Christopher P Denton

Summary

Affiliation: University College London
Country: UK

Publications

  1. doi Renal manifestations of systemic sclerosis--clinical features and outcome assessment
    C P Denton
    Centre for Rheumatology, Royal Free Hospital, London NW3 2QG, UK
    Rheumatology (Oxford) 47:v54-6. 2008
  2. pmc A multicenter study confirms CD226 gene association with systemic sclerosis-related pulmonary fibrosis
    Lara Bossini-Castillo
    Instituto de Parasitologia y Biomedicina Lopez Neyra, IPBLN CSIC, Avenida del Conocimiento s n, Granada, 18100, Spain
    Arthritis Res Ther 14:R85. 2012
  3. doi An open-label pilot study of infliximab therapy in diffuse cutaneous systemic sclerosis
    C P Denton
    Centre for Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Ann Rheum Dis 68:1433-9. 2009
  4. ncbi Therapy of pulmonary arterial hypertension in systemic sclerosis: an update
    Christopher P Denton
    Centre for Rheumatology, Pond Street, Royal Free Hospital, London, United Kingdom, NW3 2QG
    Curr Rheumatol Rep 9:158-64. 2007
  5. pmc Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions
    C P Denton
    Centre for Rheumatology, Royal Free Hospital, Pond Street, London, NW3 2QG, UK
    Ann Rheum Dis 65:1336-40. 2006
  6. ncbi Mechanisms and consequences of fibrosis in systemic sclerosis
    Christopher P Denton
    Centre for Rheumatology, Royal Free and University College Medical School, London, and Royal Free Hospital, UK
    Nat Clin Pract Rheumatol 2:134-44. 2006
  7. ncbi Recombinant human anti-transforming growth factor beta1 antibody therapy in systemic sclerosis: a multicenter, randomized, placebo-controlled phase I/II trial of CAT-192
    Christopher P Denton
    Royal Free Hospital, London, UK
    Arthritis Rheum 56:323-33. 2007
  8. ncbi Using a self-reported functional score to assess disease progression in systemic sclerosis
    K Serednicka
    Centre for Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Rheumatology (Oxford) 46:1107-10. 2007
  9. pmc Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases
    C P Denton
    Center for Rheumatology, Royal Free Hospital, Pond Street, Rheumatology Unit, Lower Ground Floor, London NW3 2QG, UK
    Ann Rheum Dis 67:1222-8. 2008
  10. pmc Systemic sclerosis-associated pulmonary hypertension: why disease-specific composite endpoints are needed
    Christopher P Denton
    Centre for Rheumatology, Royal Free Campus, University College Medical School, London, NW3 2PF, UK
    Arthritis Res Ther 13:114. 2011

Detail Information

Publications120 found, 100 shown here

  1. doi Renal manifestations of systemic sclerosis--clinical features and outcome assessment
    C P Denton
    Centre for Rheumatology, Royal Free Hospital, London NW3 2QG, UK
    Rheumatology (Oxford) 47:v54-6. 2008
    ....
  2. pmc A multicenter study confirms CD226 gene association with systemic sclerosis-related pulmonary fibrosis
    Lara Bossini-Castillo
    Instituto de Parasitologia y Biomedicina Lopez Neyra, IPBLN CSIC, Avenida del Conocimiento s n, Granada, 18100, Spain
    Arthritis Res Ther 14:R85. 2012
    ..The aim of this study was to test the influence of CD226 loci in SSc susceptibility, clinical phenotypes and autoantibody status in a large multicenter European population...
  3. doi An open-label pilot study of infliximab therapy in diffuse cutaneous systemic sclerosis
    C P Denton
    Centre for Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Ann Rheum Dis 68:1433-9. 2009
    ..The safety and potential efficacy of a chimaeric anti-tumour necrosis factor alpha monoclonal antibody (infliximab) were examined in diffuse cutaneous systemic sclerosis (dcSSc)...
  4. ncbi Therapy of pulmonary arterial hypertension in systemic sclerosis: an update
    Christopher P Denton
    Centre for Rheumatology, Pond Street, Royal Free Hospital, London, United Kingdom, NW3 2QG
    Curr Rheumatol Rep 9:158-64. 2007
    ..In recent years, publications of connective tissue disease subgroup analysis from large trials in PAH have emerged. We review the current treatment options for PAH and the evidence for their use in scleroderma-related PAH...
  5. pmc Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions
    C P Denton
    Centre for Rheumatology, Royal Free Hospital, Pond Street, London, NW3 2QG, UK
    Ann Rheum Dis 65:1336-40. 2006
    ..A frequent complication of CTD is pulmonary arterial hypertension (PAH), which has a major effect on functioning and quality of life, and is associated with a particularly poor prognosis...
  6. ncbi Mechanisms and consequences of fibrosis in systemic sclerosis
    Christopher P Denton
    Centre for Rheumatology, Royal Free and University College Medical School, London, and Royal Free Hospital, UK
    Nat Clin Pract Rheumatol 2:134-44. 2006
    ..Overall, this is an exciting time for new therapies in SSc and advances are being made in synchrony with an improved understanding of the molecular and biochemical basis of the disease...
