diffuse scleroderma

Summary

Summary: A rapid onset form of SYSTEMIC SCLERODERMA with progressive widespread SKIN thickening over the arms, the legs and the trunk, resulting in stiffness and disability.

Top Publications

  1. ncbi Kidney disease other than renal crisis in patients with diffuse scleroderma
    Virginia D Steen
    Georgetown University, Washington, DC 20007, USA
    J Rheumatol 32:649-55. 2005
  2. 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
  3. 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
  4. pmc A TGFbeta-responsive gene signature is associated with a subset of diffuse scleroderma with increased disease severity
    Jennifer L Sargent
    Department of Genetics, Dartmouth Medical School, Hanover, New Hampshire 03755, USA
    J Invest Dermatol 130:694-705. 2010
  5. ncbi A case of diffuse scleroderma successfully treated with high-dose intravenous immune globulin infusion
    Y Asano
    Rheumatology (Oxford) 44:824-6. 2005
  6. ncbi Clinical subsets, skin thickness progression rate, and serum antibody levels in systemic sclerosis patients with anti-topoisomerase I antibody
    Achini Perera
    University of Pittsburgh School of Medicine, 3500 Terrace Street, Pittsburgh, PA 15261, USA
    Arthritis Rheum 56:2740-6. 2007
  7. ncbi Cardiac magnetic resonance imaging detects subclinical right ventricular impairment in systemic sclerosis
    Gian Paolo Bezante
    Division of Cardiology, Department of Internal Medicine, University of Genoa, Genoa, Italy
    J Rheumatol 34:2431-7. 2007
  8. ncbi Magnetic resonance imaging for the detection of myocardial fibrosis in scleroderma
    Sotiris C Plastiras
    N Engl J Med 354:2194-6. 2006
  9. pmc Systemic and cell type-specific gene expression patterns in scleroderma skin
    Michael L Whitfield
    Department of Dermatology, University of California San Francisco, San Francisco, CA 94143, USA
    Proc Natl Acad Sci U S A 100:12319-24. 2003
  10. ncbi Use of antibodies recognizing cyclic citrullinated peptide in the differential diagnosis of joint involvement in systemic sclerosis
    Francesca Ingegnoli
    Department of Rheumatology, Istituto Gaetano Pini, University of Milan, Piazza Cardinal Ferrari, 1 20122 Milan, Italy
    Clin Rheumatol 26:510-4. 2007

