-6. 2002
    ..005). We conclude that NSIP is the histopathologic pattern in most patients with FASSc. However, outcome is linked more strongly to disease severity at presentation and serial DL(CO) trends than to histopathologic findings...
  20. ncbi Serum levels of a Th1 chemoattractant IP-10 and Th2 chemoattractants, TARC and MDC, are elevated in patients with systemic sclerosis
    Hidetaka Fujii
    Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan
    J Dermatol Sci 35:43-51. 2004
    ..Although abnormalities of various chemokines are detected in systemic sclerosis (SSc), there are few findings concerning Th1 or Th2 chemoattractants...
  21. pmc Rosiglitazone abrogates bleomycin-induced scleroderma and blocks profibrotic responses through peroxisome proliferator-activated receptor-gamma
    Minghua Wu
    Section of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago IL 60611, USA
    Am J Pathol 174:519-33. 2009
    ....
  22. ncbi Elevated serum BAFF levels in patients with systemic sclerosis: enhanced BAFF signaling in systemic sclerosis B lymphocytes
    Takashi Matsushita
    Kanazawa University Graduate School of Medical Science, Kanazawa, Japan
    Arthritis Rheum 54:192-201. 2006
    ..To determine serum levels of BAFF, a potent B cell survival factor, in patients with systemic sclerosis (SSc) and relate the results to the clinical features of SSc...
  23. ncbi Uncontrolled expression of vascular endothelial growth factor and its receptors leads to insufficient skin angiogenesis in patients with systemic sclerosis
    Oliver Distler
    Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, CH 8091 Zurich, Switzerland
    Circ Res 95:109-16. 2004
    ..Consequently, for therapeutic approaches aiming to improve tissue perfusion in these patients, a controlled expression and timely termination of VEGF signaling appears to be crucial for success of proangiogenic therapies...
  24. ncbi Analysis of systemic sclerosis in twins reveals low concordance for disease and high concordance for the presence of antinuclear antibodies
    Carol Feghali-Bostwick
    Division of Pulmonary, Allergy, and Critical Care Medicine and Simmons Center for Interstitial Lung Disease, University of Pittsburgh, 628 NW Montefiore, Pittsburgh, PA 15261, USA
    Arthritis Rheum 48:1956-63. 2003
    ..To examine concordance for systemic sclerosis (SSc) in monozygotic (MZ) and dizygotic (DZ) twins...
  25. ncbi Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension
    Mark H Williams
    Department of Cardiology, Royal Free Hospital, London, UK
    Eur Heart J 27:1485-94. 2006
    ....
  26. ncbi Gene profiling of scleroderma skin reveals robust signatures of disease that are imperfectly reflected in the transcript profiles of explanted fibroblasts
    Humphrey Gardner
    Biogen Idec, Cambridge, Massachusetts, USA
    Arthritis Rheum 54:1961-73. 2006
    ....
  27. ncbi Defective vasculogenesis in systemic sclerosis
    Masataka Kuwana
    Institute for Advanced Medical Research Keio University School of Medicine, Tokyo, Japan
    Lancet 364:603-10. 2004
    ..We investigated whether vasculogenesis is impaired in patients with systemic sclerosis...
  28. pmc Smad-independent transforming growth factor-beta regulation of early growth response-1 and sustained expression in fibrosis: implications for scleroderma
    Swati Bhattacharyya
    Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
    Am J Pathol 173:1085-99. 2008
    ....
  29. ncbi Serum concentrations of the CXC chemokines interleukin 8 and growth-regulated oncogene-alpha are elevated in patients with systemic sclerosis
    Shinobu Furuse
    Department of Dermatology, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan
    J Rheumatol 30:1524-8. 2003
    ....
  30. ncbi Mortality in systemic sclerosis: an international meta-analysis of individual patient data
    John P A Ioannidis
    Clinical Trials and Evidence Based Medicine Unit, Department of Hygiene and Epidemiology, University of Ioannina School of Medicine, Ioannina 45110, Greece
    Am J Med 118:2-10. 2005
    ..Studies on mortality associated with systemic sclerosis have been limited by small sample sizes. We aimed to obtain large-scale evidence on survival outcomes and predictors for this disease...
  31. ncbi Severe fibrosis and increased expression of fibrogenic cytokines in the gastric wall of systemic sclerosis patients
    Mirko Manetti
    University of Florence, Florence, Italy
    Arthritis Rheum 56:3442-7. 2007
    ..The present study was undertaken to evaluate the fibrotic process and the expression of fibrogenic cytokines in the gastric wall of SSc patients with gastroesophageal involvement...
  32. ncbi The bleomycin-induced scleroderma model: what have we learned for scleroderma pathogenesis?
    Toshiyuki Yamamoto
    Department of Dermatology, Tokyo Medical University, 6 7 1 Nishi shinjuku, 160 0023 Tokyo, Japan
    Arch Dermatol Res 297:333-44. 2006
    ..In this review, we introduce current insights into the pathogenesis of bleomycin-induced scleroderma, and discuss its contribution to our understanding of the pathogenesis of, and treatments for, human scleroderma...
  33. ncbi High N-terminal pro-brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis
    Y Allanore
    Rene Descartes University, Cochin Hospital, Assistance Publique Hopitaux de Paris, Paris, France
    Arthritis Rheum 58:284-91. 2008
    ..To evaluate predictors of pulmonary arterial hypertension (PAH) in a prospective cohort of patients with systemic sclerosis (SSc)...
  34. ncbi Matrix metalloproteinase 12-dependent cleavage of urokinase receptor in systemic sclerosis microvascular endothelial cells results in impaired angiogenesis
    Silvia D'Alessio
    University of Florence, Florence, Italy
    Arthritis Rheum 50:3275-85. 2004
    ....