  7. ncbi Recombinant human anti-transforming growth factor beta1 antibody therapy in systemic sclerosis: a multicenter, randomized, placebo-controlled phase I/II trial of CAT-192
    Christopher P Denton
    Royal Free Hospital, London, UK
    Arthritis Rheum 56:323-33. 2007
    ..To evaluate CAT-192, a recombinant human antibody that neutralizes transforming growth factor beta1 (TGFbeta1), in the treatment of early-stage diffuse cutaneous systemic sclerosis (dcSSc)...
  8. ncbi Using a self-reported functional score to assess disease progression in systemic sclerosis
    K Serednicka
    Centre for Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Rheumatology (Oxford) 46:1107-10. 2007
    ..The aim is to determine if the FS is useful as an objective assessment tool for longitudinal evaluation of the functional impact of systemic sclerosis (SSc)...
  9. pmc Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases
    C P Denton
    Center for Rheumatology, Royal Free Hospital, Pond Street, Rheumatology Unit, Lower Ground Floor, London NW3 2QG, UK
    Ann Rheum Dis 67:1222-8. 2008
    ..This study investigated the long-term effects of bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist, in patients with pulmonary arterial hypertension (PAH) exclusively related to connective tissue diseases (CTD)...
  10. pmc Systemic sclerosis-associated pulmonary hypertension: why disease-specific composite endpoints are needed
    Christopher P Denton
    Centre for Rheumatology, Royal Free Campus, University College Medical School, London, NW3 2PF, UK
    Arthritis Res Ther 13:114. 2011
    ..Failure to take this into account may compromise progress in managing PAH in SSc...
  11. 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...
  12. doi Renal complications and scleroderma renal crisis
    C P Denton
    Centre for Rheumatology, Royal Free Hospital, Rheumatology Unit, London, UK
    Rheumatology (Oxford) 48:iii32-5. 2009
    ..Modification of endothelin over-activity, which is implicated in the pathogenesis of SRC, may offer a future therapeutic approach...
  13. doi Inducible lineage-specific deletion of TbetaRII in fibroblasts defines a pivotal regulatory role during adult skin wound healing
    Christopher P Denton
    Centre for Rheumatology, Royal Free and University College Medical School, London, UK
    J Invest Dermatol 129:194-204. 2009
    ..Together, our data confirm that expression of TbetaRII in differentiated dermal fibroblasts is essential for normal wound healing and demonstrate a critical role in the development and function of myofibroblasts...
  14. ncbi Targeted therapy comes of age in scleroderma
    Christopher P Denton
    Centre for Rheumatology, Royal Free Campus, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
    Trends Immunol 26:596-602. 2005
    ..This review will consider current clinical aspects of SSc and the emergence of targeted therapy that is linked to key pathogenic processes or that targets pivotal mediators...
  15. ncbi Endogenous endothelin-1 signaling contributes to type I collagen and CCN2 overexpression in fibrotic fibroblasts
    Xu Shi-Wen
    Centre for Rheumatology, Department of Medicine, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
    Matrix Biol 26:625-32. 2007
    ..Thus endogenous endothelin signaling contributes to the fibrotic phenotype of fibrotic fibroblasts, suggesting that antagonizing endothelin receptors may be of benefit in combating fibrotic disease...
  16. ncbi Endothelin is a downstream mediator of profibrotic responses to transforming growth factor beta in human lung fibroblasts
    Xu Shi-Wen
    Royal Free Hospital, University College London, London, UK
    Arthritis Rheum 56:4189-94. 2007
    ..The goal of this study was to assess whether ET-1 is a downstream mediator of the profibrotic effects of TGFbeta in lung fibroblasts...
  17. doi Pivotal role of connective tissue growth factor in lung fibrosis: MAPK-dependent transcriptional activation of type I collagen
    Markella Ponticos
    Royal Free and University College Medical School, London, UK
    Arthritis Rheum 60:2142-55. 2009
    ....
  18. pmc Constitutive ALK5-independent c-Jun N-terminal kinase activation contributes to endothelin-1 overexpression in pulmonary fibrosis: evidence of an autocrine endothelin loop operating through the endothelin A and B receptors
    Xu Shi-Wen
    Centre for Rheumatology, Department of Medicine, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, United Kingdom
    Mol Cell Biol 26:5518-27. 2006
    ....
  19. pmc Endothelin-1 promotes myofibroblast induction through the ETA receptor via a rac/phosphoinositide 3-kinase/Akt-dependent pathway and is essential for the enhanced contractile phenotype of fibrotic fibroblasts
    Xu Shi-Wen
    Centre for Rheumatology, Royal Free and University College Medical School, London NW3 2PF, United Kingdom
    Mol Biol Cell 15:2707-19. 2004
    ..Thus, blocking ET-1 or the PI3-kinase/Akt cascades might be beneficial in reducing scar formation in pulmonary fibrosis...