Detail Information

Publications223 found, 100 shown here

  1. ncbi Kidney disease other than renal crisis in patients with diffuse scleroderma
    Virginia D Steen
    Georgetown University, Washington, DC 20007, USA
    J Rheumatol 32:649-55. 2005
    To determine the frequency and severity of kidney abnormalities in patients with diffuse scleroderma.
  2. 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)...
  3. 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...
  4. pmc A TGFbeta-responsive gene signature is associated with a subset of diffuse scleroderma with increased disease severity
    Jennifer L Sargent
    Department of Genetics, Dartmouth Medical School, Hanover, New Hampshire 03755, USA
    J Invest Dermatol 130:694-705. 2010
    ..The TGFbeta-responsive signature is found in only a subset of dSSc patients who could be targeted by specific therapies...
  5. ncbi A case of diffuse scleroderma successfully treated with high-dose intravenous immune globulin infusion
    Y Asano
    Rheumatology (Oxford) 44:824-6. 2005
  6. ncbi Clinical subsets, skin thickness progression rate, and serum antibody levels in systemic sclerosis patients with anti-topoisomerase I antibody
    Achini Perera
    University of Pittsburgh School of Medicine, 3500 Terrace Street, Pittsburgh, PA 15261, USA
    Arthritis Rheum 56:2740-6. 2007
    ....
  7. ncbi Cardiac magnetic resonance imaging detects subclinical right ventricular impairment in systemic sclerosis
    Gian Paolo Bezante
    Division of Cardiology, Department of Internal Medicine, University of Genoa, Genoa, Italy
    J Rheumatol 34:2431-7. 2007
    ..To assess myocardial involvement in patients with systemic sclerosis (SSc) with no signs or symptoms of cardiac impairment (New York Heart Association functional class I)...
  8. ncbi Magnetic resonance imaging for the detection of myocardial fibrosis in scleroderma
    Sotiris C Plastiras
    N Engl J Med 354:2194-6. 2006
  9. pmc Systemic and cell type-specific gene expression patterns in scleroderma skin
    Michael L Whitfield
    Department of Dermatology, University of California San Francisco, San Francisco, CA 94143, USA
    Proc Natl Acad Sci U S A 100:12319-24. 2003
    ..gene expression patterns in skin biopsies from individuals with a diagnosis of systemic sclerosis with diffuse scleroderma. We found consistent differences in the patterns of gene expression between skin biopsies from individuals ..
  10. ncbi Use of antibodies recognizing cyclic citrullinated peptide in the differential diagnosis of joint involvement in systemic sclerosis
    Francesca Ingegnoli
    Department of Rheumatology, Istituto Gaetano Pini, University of Milan, Piazza Cardinal Ferrari, 1 20122 Milan, Italy
    Clin Rheumatol 26:510-4. 2007
    ..To determine the prevalence of anti-cyclic citrullinated peptide (CCP) antibodies in systemic sclerosis (SSc) and to assess any association between the presence of anti-CCP, radiographic features, and clinical manifestations...
  11. ncbi Parvoviral infection of endothelial cells and stromal fibroblasts: a possible pathogenetic role in scleroderma
    Cynthia M Magro
    Department of Pathology, The Ohio State University, Columbus, OH, USA
    J Cutan Pathol 31:43-50. 2004
    ..Having previously demonstrated parvovirus B19 (B19) DNA in bone marrow and skin biopsies of SSc patients in the absence of B19 viremia, we sought to further elucidate a role for B19 in the pathogenesis of SSc...
  12. ncbi The use of the hand anatomic index to assess deformity and impaired function in systemic sclerosis
    A J Roberts-Thomson
    University of South Australia, Daw Park, South Australia, Australia
    Rheumatol Int 26:439-44. 2006
    ..measure which clearly distinguished patients with increasing hand deformity and separated patients with diffuse scleroderma (n=12) from limited scleroderma (n=18), P=0.005...
  13. ncbi Different clinical features in patients with limited and diffuse cutaneous systemic sclerosis
    Predrag Ostojic
    Clinical Rheumatology III, Institute of Rheumatology, School of Medicine, University of Belgrade, Belgrade, Yugoslavia
    Clin Rheumatol 25:453-7. 2006
    ..Fingertip osteolysis, telangiectasia, and arthritis are equally frequent in both forms of the disease...
  14. ncbi Effectiveness of a treatment based on melatonin in five patients with systemic sclerosis
    Mauro Todisco
    ASL 11 Local Health Department of National Health Service, Fermo, Italy
    Am J Ther 13:84-7. 2006
    ..6 months; range, 7-44 months). Toxicity was lacking, with the only side effect being drowsiness. Our experience suggests that the combination of melatonin-ACTH-vitamin E may be safe and effective in patients with SSc...
  15. ncbi Serum levels of 8-isoprostane, a marker of oxidative stress, are elevated in patients with systemic sclerosis
    F Ogawa
    Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, 1 7 1 Sakamoto, Nagasaki 852 8501, Japan