  35. ncbi Exhaled nitric oxide in systemic sclerosis: relationships with lung involvement and pulmonary hypertension
    G Rolla
    Department of Scienze Biomediche e Oncologia Umana, University of Torino, and Ospedale Molinette Torino, Italy
    J Rheumatol 27:1693-8. 2000
    ..To measure nitric oxide (NO) concentration in exhaled air of patients with systemic sclerosis (SSc) and to investigate its relationships with lung involvement, complicated or not by pulmonary hypertension (PH)...
  36. ncbi Evidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies
    John D Reveille
    University of Texas Health Science Center, Houston 77030, USA
    Arthritis Rheum 49:399-412. 2003
  37. ncbi Constitutive phosphorylation of focal adhesion kinase is involved in the myofibroblast differentiation of scleroderma fibroblasts
    Yoshihiro Mimura
    Department of Dermatology, Faculty of Medicine, University of Tokyo, Tokyo, Japan
    J Invest Dermatol 124:886-92. 2005
    ..These results indicate that the constitutive phosphorylation of FAK, which is possibly because of the autocrine TGF-beta signaling, may play an important role in alphaSMA expression in scleroderma fibroblasts...
  38. pmc Identification of candidate genes in scleroderma-related pulmonary arterial hypertension
    Dmitry N Grigoryev
    Division of Allergy and Clinical Immunology, Johns Hopkins University, Baltimore, MD 21224, USA
    Transl Res 151:197-207. 2008
    ..Deciphering the role of genes involved in vascular remodeling and PAH development may reveal new treatment targets for this devastating disorder...
  39. ncbi Localized and systemic scleroderma
    A Hawk
    Department of Dermatology, University of Virginia School of Medicine, Charlottesville 22908-0718, USA
    Semin Cutan Med Surg 20:27-37. 2001
    ..b>Systemic scleroderma (sclerosis) can manifest as either limited or diffuse disease...
  40. ncbi Decreased CD161+CD8+ T cells in the peripheral blood of patients suffering from rheumatic diseases
    A Mitsuo
    Department of Rheumatology and Internal Medicine, Juntendo University School of Medicine, 2 1 1 Hongo, Tokyo, Japan
    Rheumatology (Oxford) 45:1477-84. 2006
    ....
  41. ncbi Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud's phenomenon to systemic sclerosis: a twenty-year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosi
    Martial Koenig
    Notre Dame Hospital, Centre Hospitalier de l Universite de Montreal, Montreal, Quebec, Canada
    Arthritis Rheum 58:3902-12. 2008
    ....
  42. ncbi Increased circulating concentrations of the counteradhesive proteins SPARC and thrombospondin-1 in systemic sclerosis (scleroderma). Relationship to platelet and endothelial cell activation
    Richard F Macko
    Department of Neurology, VA Maryland Health Care Center, Baltimore 21201, USA
    J Rheumatol 29:2565-70. 2002
    ..The relationship of these counteradhesive proteins to measures of platelet and endothelial cell activation was examined...
  43. ncbi Systemic sclerosis Th2 cells inhibit collagen production by dermal fibroblasts via membrane-associated tumor necrosis factor alpha
    Carlo Chizzolini
    Division of Immunologya and Allergy, Geneva University Hospital, Geneva, Switzerland
    Arthritis Rheum 48:2593-604. 2003
    ....
  44. ncbi Anti-topoisomerase I (anti-Scl-70) antibodies in patients with systemic lupus erythematosus
    H A Gussin
    University of Illinois College of Medicine, Chicago 60612, USA
    Arthritis Rheum 44:376-83. 2001
    ..CONCLUSION: Anti-Scl-70 antibody is present in a significant subset of patients with SLE. For this subset, it offers a good correlate of disease activity and suggests increased risk for pulmonary hypertension and nephritis...
  45. ncbi The role of chemokines in the pathogenesis of scleroderma
    Sergei P Atamas
    Baltimore Veterans Administration Medical Center, Department of Medicine, University of Maryland School of Medicine, Baltimore, Maryland 21201, USA
    Curr Opin Rheumatol 15:772-7. 2003
    ..This review focuses on chemokines and their contribution to tissue fibrosis and pulmonary hypertension in scleroderma...
  46. ncbi Interleukin 4 and prolonged hypoxia induce a higher gene expression of lysyl hydroxylase 2 and an altered cross-link pattern: important pathogenetic steps in early and late stage of systemic scleroderma?
    J Brinckmann
    Department of Dermatology, University of Lubeck, Ratzeburger Allee 160, 23538 Lubeck, Germany
    Matrix Biol 24:459-68. 2005
    The major pathological processes of systemic scleroderma (SSc) comprise inflammation and microvascular damage in the early or acute progressive stage as well as tissue fibrosis and hypoxia in the chronic end stage...
  47. ncbi Low levels of nitric oxide (NO) in systemic sclerosis: inducible NO synthase production is decreased in cultured peripheral blood monocyte/macrophage cells
    Y Allanore
    Department of Rheumatology A, Cochin Hospital, , Paris 75014, France
    Rheumatology (Oxford) 40:1089-96. 2001
    ..Low levels of NO, a vasodilator, may be involved in vasospasm, which is critical in SSc. This may have therapeutic implications...
  48. ncbi The pathology of scleroderma vascular disease
    Jo Nadine Fleming
    Department of Pathology, 815 Mercer Street, Room 421, Brotman Building, Box 358050, University of Washington, Seattle WA 98109 4717, USA
    Rheum Dis Clin North Am 34:41-55; vi. 2008
    ....
  49. ncbi Animal model of sclerotic skin. III: Histopathological comparison of bleomycin-induced scleroderma in various mice strains
    T Yamamoto
    Department of Dermatology, Tokyo Medical and Dental University, School of Medicine, Japan
    Arch Dermatol Res 292:535-41. 2000
    ..Taken together, we conclude that C3H/He and B10.A mouse strains are bleomycin-'susceptible', and these strains are considered to be a suitable experimental model of bleomycin-induced scleroderma...
  50. ncbi Mechanisms of vascular damage in SSc--implications for vascular treatment strategies
    S Guiducci
    Department of Biomedicine, Division of Rheumatology, AOUC Florence, Italy
    Rheumatology (Oxford) 47:v18-20. 2008
    ....