  20. doi Cross-talk between MCP-3 and TGFbeta promotes fibroblast collagen biosynthesis
    Voon H Ong
    Centre for Rheumatology and Connective Tissue Diseases, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
    Exp Cell Res 315:151-61. 2009
    ..Together, our results confirm cross-talk between MCP-3 and TGFbeta that may be critical in the development of fibrosis...
  21. pmc Rac inhibition reverses the phenotype of fibrotic fibroblasts
    Shi wen Xu
    Centre for Rheumatology, Department of Medicine, University College London Royal Free Campus, London, United Kingdom
    PLoS ONE 4:e7438. 2009
    ..The basis for this phenomenon is poorly understood, and is a necessary prerequisite for developing novel, rational anti-fibrotic strategies...
  22. doi Fibroblast-specific perturbation of transforming growth factor beta signaling provides insight into potential pathogenic mechanisms of scleroderma-associated lung fibrosis: exaggerated response to alveolar epithelial injury in a novel mouse model
    Rachel K Hoyles
    Center for Rheumatology, Royal Free and University College Medical School, and Interstitial Royal Brompton Hospital, London, UK
    Arthritis Rheum 58:1175-88. 2008
    ....
  23. doi Requirement of transforming growth factor beta-activated kinase 1 for transforming growth factor beta-induced alpha-smooth muscle actin expression and extracellular matrix contraction in fibroblasts
    Xu Shi-Wen
    University College London Royal Free Campus, London, UK
    Arthritis Rheum 60:234-41. 2009
    ..The present study was undertaken to assess whether TGFbeta-activated kinase 1 (TAK1) acts downstream of FAK/Src to mediate fibrogenic responses in fibroblasts...
  24. doi Epithelial cells promote fibroblast activation via IL-1alpha in systemic sclerosis
    Nima Aden
    Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital Campus, University College Medical School, University College London, London, UK
    J Invest Dermatol 130:2191-200. 2010
    ..These findings are important because biologic therapies could target epithelial-fibroblast interactions in the disease...
  25. doi An essential role for resident fibroblasts in experimental lung fibrosis is defined by lineage-specific deletion of high-affinity type II transforming growth factor β receptor
    Rachel K Hoyles
    Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, Royal Free Campus, London, United Kingdom
    Am J Respir Crit Care Med 183:249-61. 2011
    ..Fibrotic response to lung injury depends on development of a fibrogenic population of myofibroblasts. The importance of resident interstitial fibroblasts and role of transforming growth factor β (TGFβ) in this process is unclear...
  26. ncbi A polymorphism in the CTGF promoter region associated with systemic sclerosis
    Carmen Fonseca
    Centre for Rheumatology, Royal Free and University College Medical School, London, United Kingdom
    N Engl J Med 357:1210-20. 2007
    ..Systemic sclerosis (scleroderma) is a life-threatening autoimmune disease that is characterized by the presence of specific autoantibodies and fibrosis of the skin and major internal organs...
  27. ncbi Activation of key profibrotic mechanisms in transgenic fibroblasts expressing kinase-deficient type II Transforming growth factor-{beta} receptor (T{beta}RII{delta}k)
    Christopher P Denton
    Centre for Rheumatology, Royal Free and University College Medical School, Hampstead Campus, London NW3 2PF, United Kingdom
    J Biol Chem 280:16053-65. 2005
    ..Our study demonstrates that altered high affinity TGFbeta receptor function may lead to ligand-dependent activation of downstream signaling, and provides further evidence of a pivotal role for sustained TGFbeta overactivity in fibrosis...
  28. pmc Systemic vasculopathy with altered vasoreactivity in a transgenic mouse model of scleroderma
    Emma C Derrett-Smith
    Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, Royal Free Campus, Rowland Hill Street, London, NW3 2PF, UK
    Arthritis Res Ther 12:R69. 2010
    ....
  29. doi Borderline mean pulmonary artery pressure in patients with systemic sclerosis: transpulmonary gradient predicts risk of developing pulmonary hypertension
    Christopher J Valerio
    National Pulmonary Hypertension Unit, Royal Free Hospital, London, UK
    Arthritis Rheum 65:1074-84. 2013
    ....
  30. doi Clinical and pathological significance of interleukin 6 overexpression in systemic sclerosis
    Korsa Khan
    Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, Royal Free Campus, London, UK
    Ann Rheum Dis 71:1235-42. 2012
    ..To determine the potential clinical and pathological significance of altered expression of interleukin 6 (IL-6) in systemic sclerosis (SSc)...
  31. doi Heparan sulfate-dependent ERK activation contributes to the overexpression of fibrotic proteins and enhanced contraction by scleroderma fibroblasts
    Yunliang Chen
    University College London, London, UK
    Arthritis Rheum 58:577-85. 2008
    ....
  32. ncbi Transgenic analysis of scleroderma: understanding key pathogenic events in vivo
    Christopher P Denton
    Centre for Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Autoimmun Rev 3:285-93. 2004
    ..Better mouse models will improve understanding of this intractable rheumatic disease and can be expected to ultimately lead to improved treatments and outcome...