    Rheumatology (Oxford) 45:815-8. 2006
    ..To determine serum levels and clinical correlation of 8-isoprostane, which is produced in vivo through free radical-catalysed peroxidation of arachidonic acid and reflects oxidative stress, in patients with systemic sclerosis (SSc)...
  16. ncbi Morbidity and mortality of patients diagnosed with systemic sclerosis after the age of 75: a nested case-control study
    Chris T Derk
    Department of Medicine, Division of Rheumatology, Thomas Jefferson University, Philadelphia, PA, 19017, USA
    Clin Rheumatol 25:831-4. 2006
    ..A higher clinical suspicion will lead to an earlier diagnosis and a potential decrease in morbidity and mortality...
  17. ncbi Functional hyperprolactinemia and hypophyseal microadenoma in systemic sclerosis
    Olga Vera-Lastra
    Department of Internal Medicine, Division of Research, Clinical and Epidemiology Research, Office of Education Research, Hospital de Espcialidades, Centro Medico La Raza, IMSS, Mexico City, Mexico
    J Rheumatol 33:1108-12. 2006
    ..We investigated the prevalence of prolactin (PRL)-secreting pituitary adenoma and evaluated production of PRL by dynamic testing with MTC in SSc...
  18. ncbi Factors related to oral hygiene in persons with scleroderma
    J L Poole
    Occupational Therapy Graduate Program, Department of Pediatrics, University of New Mexico, Albuquerque, NM 87131 0001, USA
    Int J Dent Hyg 3:13-7. 2005
    ..The purpose of this study was to assess potential risk factors for the development of poor oral hygiene and increased dental caries in individuals with scleroderma...
  19. ncbi Proton magnetic resonance spectroscopy reveals central neuroaxonal impairment in systemic sclerosis
    Luca Bertinotti
    Department of Internal Medicine, Section of Pheumatology, AOUC, University of Florence, Italy
    J Rheumatol 33:546-51. 2006
    ..Involvement of the central nervous system (CNS) in systemic sclerosis (SSc) is rare. Proton magnetic resonance spectroscopy (1H-MRS) assesses in vivo cerebral metabolites. We investigated the biochemical modifications of the CNS in SSc...
  20. ncbi Carotid and femoral arterial wall mechanics in scleroderma
    K S Cheng
    University Department of Surgery, University College London, London, UK
    Rheumatology (Oxford) 42:1299-305. 2003
    ....
  21. ncbi Soluble P selectin in systemic sclerosis: relationship with von Willebrand factor, autoantibodies and diffuse or localised/limited disease
    A D Blann
    Haemostasis, Thrombosis and Vascular Biology Unit, University Department of Medicine, City Hospital, Dudley Road, Birmingham B18 7QH, UK
    Thromb Res 109:203-6. 2003
  22. ncbi Anti-endothelial cell antibodies from patients with limited cutaneous systemic sclerosis bind to centromeric protein B (CENP-B)
    Amélie Servettaz
    Universite Paris Descartes, Faculte de Medecine, UPRES EA 1833, Laboratoire d Immunologie, Paris, France
    Clin Immunol 120:212-9. 2006
    ..The main target of anti-endothelial cell antibodies in patients with limited cutaneous SSc is the nuclear and ubiquitous protein CENP-B...
  23. ncbi Distinct expression of adhesion molecules on skin fibroblasts from patients with diffuse and limited systemic sclerosis. A pilot study
    Florenzo Iannone
    Rheumatology Unit DIMIMP, University of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy
    J Rheumatol 32:1893-8. 2005
    ..We investigated the expression of integrins and CD44 on skin fibroblasts from patients with limited and diffuse SSc...
  24. 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)...
  25. ncbi Serum pulmonary and activation-regulated chemokine/CCL18 levels in patients with systemic sclerosis: a sensitive indicator of active pulmonary fibrosis
    Masanari Kodera
    Kanazawa University Graduate School of Medical Science, Kanazawa, Japan
    Arthritis Rheum 52:2889-96. 2005
    ....
  26. ncbi Damage of cutaneous peripheral nervous system evolves differently according to the disease phase and subset of systemic sclerosis
    L Ibba Manneschi
    Department of Anatomy, Histology and Forensic Medicine, University of Florence, Viale Morgagni, 85, 50134, Florence, Italy
    Rheumatology (Oxford) 44:607-13. 2005
    ..The aim was to study ultrastructural modifications of skin PNS fibres in SSc according to subsets [limited SSc (lSSc) and diffuse SSc (dSSc)] and phases (early and advanced) of the disease...
  27. ncbi Differential expression of tissue kallikrein in the skin of systemic sclerosis
    A F Milia
    Department of Medicine, Division of Rheumatology, University of Florence, Florence, Italy
    Histol Histopathol 20:415-22. 2005
    ..The decreased expression of t-kallikrein may be involved in the impairment of the sweating process, vessel functionality and angiogenesis...
  28. ncbi HLA allelic variants encoding DR11 in diffuse and limited systemic sclerosis in Caucasian women