  51. ncbi Class II HLA associations with autoantibodies in scleroderma: a highly significant role for HLA-DP
    F C Gilchrist
    Interstitial Lung Disease Unit, Imperial College at the National Heart and Lung Institute, London, UK
    Genes Immun 2:76-81. 2001
    ..0000003). We have identified a significant association between the ATA and HLA-DPB1*1301 which may provide an insight into how this autoantibody is formed. Patient clinical characteristics depend on the autoantibodies they carry...
  52. ncbi CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia
    Sujal R Desai
    Department of Radiology, King s College Hospital, Denmark Hill, London SE5 9RS, England
    Radiology 232:560-7. 2004
    ....
  53. ncbi Cardiac involvement in systemic sclerosis assessed by tissue-doppler echocardiography during routine care: A controlled study of 100 consecutive patients
    Christophe Meune
    Hopital Cochin, Universite Paris Descartes, Assistance Publique Hopitaux de Paris, Paris, France
    Arthritis Rheum 58:1803-9. 2008
    ..To assess the prevalence of primary cardiac complications in a large population of patients with systemic sclerosis (SSc), using recently developed echocardiographic techniques...
  54. ncbi The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia
    Dong Soon Kim
    Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea
    Sarcoidosis Vasc Diffuse Lung Dis 19:121-7. 2002
    ....
  55. ncbi Induction of apoptosis and fibrillin 1 expression in human dermal endothelial cells by scleroderma sera containing anti-endothelial cell antibodies
    S Sohail Ahmed
    Boston University School of Medicine, Arthritis Center E 5, 715 Albany Street, Boston, MA 02118, USA
    Arthritis Rheum 54:2250-62. 2006
    ....
  56. ncbi Clinical association of serum interleukin-17 levels in systemic sclerosis: is systemic sclerosis a Th17 disease?
    Maki Murata
    J Dermatol Sci 50:240-2. 2008
  57. ncbi Increased serum interleukin 23 in patients with systemic sclerosis
    Kazuhiro Komura
    Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
    J Rheumatol 35:120-5. 2008
    ..We investigated serum IL-23 levels and their clinical association in patients with SSc...
  58. ncbi Assessment of disease severity and prognosis
    T A Medsger
    Division of Rheumatology and Clinical Immunology, University of Pittsburgh, School of Medicine, 3471 Fifth Avenue, Suite 502, Pittsburgh, PA 15213, USA
    Clin Exp Rheumatol 21:S42-6. 2003
    ..It was decided to start with the assessment of severity using the Medsger's severity scale. A revised version of this scale was constructed. The rationale for the exclusion of other variables was provided...
  59. ncbi Platelet contributions to the pathogenesis of systemic sclerosis
    Arnold E Postlethwaite
    Division of Connective Tissue Diseases, Department of Medicine and Molecular Sciences, University of Tennessee Health Science Center, Memphis, Tennessee 38163, USA
    Curr Opin Rheumatol 19:574-9. 2007
    ..There have also been recent advances in characterization of the phenotype of platelets in systemic sclerosis which will be reviewed...
  60. ncbi Th2-oriented profile of male offspring T cells present in women with systemic sclerosis and reactive with maternal major histocompatibility complex antigens
    Cristina Scaletti
    Dipartimento di Medicina Interna, University of Florence, Viale Morgagni 85, Florence 50134, Italy
    Arthritis Rheum 46:445-50. 2002
    ..To characterize the cytokine production profile of male-offspring T cells reactive against maternal major histocompatibility complex (MHC) antigens present in the peripheral blood and/or skin from women with systemic sclerosis (SSc)...
  61. pmc Hypoxia induces expression of connective tissue growth factor in scleroderma skin fibroblasts
    K H Hong
    Department of Internal Medicine, Division of Rheumatology, School of Medicine, Catholic University of Korea, Seoul, Korea
    Clin Exp Immunol 146:362-70. 2006
    ....
  62. ncbi Hypoxia-induced increase in the production of extracellular matrix proteins in systemic sclerosis
    Jörg H W Distler
    Center of Experimental Rheumatology and Zurich Center of Integrative Human Physiology, University Hospital Zurich, Gloriastrasse 25, Zurich, Switzerland
    Arthritis Rheum 56:4203-15. 2007
    ..Based on the severely decreased oxygen levels in the skin of patients with SSc, we aimed to investigate the role of hypoxia in the pathogenesis of SSc...
  63. pmc Submaximal exercise testing in the assessment of interstitial lung disease secondary to systemic sclerosis: reproducibility and correlations of the 6-min walk test
    M H Buch
    University of Michigan Scleroderma Program, 3918 Taubman Centre, Box 0358, 1500 E Medical Center Drive, Ann Arbor, MI 48109 0358, USA
    Ann Rheum Dis 66:169-73. 2007
    ..Aim: To evaluate the usefulness of the 6MWT in a cohort of patients with ILD secondary to systemic sclerosis (SSc) and to correlate with established physiological parameters...
  64. ncbi Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study
    George E Tzelepis
    University of Athens Medical School, Laiko University Hospital, Athens, Greece
    Arthritis Rheum 56:3827-36. 2007
    ....
  65. pmc Serum nitric oxide (NO) levels in systemic sclerosis patients: correlation between NO levels and clinical features
    K Takagi
    Institute of Rheumatology, Tokyo Women s Medical University, Tokyo, Japan
    Clin Exp Immunol 134:538-44. 2003
    ....
  66. ncbi Cellular and molecular mechanisms of bleomycin-induced murine scleroderma: current update and future perspective
    Toshiyuki Yamamoto
    Department of Dermatology, Tokyo Medical and Dental University, School of Medicine, Tokyo, Japan
    Exp Dermatol 14:81-95. 2005
    ..This review summarizes recent progress of the cellular and molecular events in the pathogenesis of bleomycin-induced scleroderma; moreover, further perspective by using this mouse model has been discussed...