  33. ncbi Severe interstitial lung disease in connective tissue disease: rituximab as rescue therapy
    Gregory J Keir
    Interstitial Lung Disease Unit, Royal Brompton Hospital, Sydney Street, SW3 6NP, London, UK
    Eur Respir J 40:641-8. 2012
    ..03), in the 9-12 months following treatment with rituximab. In very severe CTD-ILD unresponsive to conventional immunosuppression, rituximab may represent an effective, potentially life-saving, therapeutic intervention...
  34. doi Animal models of scleroderma: lessons from transgenic and knockout mice
    Emma C Derrett-Smith
    Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, London, UK
    Curr Opin Rheumatol 21:630-5. 2009
    ....
  35. pmc Shared expression of phenotypic markers in systemic sclerosis indicates a convergence of pericytes and fibroblasts to a myofibroblast lineage in fibrosis
    Vineeth S Rajkumar
    Centre for Rheumatology and Connective Tissue Disease, Department of Medicine, Royal Free Campus, University College London, London, UK
    Arthritis Res Ther 7:R1113-23. 2005
    ....
  36. ncbi Single-nucleotide polymorphisms in CCL2 gene are not associated with susceptibility to systemic sclerosis
    Maria Teresa Carulli
    Centre for Rheumatology, Royal Free Hospital and University College Medical School, Hampstead, London, UK
    J Rheumatol 35:839-44. 2008
    ..We also performed subgroup analysis to test the hypothesis that CCL2 variants predispose to specific disease phenotypes...
  37. ncbi Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression
    Nicole S L Goh
    Royal Brompton Hospital and National Heart and Lung Institute, London, UK
    Arthritis Rheum 56:2005-12. 2007
    ..To evaluate the prognostic value of bronchoalveolar lavage (BAL) cellular profiles in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD)...
  38. doi Rosiglitazone alleviates the persistent fibrotic phenotype of lesional skin scleroderma fibroblasts
    Xu Shi-Wen
    Centre for Rheumatology, University College London Royal Free Campus, London, UK
    Rheumatology (Oxford) 49:259-63. 2010
    ..The aim of the present study was to examine whether PPAR-gamma expression was reduced in skin scleroderma fibroblasts and whether PPAR-gamma agonists could suppress the persistent fibrotic phenotype of skin scleroderma fibroblasts...
  39. ncbi Contribution of activin receptor-like kinase 5 (transforming growth factor beta receptor type I) signaling to the fibrotic phenotype of scleroderma fibroblasts
    Yunliang Chen
    Royal Free and University College Medical School, London, UK
    Arthritis Rheum 54:1309-16. 2006
    ....
  40. doi Mucin 5B promoter polymorphism is associated with idiopathic pulmonary fibrosis but not with development of lung fibrosis in systemic sclerosis or sarcoidosis
    Carmel J Stock
    Interstitial Lung Disease Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, London, UK
    Thorax 68:436-41. 2013
    ..We set out to verify whether this variant is also a risk factor for fibrotic lung disease in other settings and to confirm the published findings in a UK Caucasian IPF population...
  41. ncbi Monocyte chemoattractant protein 3 as a mediator of fibrosis: Overexpression in systemic sclerosis and the type 1 tight-skin mouse
    Voon H Ong
    Royal Free Hospital and University College Medical School, London, UK
    Arthritis Rheum 48:1979-91. 2003
    ....
  42. doi 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...
  43. doi Disease-modifying treatment in systemic sclerosis: current status
    Niamh P Quillinan
    Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK
    Curr Opin Rheumatol 21:636-41. 2009
    ..To review evidence and best practice for current disease-modifying therapies for the treatment of systemic sclerosis...
  44. doi Endothelial injury in a transforming growth factor β-dependent mouse model of scleroderma induces pulmonary arterial hypertension
    Emma C Derrett-Smith
    University College London Medical School, Royal Free Campus, London, UK
    Arthritis Rheum 65:2928-39. 2013
    ....
  45. doi Revisiting ANCA-associated vasculitis in systemic sclerosis: clinical, serological and immunogenetic factors
    Emma C Derrett-Smith
    Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK
    Rheumatology (Oxford) 52:1824-31. 2013
    ..To define the clinical, serological, histological and immunogenetic features of patients with scleroderma and ANCA-associated vasculitis (AAV)...
  46. doi Gut fibrosis with altered colonic contractility in a mouse model of scleroderma
    Nora M Thoua
    Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, Royal Free Campus, Rowland Hill Street, London, NW3 2PF, UK
    Rheumatology (Oxford) 51:1989-98. 2012
    ..We investigated if similar changes are observed in the gut tissue in this mouse model...
  47. pmc Gene expression profiling reveals novel TGFbeta targets in adult lung fibroblasts
    Elisabetta A Renzoni
    Interstitial Lung Disease Unit, Royal Brompton Hospital, Imperial College of Science, Technology and Medicine, Emmanuel Kaye Building, 1B Manresa Road, SW3 6LR, London, UK
    Respir Res 5:24. 2004
    ..Even though the effects of TGFbeta on the gene expression of several proteins have been investigated in several lung fibroblast cell lines, the global pattern of response to this cytokine in adult lung fibroblasts is still unknown...
  48. ncbi Validation of a protocol for the assessment of skin temperature and blood flow in childhood localised scleroderma
    Kevin J Howell
    Department of Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Skin Res Technol 15:346-56. 2009
    ..We developed and validated a protocol using these techniques for the assessment of unilateral LS activity in children...
  49. doi Focal adhesion kinase and reactive oxygen species contribute to the persistent fibrotic phenotype of lesional scleroderma fibroblasts
    Xu Shi-Wen
    Centre for Rheumatology, University College London Royal Free Campus, London, UK
    Rheumatology (Oxford) 51:2146-54. 2012
    ..We aim to identify the precise contribution of adhesive signalling, which requires integrin-mediated activation of focal adhesion kinase (FAK)/src, to fibrogenic gene expression in normal and fibrotic SSc fibroblasts...
  50. doi Modulation of collagen type I, fibronectin and dermal fibroblast function and activity, in systemic sclerosis by the antioxidant epigallocatechin-3-gallate
    Audrey Dooley
    Centre for Rheumatology and Connective Tissue Disease, University College London Medical School, Royal Free Campus, London, UK
    Rheumatology (Oxford) 49:2024-36. 2010
    ..This study investigates the anti-fibrotic potential of the antioxidant epigallocatechin-3-gallate (EGCG) on activated dermal fibroblasts from SSc patients...
  51. ncbi Endothelin axis polymorphisms in patients with scleroderma
    Carmen Fonseca
    Royal Free and University College Medical School, Center for Rheumatology and Connective Tissue Diseases, Department of Medicine, University College London Hampstead Campus, Hampstead, London NW3 2PF, UK
    Arthritis Rheum 54:3034-42. 2006
    ..To evaluate the distribution of polymorphisms in the endothelin 1 (EDN1), endothelin receptor A (EDNRA) and endothelin receptor B (EDNRB) genes in systemic sclerosis (SSc; scleroderma) and SSc subsets...
  52. pmc Thrombospondin 1 is a key mediator of transforming growth factor β-mediated cell contractility in systemic sclerosis via a mitogen-activated protein kinase kinase (MEK)/extracellular signal-regulated kinase (ERK)-dependent mechanism
    Yunliang Chen
    School of Life Sciences, University of Westminster, London, UK
    Fibrogenesis Tissue Repair 4:9. 2011
    ..In this report we investigate whether activation of latent TGFβ by TSP1 plays a key role in matrix contraction by normal and scleroderma fibroblasts...
  53. ncbi Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: application of a latent linear trajectory model
    Lynne Shand
    Royal Free Hospital, London, UK
    Arthritis Rheum 56:2422-31. 2007
    ..To explore the relationship between changes in the severity of skin disease and morbidity and mortality in patients with diffuse cutaneous systemic sclerosis (dcSSc)...
  54. ncbi Prediction of pulmonary complications and long-term survival in systemic sclerosis
    Svetlana I Nihtyanova
    University College London Medical School, Royal Free Hospital, London, UK
    Arthritis Rheumatol 66:1625-35. 2014
    ....
  55. ncbi A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma
    Rachel K Hoyles
    Royal Brompton Hospital, Imperial College, London, UK
    Arthritis Rheum 54:3962-70. 2006
    ..This RCT was undertaken to investigate the effects of intravenous (IV) cyclophosphamide (CYC) followed by azathioprine (AZA) treatment in pulmonary fibrosis in SSc...
  56. doi Improving the detection of pulmonary hypertension in systemic sclerosis using pulmonary function tests
    Benjamin E Schreiber
    Royal Free Hampstead NHS Trust, London, UK
    Arthritis Rheum 63:3531-9. 2011
    ..To construct a readily applicable formula for selecting patients with systemic sclerosis (SSc) for right-sided heart catheterization (RHC) based on the results of their pulmonary function tests (PFTs)...
  57. ncbi Scleroderma--clinical and pathological advances
    Christopher P Denton
    Centre for Rheumatology, Royal Free Hospital, Pond Street, London, NW3 2QG, UK
    Best Pract Res Clin Rheumatol 18:271-90. 2004
    ..It is likely that clinical trials performed over the next few years will transform the management of systemic sclerosis and help to dispel its reputation as one of the least treatable of the autoimmune rheumatic diseases...
  58. doi Clinical experience with bosentan and sitaxentan in connective tissue disease-associated pulmonary arterial hypertension
    Christopher J Valerio
    The National Pulmonary Hypertension Unit, Royal Free Hospital, London, UK
    Rheumatology (Oxford) 49:2147-53. 2010
    ....
  59. doi Innovative therapies for systemic sclerosis
    Voon H Ong
    Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, Royal Free Hospital, London, UK
    Curr Opin Rheumatol 22:264-72. 2010
    ..The purpose of this study is to review the evidence and recent developments leading to novel therapeutics in scleroderma...
  60. doi Impact of hallmark autoantibody reactivity on early diagnosis in scleroderma
    Pia Moinzadeh
    Centre for Rheumatology, Royal Free Hospital and UCL Medical School, London, UK
    Clin Rev Allergy Immunol 43:249-55. 2012
    ..Duration of antecedent Raynaud's phenomenon differs between disease subsets and scleroderma-specific ANA patterns...