  67. ncbi Gene expression in bronchoalveolar lavage cells from scleroderma patients
    Irina G Luzina
    Research Service, Veterans Affairs Maryland Health Care System and Department of Medicine, University of Maryland School of Medicine, Baltimore, Maryland 21201, USA
    Am J Respir Cell Mol Biol 26:549-57. 2002
    ....
  68. pmc Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database
    U A Walker
    Department of Rheumatology, Basle University, Felix Platter Spital, Burgfelderstrasse 101, Basel 4012, Switzerland
    Ann Rheum Dis 66:754-63. 2007
    ..In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004...
  69. ncbi Nailfold videocapillaroscopic patterns and serum autoantibodies in systemic sclerosis
    M Cutolo
    Research Laboratory and Division of Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy
    Rheumatology (Oxford) 43:719-26. 2004
    ....
  70. ncbi Scleroderma overlap syndromes
    Janet E Pope
    Department of Medicine, Division of Rheumatology, University of Western Ontario, London, Canada
    Curr Opin Rheumatol 14:704-10. 2002
    ..A better prognosis is found with PM Scl antibody in myositis. Vasculitis is not a typical feature of scleroderma, but has been reported. Eosinophilic fasciitis is rare, and the onset could be associated with simvastatin...
  71. ncbi Systemic sclerosis in 3 US ethnic groups: a comparison of clinical, sociodemographic, serologic, and immunogenetic determinants
    J D Reveille
    Department of Medicine, Divisions of Rheumatology and Clinical Immunogenetics and General Medicine, The University of Texas Houston Health Science Center, Houston, TX 77030 USA
    Semin Arthritis Rheum 30:332-46. 2001
    ....
  72. ncbi Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial
    D B Badesch
    Division of Pulmonology, University of Colorado, Denver 80262, USA
    Ann Intern Med 132:425-34. 2000
    ..Pulmonary hypertension is a progressive and often fatal complication of the scleroderma spectrum of disease for which no treatment has been proven effective in a randomized trial...
  73. ncbi Assessment of vascular involvement
    B Kahaleh
    Division of Rheumatology and Immunology, Medical College of Ohio, USA
    Clin Exp Rheumatol 21:S9-14. 2003
    ..The subcommittee also however recognizes that there exists a promising set of vascular variable that still needs further investigation...
  74. pmc Molecular subsets in the gene expression signatures of scleroderma skin
    Ausra Milano
    Department of Genetics, Dartmouth Medical School, Hanover, New Hampshire, United States of America
    PLoS ONE 3:e2696. 2008
    ..Scleroderma is a clinically heterogeneous disease with a complex phenotype. The disease is characterized by vascular dysfunction, tissue fibrosis, internal organ dysfunction, and immune dysfunction resulting in autoantibody production...
  75. ncbi B lymphocytes and systemic sclerosis
    Manabu Fujimoto
    Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan
    Curr Opin Rheumatol 17:746-51. 2005
    ..This review focuses on recent advances in understanding the B cell's role in systemic sclerosis...
  76. ncbi Cellular origins of fibroblasts: possible implications for organ fibrosis in systemic sclerosis
    Arnold E Postlethwaite
    Division of Connective Tissue Diseases, University of Tennessee Health Science Center, 956 Court Avenue, Room G326, Memphis, TN 38163, USA
    Curr Opin Rheumatol 16:733-8. 2004
    ..These sources of fibroblasts may have importance in systemic sclerosis (scleroderma)...
  77. ncbi Curcumin-induced apoptosis in scleroderma lung fibroblasts: role of protein kinase cepsilon
    Elena Tourkina
    Department of Medicine, Medical University of South Carolina, Charleston, SC 29425, USA
    Am J Respir Cell Mol Biol 31:28-35. 2004
    ..These observations suggest that curcumin may have therapeutic value in treating scleroderma, just as it has already been shown to protect rats from lung fibrosis induced by a variety of agents...
  78. ncbi Myofibroblasts and hyalinized collagen as markers of skin disease in systemic sclerosis
    Eugene Y Kissin
    Boston University Medical Center, Boston, Massachusetts, USA
    Arthritis Rheum 54:3655-60. 2006
    ..To investigate the correlation between the degree of dermal fibrosis and myofibroblast infiltration using clinical assessments of skin thickness and hardness in systemic sclerosis (SSc)...
  79. pmc Elevated matrix metalloproteinase-9 in patients with systemic sclerosis
    Wan Uk Kim
    Division of Rheumatology, Department of Internal Medicine, Catholic Research Institutes of Medical Science, School of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
    Arthritis Res Ther 7:R71-9. 2005
    ..These findings suggest that the enhanced production of MMP-9 may contribute to fibrogenic remodeling during the progression of skin sclerosis in SSc...
  80. ncbi Soluble adhesion molecules (sVCAM-1, sE-selectin), vascular endothelial growth factor (VEGF) and endothelin-1 in patients with systemic sclerosis: relationship to organ systemic involvement
    Anna Kuryliszyn-Moskal
    Department of Rheumatology and Internal Diseases, Medical University of Bialystok, M C Sklodowskiej 24a, 15 276 Bialystok, Poland
    Clin Rheumatol 24:111-6. 2005
    ....
  81. pmc Impaired Smad7-Smurf-mediated negative regulation of TGF-beta signaling in scleroderma fibroblasts
    Yoshihide Asano
    Department of Dermatology, Faculty of Medicine, University of Tokyo, Tokyo, Japan
    J Clin Invest 113:253-64. 2004
    ..To our knowledge, this is the first report of a disturbed negative regulation of TGF-beta signaling in fibrotic disorders...
  82. ncbi Increased nitric oxide production in patients with systemic sclerosis
    A Sud
    Department of Internal Medicine, Rheumatology Unit, Post Graduate Institute of Medical Education and Research, Chandigarh, India 160012
    Nitric Oxide 4:615-9. 2000
    ..07). Hence, there is evidence of increased NO production in patients with SSc. There is no difference between NO levels in disease subgroups or those with systemic involvement...
  83. ncbi Possible implication of the effector CD4+ T-cell subpopulation TH17 in the pathogenesis of systemic scleroderma
    Bent Deleuran
    Department of Rheumatology, Aarhus University Hospital, Denmark
    Nat Clin Pract Rheumatol 3:682-3. 2007
  84. pmc Therapeutic targets in systemic sclerosis
    Christopher P Denton
    Centre for Rheumatology, Royal Free and University College Medical School, Rowland Hill Street, London, NW3 2PF, UK
    Arthritis Res Ther 9:S6. 2007
    ..It remains to be determined whether the identification of additional mediators merely furthers our knowledge of the natural history of SSc or presents targets that can be manipulated to manage SSc patients effectively...
  85. ncbi Need for improved outcome measures in pulmonary arterial hypertension related to systemic sclerosis
    O Distler
    Rheumatology (Oxford) 45:1455-7. 2006
  86. ncbi Interstitial lung disease in the patient who has connective tissue disease
    Charlie Strange
    Division of Pulmonary and Critical Care Medicine, Medical University of South Carolina, 96 Jonathan Lucas Street, 812 CSB, Charleston, SC 29425, USA
    Clin Chest Med 25:549-59, vii. 2004
    ..Additionally, the challenge of finding the patient who has occult connective tissue disease in an interstitial lung disease clinic is discussed...
  87. ncbi Acute and sustained effects of dihydropyridine-type calcium channel antagonists on oxidative stress in systemic sclerosis
    Yannick Allanore
    Rheumatology A Departments, Paris V University, Assistance Publique Hopitaux de Paris, Cochin Hospital, Paris, France
    Am J Med 116:595-600. 2004
    ..To evaluate the potential antioxidant properties of dihydropyridine calcium channel antagonists in systemic sclerosis...
  88. ncbi High expression and autoinduction of monocyte chemoattractant protein-1 in scleroderma fibroblasts
    T Yamamoto
    Department of Dermatology, University of Cologne, Cologne, Germany
    Eur J Immunol 31:2936-41. 2001
    ..Moreover, increased responsiveness of scleroderma fibroblasts to MCP-1 could result in a continuation of the fibrotic response...
  89. ncbi Criteria for the classification of early systemic sclerosis
    E C LeRoy
    Department of Medicine, Medical University of South Carolina, Charleston 29425, USA
    J Rheumatol 28:1573-6. 2001
    ..We propose criteria for the early diagnosis and classification of systemic sclerosis that reflect the vascular and serological advances of the last 2 decades...
  90. ncbi Development of a CENP-A/CENP-B-specific immune response in a patient with systemic sclerosis
    Michael Mahler
    Institute of Molecular Genetics, University of Heidelberg, Heidelberg, Germany
    Arthritis Rheum 46:1866-72. 2002
    ..Whether the D-penicillamine treatment received by this patient was involved in the triggering of this cascade remains a matter of speculation...
  91. pmc CD19-dependent B lymphocyte signaling thresholds influence skin fibrosis and autoimmunity in the tight-skin mouse
    Eriko Saito
    Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan
    J Clin Invest 109:1453-62. 2002
    ..CD19 deficiency also inhibited IL-6 production by TSK/+ B cells. Thus, chronic B cell activation resulting from augmented CD19 signaling in TSK/+ mice leads to skin sclerosis possibly through IL-6 overproduction as well as autoimmunity...
  92. ncbi Systemic sclerosis-scleroderma
    U F Haustein
    Department of Dermatology, University of Leipzig, Germany
    Dermatol Online J 8:3. 2002
    ..Therapy involves immunomodulation as well as the targeting of blood vessel mechanics and fibrosis. Physical therapy and psychotherapy are also important adjunctive therapies in this multifactorial disease...
  93. ncbi A variant of acrokeratoelastoidosis in systemic scleroderma: report of 7 cases
    Shingo Tajima
    Department of Dermatology, National Defense Medical College, Tokorozawa, Saitama, Japan
    J Am Acad Dermatol 46:767-70. 2002
    We describe acrokeratoelastoidosis-like lesions on the palms of the patients with systemic scleroderma. Histology showed a focal hyperkeratosis with or without epidermal concavity, regular acanthosis, and hyalinization of collagen fibers ..
  94. pmc Lack of association between the protein tyrosine phosphatase non-receptor 22 (PTPN22)*620W allele and systemic sclerosis in the French Caucasian population
    J Wipff
    Rheumatology A Department, Cochin Hospital, Paris, France
    Ann Rheum Dis 65:1230-2. 2006
    ..38). The PTPN22*620W allele was also not associated with autoantibody patterns. Thus, the PTPN22*R620W polymorphism cannot be regarded as a genetic susceptibility factor for SSc in the French Caucasian population...
  95. pmc Radiological hand involvement in systemic sclerosis
    J Avouac
    Service de Rhumatologie A, Hopital Cochin, 27 rue du Faubourg Saint Jacques, 75014 Paris, France
    Ann Rheum Dis 65:1088-92. 2006
    ..The osteoarticular and soft tissue structures of the hand may be involved in systemic sclerosis (SSc), causing functional disability...
  96. ncbi Surfactant protein D (SP-D) and systemic scleroderma (SSc)
    M Maeda
    Departament of Dermatology, Gifu Prefectural Hospital, Japan
    J Dermatol 28:467-74. 2001
    We measured serum levels of SP-D in collagen diseases (110 cases) such as systemic scleroderma (SSc), scleroderma spectrum disorders (SSD), systemic lupus erythematodes (SLE), Sjogren syndrome (Sjs), dermatomyositis (DM), rheumatoid ..
  97. ncbi A comparison between anti-Th/To- and anticentromere antibody-positive systemic sclerosis patients with limited cutaneous involvement
    Ghaith M Mitri
    HealthStar Physicians, Jefferson City, Tennessee, USA
    Arthritis Rheum 48:203-9. 2003
    ..To compare anti-Th/To-positive and anticentromere antibody (ACA)-positive patients with limited cutaneous systemic sclerosis (lcSSc)...