  61. ncbi Fibroblast-specific expression of a kinase-deficient type II transforming growth factor beta (TGFbeta) receptor leads to paradoxical activation of TGFbeta signaling pathways with fibrosis in transgenic mice
    Christopher P Denton
    Centre for Rheumatology, University College London, Royal Free Campus, Rowland Hill Street, United Kingdom
    J Biol Chem 278:25109-19. 2003
    ....
  62. ncbi Lack of agreement between rheumatologists in defining digital ulceration in systemic sclerosis
    Ariane L Herrick
    University of Manchester, Manchester, UK, and Salford Royal National Health Service Foundation Trust, Salford, UK
    Arthritis Rheum 60:878-82. 2009
    ..To test the intra- and interobserver variability, among clinicians with an interest in systemic sclerosis (SSc), in defining digital ulcers...
  63. doi Current approaches to the management of early active diffuse scleroderma skin disease
    Svetlana I Nihtyanova
    Centre for Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Rheum Dis Clin North Am 34:161-79; viii. 2008
    ..This article reviews the evidence for efficacy of current treatment approaches and future developments for managing skin disease in early diffuse cutaneous SSc...
  64. ncbi Normal circulating serum amyloid P component concentration in systemic sclerosis
    Glenys A Tennent
    Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, University College London, London, UK
    Arthritis Rheum 56:2013-7. 2007
    ..Because neither sustained SAP depletion in humans nor SAP deficiency in mice is associated with fibrosis, we sought to establish rigorously the serum SAP concentration in well-characterized patients with SSc...
  65. ncbi Management of systemic sclerosis
    Voon H Ong
    Centre for Rheumatology, Royal Free Hospital, London
    Clin Med 5:214-9. 2005
  66. doi A longitudinal study of anti-RNA polymerase III antibody levels in systemic sclerosis
    Svetlana I Nihtyanova
    Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Rheumatology (Oxford) 48:1218-21. 2009
    ..We analysed serial ARA levels and explored the relationship with clinical features and disease outcome...
  67. doi Clinical and serological hallmarks of systemic sclerosis overlap syndromes
    Angela Pakozdi
    Royal Free and University College Medical School, Centre for Rheumatology, London NW3 2PF, UK
    J Rheumatol 38:2406-9. 2011
    ..To determine the prevalence of systemic sclerosis (SSc) overlap syndrome and autoantibody profile in a large single-center cohort...
  68. doi Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study
    S I Nihtyanova
    Centre for Rheumatology, Royal Free Hospital, London NW3 2QG, UK
    QJM 103:109-15. 2010
    ..Systemic sclerosis (SSc) has high mortality and morbidity. Current management focuses on early detection and treatment of organ-based manifestations...
  69. doi Diagnosis, management and prevention of scleroderma renal disease
    Henry Penn
    Centre for Rheumatology, Royal Free Hospital, London, UK
    Curr Opin Rheumatol 20:692-6. 2008
    ..This is a medical emergency that requires careful management. Recent cohort studies have highlighted key aspects of management and outcome and these are reviewed...
  70. pmc Elevated CCN2 expression in scleroderma: a putative role for the TGFβ accessory receptors TGFβRIII and endoglin
    Alan M Holmes
    Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, Royal Free Campus, London, UK, NW3 2PF
    J Cell Commun Signal 5:173-7. 2011
    ..TGFβRIII alone or in combination with endoglin was sufficient to enhance basal CCN2 promoter activity. Thus TGFβ accessory receptors may play a significant role in the altered expression of fibrogenic genes in SDF...
  71. doi Autoantibodies as predictive tools in systemic sclerosis
    Svetlana I Nihtyanova
    Royal Free and University College Medical School, London, UK
    Nat Rev Rheumatol 6:112-6. 2010
    ..Strong links exist between autoantibody specificities and disease presentation and outcome, which make autoantibodies essential assessment tools in patients with SSc...
  72. pmc Pericytes display increased CCN2 expression upon culturing
    Xu Shiwen
    Centre for Rheumatology, University College London Royal Free Campus, Rowland Hill Street, London, UK, NW3 2PF
    J Cell Commun Signal 3:61-4. 2009
    ..These results indicate that, in principle, pericytes have the capacity to become fibroblast-like and that pericytes may contribute to the population of fibroblasts in a healed wound...
  73. ncbi Recent advances in the diagnosis and treatment of interstitial lung disease in systemic sclerosis (scleroderma): a review
    Aaron Tan
    Division of Surgery and Interventional Science, University College London, UK
    Clin Exp Rheumatol 29:S66-74. 2011
    ..Here, we review the current knowledge pertaining to the treatment of ILD in systemic sclerosis, and also address the various challenges involved as well as implications for the future...
  74. pmc Systemic sclerosis associated pulmonary hypertension: improved survival in the current era
    M H Williams
    Department of Cardiology, Royal Free Hospital, London, UK
    Heart 92:926-32. 2006
    ..To measure survival, haemodynamic function and functional class in patients with systemic sclerosis associated pulmonary arterial hypertension (SSc-PAH) in two treatment eras...
  75. doi Proteomic analysis of scleroderma lesional skin reveals activated wound healing phenotype of epidermal cell layer
    N Aden
    Centre for Rheumatology, Royal Free Hospital and University College School of Medicine, Royal Free Campus, Rowland Hill Street, London NW3 2QG, UK
    Rheumatology (Oxford) 47:1754-60. 2008
    ..To identify using proteomic analysis, proteins of altered abundance in the skin of patients with SSc...
  76. ncbi Transforming growth factor-beta and connective tissue growth factor: key cytokines in scleroderma pathogenesis
    C P Denton
    Centre for Rheumatology, Royal Free and University College Medical School, London, United Kingdom
    Curr Opin Rheumatol 13:505-11. 2001
    ..These agents will further clarify the role of this ligand in initiating or sustaining fibrosis and offer the exciting possibility of targeted therapy for this disease...
  77. ncbi Scleroderma renal crisis: patient characteristics and long-term outcomes
    H Penn
    Department of Rheumatology, Royal Free Hospital, London, UK
    QJM 100:485-94. 2007
    ..Scleroderma renal crisis (SRC) is an important complication of systemic sclerosis, causing acute renal failure, and usually hypertension...
  78. pmc Demographic, clinical and antibody characteristics of patients with digital ulcers in systemic sclerosis: data from the DUO Registry
    Christopher P Denton
    Centre for Rheumatology and Connective Tissue Diseases, Department of Inflammation, Division of Medicine, UCL Medical School, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK
    Ann Rheum Dis 71:718-21. 2012
    ..The Digital Ulcers Outcome (DUO) Registry was designed to describe the clinical and antibody characteristics, disease course and outcomes of patients with digital ulcers associated with systemic sclerosis (SSc)...
  79. doi Internal anal sphincter atrophy in patients with systemic sclerosis
    Nora M Thoua
    GI Physiology Unit, UniversityCollege Hospital, London, UK
    Rheumatology (Oxford) 50:1596-602. 2011
    ..Thinning of the internal anal sphincter (IAS) has been demonstrated in SSc with faecal incontinence. We aimed to investigate anal sphincter structure in patients with SSc...
  80. ncbi Insights into the molecular mechanism of chronic fibrosis: the role of connective tissue growth factor in scleroderma
    Andrew Leask
    Center for Rheumatology, Royal Free and University College Medical School, University College London, Royal Free Campus, London, UK
    J Invest Dermatol 122:1-6. 2004
    ..This review discusses recent information regarding insights into connective tissue growth factor biology and, using scleroderma as a model system, the part connective tissue growth factor might play in fibrotic disease...
  81. ncbi Pulmonary hypertension in systemic sclerosis
    Christopher P Denton
    Center for Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Rheum Dis Clin North Am 29:335-49, vii. 2003
    ..The next major challenge is to improve diagnosis and treatment of early stage or presymptomatic pulmonary hypertension with the goal of preventing this important cause of premature SSc-related mortality...
  82. 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
    ....
  83. ncbi Anti-fibrillarin antibodies in systemic sclerosis
    V J Tormey
    Department of Immunology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Rheumatology (Oxford) 40:1157-62. 2001
    ..In contrast to previous reports, AFA was not restricted to dcSSc patients in Caucasians...
  84. ncbi Can CCL2 serum levels be used in risk stratification or to monitor treatment response in systemic sclerosis?
    M T Carulli
    Centre for Rheumatology, Royal Free and University College Medical School, Rowland Hill Street, Hampstead, London, NW3 2PF, UK
    Ann Rheum Dis 67:105-9. 2008
    ..To explore the potential value of serum CCL2 measurement in disease assessment, we have compared CCL2 levels with clinical phenotype and investigated effect of therapy on circulating CCL2...
  85. ncbi Circulating levels of active transforming growth factor beta1 are reduced in diffuse cutaneous systemic sclerosis and correlate inversely with the modified Rodnan skin score
    M Dziadzio
    Centre for Rheumatology, Royal Free and University College Medical School, University College London, London NW3 2PF, UK
    Rheumatology (Oxford) 44:1518-24. 2005
    ..To determine the relationship between clinical features and circulating levels of active transforming growth factor (TGF) beta1 in the major subsets of systemic sclerosis (SSc)...
  86. ncbi Endothelin-1 induces expression of matrix-associated genes in lung fibroblasts through MEK/ERK
    Shi wen Xu
    Centre for Rheumatology, Department of Medicine, Royal Free and University College London, United Kingdom
    J Biol Chem 279:23098-103. 2004
    ..Our results suggest that ET-1 induces a program of matrix synthesis in lung fibroblasts and that ET-1 may play a key role in connective tissue deposition during wound repair and in pulmonary fibrosis...
  87. ncbi Serological assessment of type I collagen burden in scleroderma spectrum disorders: a systematic review
    M Dziadzio
    Centre for Rheumatology and Connective Tissue Diseases, Royal Free and University College Medical School, University College, London, UK
    Clin Exp Rheumatol 22:356-67. 2004
    ....