  98. ncbi Angiotensin-converting enzyme I/D polymorphism and macrovascular disease in systemic sclerosis
    F Bartoli
    Department of Medicine Section of Rheumatology, Villa Monna Tessa, Viale Pieraccini 18, 50122 Firenze, Italy
    Rheumatology (Oxford) 46:772-5. 2007
    ..This study evaluates the relationship between intima-media thickness (IMT), ankle-brachial pressure measurements (ABPI) and ACE I/D polymorphism in SSc patients...
  99. ncbi Cartilage oligomeric matrix protein expression in systemic sclerosis reveals heterogeneity of dermal fibroblast responses to transforming growth factor beta
    G Farina
    Rheumatology Section, Department of Medicine, Boston University School of Medicine, Arthritis Center, Boston, Massachusetts 02118, USA
    Ann Rheum Dis 68:435-41. 2009
    ..To further characterise the response to TGFbeta in SSc, we investigated TGFbeta1 and COMP expression and myofibroblast staining in SSc skin...
  100. pmc Ultrastructural study of the muscle coat of the gastric wall in a case of systemic sclerosis
    L Ibba-Manneschi
    Ann Rheum Dis 61:754-6. 2002
  101. ncbi Autoantibodies in scleroderma and tightskin mice
    C Bona
    Mount Sinai Medical Center, New York
    Curr Opin Immunol 6:931-7. 1994
    ..In tightskin mice, the scleroderma syndrome is associated with autoimmunity, particularly autoantibodies interacting with scleroderma target antigens...

Research Grants69

  1. Development of a Nonpsychotropic Cannabinoid Agonist for the Treatment of Sclerod
    Robert B Zurier; Fiscal Year: 2012
    ..The proposed experiments will determine in animal models the efficacy and mechanisms of action responsible for the antifibrotic effects of ajulemic acid (AJA). ..
  2. Molecular Markers for Progression of Pulmonary Fibrosis in Systemic Sclerosis
    Shervin Assassi; Fiscal Year: 2013
    ..Furthermore, the accompanying mentoring and course work will prepare Dr. Assassi to become an independent and productive clinician scientist and a leader in rigorously designed and conducted translational research in rheumatic diseases. ..
  3. Autoimmunity Center of Excellence (ACE) at Stanford
    CHARLES GARRISON FATHMAN; Fiscal Year: 2013
    ..The Stanford ACE proposes clinical research projects that encompass three different autoimmune diseases (SSc, psoriatic arthritis and SJIA), and proposes to study the MoA of therapeutics for preventing or treating different Al diseases. ..
  4. IGFBP-5 and Hsp70 in Extracellular Matrix Homeostasis and Fibrosis
    Kristen L Veraldi; Fiscal Year: 2013
    ..End of Abstract) ..
  5. Anti-Fibrotic Therapy for Scleroderma/SSc
    Latha Paka; Fiscal Year: 2010
    ..The objective of this application is to evaluate a lead small- molecule drug candidate in two preclinical models of scleroderma/SSc to advance to SBIR Phase-II project with the ultimate objective of bringing our drug to clinical trials. ..
  6. TNF Family Members and Control of Fibrotic Disease
    Michael Croft; Fiscal Year: 2013
    ..By determining whether LIGHT and its receptors promote fibrosis in animal models, we will gain knowledge that might lead to ways to therapeutically target human fibrotic disease. ..
  7. A Novel Model of, and Pre-clinical Therapy for, Scleroderma Lung Disease
    Sergei P Atamas; Fiscal Year: 2012
    ..The results are likely to be beneficial for not only patients with scleroderma, but also patients with asthma, sepsis, and tuberculosis, in all of which IL-442 has been suggested to play a significant role. ..
  8. Using a MALDI-TOF System for SNP Analysis
    Nadereh Jafari; Fiscal Year: 2010
    ....
  9. Innate immunity in dermal fibrosis and systemic sclerosis
    ROBERT A LAFYATIS; Fiscal Year: 2013
    ....
  10. Predictive Ability of Gene Expression Signatures In Skin as SSc Biomarkers
    MONIQUE EVANGELINE HINCHCLIFF; Fiscal Year: 2013
    ....
  11. Scleroderma Subtyping from Fresh and Archived Biopsies using NextGen Sequencing
    Michael W Fanger; Fiscal Year: 2011
    ..The goal of this project is to simplify the currently complex sample preparation protocol while improving the robustness and decreasing the cost of the genetic analyses. ..
  12. Anti-Fibrotic Therapy for Scleroderma/SSc
    Latha Paka; Fiscal Year: 2013
    ..We will also evaluate the safety/toxicology profile of ANG3070, in order to assemble a complete IND-enabling preclinical data package to submit to FDA for advancing to human studies. ..
  13. 2013 Elastin, Elastic Fibers &Microfibrils Gordon Research Conference &Gordon R
    Zsolt Urban; Fiscal Year: 2013
    ..4) To encourage and mentor new trainees through the new Gordon Research Seminar toward career success in elastic fiber-related research and support them through travel and excellence awards. ..
  14. Cervical Dysplasia and Human Papillomavirus Infection in Rheumatoid Arthritis.
    SEOYOUNG CATHERINE KIM; Fiscal Year: 2013
    ..Both Divisions have valuable resources including space, equipment, and knowledgeable faculty including experts in RA research as well as pharmacoepidemiology to help me achieve my goals. ..
  15. Mechanisms of Increased Lung Fibrosis in Males
    ISELA CARIDAD VALERA; Fiscal Year: 2013
    ..End of Abstract) ..
  16. Immunological Mechanisms in Systemic Autoimmune Disease
    Ann Marshak-Rothstein; Fiscal Year: 2012
    ..This study could lead to significant insights into the factors that trigger the development of SSc and strategies that might prove useful in limiting the progression of disease in afflicted patients. ..
  17. PPAR-Gamma's Role in Aberrant Adipogenesis and Fibrosis in Systemic Sclerosis
    BENJAMIN DOUGLAS KORMAN; Fiscal Year: 2013
    ..This work will help me to develop the knowledge and skills necessary to become an independent investigator and ultimately a future leader in rheumatology and the field of SSc research. ..
  18. TNIP1 risk haplotypes and immune endophenotypes
    Peter K Gregersen; Fiscal Year: 2013
    ..We will focus our studies particularly on the potential involvement of TNIP1 regulation of nuclear receptor function in the PPAR/RAR family. ..
  19. Inflammation, fibrosis and myocardial function in schleroderma
    Dorothee Weihrauch; Fiscal Year: 2012
    ..excess cytokine production, fibrosis and subsequent myocardial dysfunction and heart failure in systemic scleroderma. Based on preliminary data we will use an ApoAl mimetic, D-4F, to improve fibrosis, myocardial function and ..
  20. Mechanisms of Tolerance to Renal Maternal Microchimerism
    ANNE MARGUERITE STEVENS; Fiscal Year: 2011
    ..Peptides blocking host- maternal cell interactions in vitro could potentially be used in vivo as specific treatments for SLE nephritis. ..
  21. MCRC for Rheumatic Diseases in African Americans
    Gary S Gilkeson; Fiscal Year: 2013
    ..abstract_text> ..
  22. Mineralization/Anti-Mineralization Networks in the Skin
    Jouni Uitto; Fiscal Year: 2013
    ....
  23. Mechanisms of Right Ventricular Dysfunction in PAH
    DAVID ALAN KASS; Fiscal Year: 2013
    ....
  24. Retinoic Acid Modulation for Scleroderma
    BERT J W M OEHLEN; Fiscal Year: 2012
    ..We propose here to also evaluate their activity in preclinica animal models of SSc. We thus aim to generate critical proof of concept data that could warrant their further preclinical and clinical development for SSc. ..
  25. Thyroid Epithelial Cell Hyperplasia: Mechanisms of Induction
    HELEN B MULLEN; Fiscal Year: 2012
    ....
  26. Identification of Novel Markers for Systemic Sclerosis Employing Proteomics
    Sergio A Jimenez; Fiscal Year: 2011
    ....
  27. Scleroderma Twin Study
    Carol A Feghali-Bostwick; Fiscal Year: 2013
    ..Our success will provide a pipeline of clinical investigators to continue the mission of treating patients and identifying te cause and cure for Scleroderma and related diseases. ..
  28. T helper 2 Inflammation &Severe Muscularization of Arteries in the Lungs.
    Gabriele Grunig; Fiscal Year: 2013
    ..An immune-pathology has been suspected in PAH associated with autoimmune diseases such as systemic scleroderma, viral, or parasitic diseases...
  29. Right Ventricular-Pulmonary Vascular Interactions in Pulmonary Hypertension
    Naomi C Chesler; Fiscal Year: 2013
    ..1 ..
  30. Post-9/11 Incidence of Systemic Autoimmune Diseases in the FDNY Cohort
    Mayris P Webber; Fiscal Year: 2013
    ..incidence of confirmed cases of SAID in the FDNY population from 9/11/2001 until 9/10/2013; and Aim 2: Perform a nested case-control study to estimate the effect size of WTC exposure on confirmed cases of all subtypes of SAID combined ..
  31. Health Values and Minimally Important Differences in Systemic Sclerosis
    Dinesh Khanna; Fiscal Year: 2011
    ....
  32. The Role of Plexin C1 in Scleroderma Related Interstitial Lung Disease
    Aditi Mathur; Fiscal Year: 2013
    ..If successful, these studies could lead to novel insights into the immunopathogenesis of pulmonary fibrosis that might one day allow Plexin C1-based therapeutics. ..
  33. Center for Research Translation in Scleroderma
    MAUREEN MAUREEN MAYES; Fiscal Year: 2010
    ..abstract_text> ..
  34. Role of Caveolin in Scleroderma Tissue Fibrosis and Vasculopathy
    Sergio A Jimenez; Fiscal Year: 2012
    ..abstract_text> ..
  35. Fibroblast TGF-beta/Signaling in Scleroderma: Modulation by PPAR-gamma
    John Varga; Fiscal Year: 2012
    ..The proposed studies will provide a better definition of fibrosis in SSc, the role of PPAR-g in regulating the response, and the potential clinical utility of therapies targeting PPAR-g in SSc. ..
  36. Interplay between Type I IFN and TGF-beta signaling in systemic sclerosis
    GIUSEPPINA ALESSANDRA FARINA; Fiscal Year: 2013
    ....
  37. THE ROLE OF IL-31 IN TH2 CYTOKINE-DRIVEN SYSTEMIC SCLEROSIS
    Satish K Madala; Fiscal Year: 2013
    ..The proposed research is significant, because completion of this study will increase our knowledge of the mechanisms causing SSc and will lead to better medical treatments, cure or prevention. ..
  38. Cadherin-11 Regulation Of Dermal Fribrosis And Macrophage Function
    Sandeep K Agarwal; Fiscal Year: 2013
    ....
  39. Mediators of Fibrosis in Scleroderma Skin and Lung
    Carol A Feghali-Bostwick; Fiscal Year: 2012
    ..Our studies will also generate new targets for the development of future therapies for the treatment of systemic sclerosis and other fibrotic diseases. ..
  40. NIAMS: CORT
    ROBERT A LAFYATIS; Fiscal Year: 2013
    ....
  41. Identifying the Gene for Scleroderma in Tsk/2 mice that model Human SSc Subsets
    Elizabeth P Blankenhorn; Fiscal Year: 2013
    ..Tsk2/+ mice be used as a good model for diffuse-proliferative SSc? ..
  42. Novel Rho Kinase Inhibitor for Systemic Sclerosis
    Susan C Wright; Fiscal Year: 2013
    ....