  88. doi Multiplex immune serum biomarker profiling in sarcoidosis and systemic sclerosis
    P Beirne
    Dept of Respiratory Medicine, Royal Brompton and Harefield NHS Trust, London, UK
    Eur Respir J 34:1376-82. 2009
    ..These findings, if they are corroborated by independent studies in other centres, have potential for clinical application and may generate novel insights into the modulation of immune profiles during disease evolution...
  89. ncbi The role of radiology in the management of systemic sclerosis
    G Madani
    Radiology Department, St Mary s Hospital, Royal Free Campus, University College London, London, UK
    Clin Radiol 63:959-67. 2008
    ..Neurological involvement is increasingly recognized and the major role of radiology is the exclusion of coexistent pathology. We present a thorough review of the role of radiology in the management of systemic sclerosis...
  90. ncbi Angiographically proven coronary artery disease in scleroderma
    M R Akram
    Department of Cardiology, Royal Free Hospital, London, NW3 2QG, UK
    Rheumatology (Oxford) 45:1395-8. 2006
    ..It has been suggested that macrovascular disease is more common in patients with scleroderma (SSc). We investigated the prevalence of coronary artery disease (CAD) in SSc using coronary angiography...
  91. pmc The myth of pulmonary Raynaud's phenomenon: the contribution of pulmonary arterial vasospasm in patients with systemic sclerosis related pulmonary arterial hypertension
    D Mukerjee
    Centre for Rheumatology, Royal Free Hospital, London, UK
    Ann Rheum Dis 63:1627-31. 2004
    ..To investigate the contribution of cold induced pulmonary vasospasm by peripheral and central cold stimulus in exacerbating pulmonary arterial hypertension (PAH) in patients with systemic sclerosis undergoing cardiac catheterisation...
  92. pmc Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis
    C Rigamonti
    Liver Transplantation and Hepatobiliary Medicine, Royal Free Hospital, Pond St, Hampstead, London NW3 2QG, UK
    Gut 55:388-94. 2006
    ..To evaluate the prognosis of primary biliary cirrhosis (PBC) together with systemic sclerosis (SSc), as this is unknown...
  93. ncbi Fibroblast matrix gene expression and connective tissue remodeling: role of endothelin-1
    X Shi-Wen
    Center for Rheumatology and Department of Molecular Pathology, Royal Free and University College Medical School, London, UK
    J Invest Dermatol 116:417-25. 2001
    ..These data further implicate dysregulated endothelin-receptor pathways in fibroblasts in the pathogenesis of connective tissue fibrosis...
  94. 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...
  95. ncbi A cross-sectional comparison of three self-reported functional indices in scleroderma
    A E Smyth
    Centre for Rheumatology, Royal Free Hospital, London, UK
    Rheumatology (Oxford) 42:732-8. 2003
    ..In addition, the use of clinical and laboratory measures as predictors of disease severity have been examined...
  96. ncbi Laser Doppler flowmetry for assessing localized scleroderma in children
    Lisa Weibel
    Great Ormond Street Hospital for Children, London, UK
    Arthritis Rheum 56:3489-95. 2007
    ..The aim of this study was to evaluate the role of laser Doppler flowmetry (LDF) in comparison with infrared thermography in the detection of scleroderma disease activity...
  97. ncbi Does long term therapy with lansoprazole slow progression of oesophageal involvement in systemic sclerosis?
    A Pakozdi
    Royal Free and University College Medical School, Centre for Rheumatology and Connective Tissue Diseases, Department of Medicine, University College London Hampstead Campus, Hampstead, London, UK
    Clin Exp Rheumatol 27:5-8. 2009
    ..Our objective was to assess the long term effect of lansoprazole therapy on gastroesophageal dysmotility in systemic sclerosis (SSc)...
  98. ncbi Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival
    A J MacGregor
    Department of Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Rheumatology (Oxford) 40:453-9. 2001
    ..These results demonstrate the value of echocardiographic screening for PHT in all patients with SSC...
  99. pmc Low plasma protein nitrotyrosine levels distinguish primary Raynaud's phenomenon from scleroderma
    E J Kingdon
    Royal Free and University College Medical School, University College London UCL, Rowland Hill Street, London NW3 2PF, UK
    Ann Rheum Dis 65:952-4. 2006
    ..To investigate the hypothesis that increased formation of reactive nitrogen species may contribute to the vascular pathology that develops in patients with connective tissue disease such as scleroderma...
  100. ncbi Clinical burden of digital vasculopathy in limited and diffuse cutaneous systemic sclerosis
    S I Nihtyanova
    Centre for Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    Ann Rheum Dis 67:120-3. 2008
    ..To determine the clinical burden of severe digital vasculopathy (SDV), we have reviewed hospital-based treatment for this important complication of SSc in a large single centre cohort...
  101. doi Systemic sclerosis as a model of chronic rejection in facial composite tissue transplantation
    B Sivakumar
    Royal Free Hospital School of Medicine, Pond Street, London NW3 2QG, UK
    J Plast Reconstr Aesthet Surg 63:1669-76. 2010
    ..Through the analysis of facial changes in scleroderma we demonstrate how chronic facial allograft rejection may present and progress...