  43. Studies of HLA Region Genomics in Systemic Sclerosis and Ankylosing Spondyilitis
    Xiaodong Zhou; Fiscal Year: 2013
    ..This proposal will fulfill the mission of the FOA to study HLA region genomics in immune-mediated diseases. ..
  44. Exhaled NO and Oxidative Stress in Systemic Sclerosis Pulmonary Hypertension
    Paul M Hassoun; Fiscal Year: 2012
    ..By providing mechanistic insights, this research may also serve as a basis for the testing of novel therapeutic classes, such as anti-oxidants and/or NO donors. ..
  45. Rheumatic Diseases Research Core Centers
    ROBERT A LAFYATIS; Fiscal Year: 2013
    ..In addition, the common platform and clinical data collected across cores will provide a large, common database for understanding clinical-pathological associations. ..
  46. Translational Vascular Biology in Autoimmune Diseases
    MARK THOMAS GLADWIN; Fiscal Year: 2010
    ..The strength of our program resides in the balance between basic and clinical research and robust programs led by experienced investigators with longstanding proven records in mentoring the next generation of investigators. ..
  47. Study of the Genetic Basis for Fibrotic Diseases in a Mouse Model
    Weikuan Gu; Fiscal Year: 2013
    ..If successful, we will remove a significant bottleneck from the study of this important mouse model of fibrosis. ..
  48. T-helper cell cytokine profile in localized scleroderma
    Kathryn S Torok; Fiscal Year: 2013
    ..The resources available at the University of Pittsburgh combined with the mentorship and training supported through this application will provide me with the necessary foundation to accomplish these goals. ..
  49. SCLERODERMA-ASSOCIATED PAH
    Paul M Hassoun; Fiscal Year: 2011
    ..Completion of these studies will provide invaluable information that will guide future mechanistic and clinical studies designed to improve clinical outcomes in PAH-SSc. ..
  50. Cancer, Autoantigens, and Scleroderma Investigating the Connection
    AMI AALOK SHAH; Fiscal Year: 2013
    ....
  51. Two-Stage Genome-Wide Association Study in Systemic Sclerosis
    MAUREEN MAUREEN MAYES; Fiscal Year: 2012
    ....
  52. Investigation and Development of New Therapeutic Avenues for Scleroderma
    EMANUAL M MAVERAKIS; Fiscal Year: 2011
    ..Scleroderma is an autoimmune disease that does not respond to therapy. We will study the immune cells isolated from patients with scleroderma and attempt to identify new therapeutic targets for medications. ..
  53. International Workshop on Scleroderma Research
    ROBERT A LAFYATIS; Fiscal Year: 2013
    ..The scientific Committee includes a broad array of international scientists with expertise in the various pathogenic pathways and complications of the disease. Based on past success we anticipate another highly successful meeting. ..
  54. Purinergic Modulation of Dermal Fibrosis
    Edwin Sl Chan; Fiscal Year: 2011
    ....
  55. Development and Initial Validation of PROMIS GI Distress Scale
    Brennan Spiegel; Fiscal Year: 2013
    ..The narrative describes our evolving conceptual framework for Gl Distress, reviews preliminary data from our group regarding PRO measurement in Gl disorders, emphasizes our past experience using PROMIS. ..
  56. Stem Cell Transplantation for Treatment of Autoimmune Diseases
    Keith Sullivan; Fiscal Year: 2010
    ..abstract_text> ..
  57. Stem Cell Transplantation for Treatment of Autoimmune Diseases
    Keith M Sullivan; Fiscal Year: 2011
    ..abstract_text> ..
  58. MECHANISMS OF COLLAGEN ACCUMULATION IN DERMAL FIBROSES
    Jouni Uitto; Fiscal Year: 1990
    ..This information may allow us to develop pharmacologic means which could counteract these debilitating diseases by targeting the precise levels of aberration...
  59. A LINKAGE STUDY IN FAMILIAL PULMONARY FIBROSIS
    Mark Steele; Fiscal Year: 2004
    ....
  60. ORAL CYCLOPHOSPHAMIDE VS ORAL PLACEBO IN SSC ALVEOLITIS
    James Dauber; Fiscal Year: 2000
    ..abstract_text> ..
  61. ORAL CYCLOPHOSPHAMIDE VS ORAL PLACEBO IN SSC ALVEOLITIS
    Dean Schraufnagel; Fiscal Year: 2003
    ..abstract_text> ..
  62. ORAL CYCLOPHOSPHAMIDE VS ORAL PLACEBO IN SSC ALVEOLITIS
    Daniel Furst; Fiscal Year: 2001
    ..abstract_text> ..
  63. ORAL CYCLOPHOSPHAMIDE VS ORAL PLACEBO IN SSC ALVEOLITIS
    Charles Read; Fiscal Year: 2003
    ..abstract_text> ..
  64. Origin of the Dermal Fibroblast
    Stephen Clark; Fiscal Year: 2006
    ..abstract_text> ..
  65. IMMUNE MEDIATED ALTERATIONS IN CONNECTIVE TISSUE
    JOSEPH KORN; Fiscal Year: 2000
    ..The genes identified will be cloned and characterized. The third specific aim is to evaluate selective influences for clonal proliferation of fibroblasts including apoptosis and hypoxia. ..
  66. T Cells in the Pathogenesis of Systemic Sclerosis
    Chris Platsoucas; Fiscal Year: 2005
    ..d. To identify Thl, Th2, suppressor, anergic and CTL T-cell clones from those developed above, on the basis of expression patterns of their genes, using DNA array technology. ..
  67. Self-and Foreign-Lipids Presented by CD1 in Scleroderma
    Michael Vincent; Fiscal Year: 2003
    ..Just as previous work has focused on the role of CD1 a, b, and c in host defense against microbes, it is now possible, here, to begin to unravel the molecular basis for T cells autoreactive to self-lipids presented by CD1 a, b, and c. ..
  68. SCLERODERMA LUNG STUDY
    Mitchell Olman; Fiscal Year: 2001
    ..abstract_text> ..