    L S Loubière
    Immunogenetics Program, Fred Hutchinson Cancer Research Center, 1100 Fairview Ave N, Seattle, WA 98109, USA
    Rheumatology (Oxford) 44:318-22. 2005
    ..We investigated HLA class II alleles in women with systemic sclerosis (SSc), a rare disease that preferentially affects women...
  29. ncbi Single-nucleotide polymorphisms in the SPARC gene are not associated with susceptibility to scleroderma
    A L Lagan
    Clinical Genomics Group, National Heart and Lung Institute and Royal Brompton Hospital, Imperial College, 1B Manresa Road, London SW3 6LR, UK
    Rheumatology (Oxford) 44:197-201. 2005
    ..We set out to reproduce and to positionally clone these disease associations in a set of UK Caucasian scleroderma patients and ethnically matched controls...
  30. ncbi Aortic stiffness in systemic sclerosis is increased independently of the extent of skin involvement
    I Moyssakis
    Laikon Hospital, Department of Cardiology, 17 Agiou Thomas Street, GR 15727 Goudi, Athens, Greece
    Rheumatology (Oxford) 44:251-4. 2005
    ..To study the stiffness of large arteries in relation to the extent of skin and lung fibrosis, aortic distensibility was examined in patients with diffuse and limited systemic sclerosis (SSc)...
  31. ncbi Clonal T cells in the blood of patients with systemic sclerosis
    Isabelle Marie
    Department of Internal Medicine and INSERM Unit 644, Centre Hospitalier Universitaire de Rouen Boisguillaume, Rouen, France
    Arch Dermatol 141:88-9. 2005
    ..It has been suggested that clonal T cells may play a critical role in the pathogenesis of systemic sclerosis...
  32. ncbi Classification of systemic sclerosis. Visions and reality
    F A Wollheim
    Department of Rheumatology, Lund University Hospital, S 21774 Lund, Sweden
    Rheumatology (Oxford) 44:1212-6. 2005
    ..There is no convincing evidence of any advantage for distinguishing the limited, intermediate and diffuse forms of SSc rather than only the limited and diffuse forms...
  33. ncbi Prognostic factors of low bone mineral density in systemic sclerosis
    P D Sampaio-Barros
    Department of Internal Medicine, State University of Campinas, Campinas, Brazil
    Clin Exp Rheumatol 23:180-4. 2005
    ..To analyse the results of bone densitometry in patients with systemic sclerosis (SSc), evaluating the prognostic factors of low bone mineral density (BMD) in fertile and postmenopausal patients, and comparing to a control healthy group...
  34. ncbi Prevalence of antiphospholipid antibodies in systemic sclerosis and association with primitive pulmonary arterial hypertension and endothelial injury
    N Assous
    Department of Rheumatology A, Paris 5 University, Assistance Publique Hopitaux de Paris, Cochin Hospital, Paris, France
    Clin Exp Rheumatol 23:199-204. 2005
    ..To investigate the prevalence and clinical significance of antiphospholipid antibodies in patients with systemic sclerosis (SSc)...
  35. ncbi Prevalence of systemic sclerosis in a French multi-ethnic county
    V Le Guern
    Department of Internal Medicine, Hopital Avicenne, Assistance Publique Hopitaux de Paris, Bobigny, France
    Rheumatology (Oxford) 43:1129-37. 2004
    ..To assess the prevalence of systemic sclerosis (SSc) in a French multi-ethnic population and to examine ethnic differences...
  36. ncbi N-terminal connective tissue growth factor is a marker of the fibrotic phenotype in scleroderma
    M Dziadzio
    Centre for Rheumatology, Royal Free Hospital and University College School of Medicine, Pond Street, London, UK
    QJM 98:485-92. 2005
    ..Elevated production of CTGF might be responsible for maintenance of the fibrotic phenotype in scleroderma. Assays of CTGF or of its fragments are potential non-invasive measures of the fibrotic response in scleroderma...
  37. 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...
  38. ncbi Augmented production of transforming growth factor-beta by cultured peripheral blood mononuclear cells from patients with systemic sclerosis
    Minoru Hasegawa
    Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan
    Arch Dermatol Res 296:89-93. 2004
    ..Thus, the enhanced production of TGF-beta by PBMC may contribute to the disease process in SSc..
  39. ncbi HLA markers for susceptibility and expression in scleroderma
    Dafna D Gladman
    Centre for Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto Western Hospital, Ontario, Canada
    J Rheumatol 32:1481-7. 2005
    ..Our objective was (1) to determine the role of HLA alleles in the susceptibility to scleroderma; and (2) to determine the role of HLA alleles in various aspects of disease expression...
  40. ncbi Delayed gastric emptying in patients with diffuse versus limited systemic sclerosis, unrelated to gastrointestinal symptoms and myoelectric gastric activity
    K Franck-Larsson
    Department of Medicine, Division of Rheumatology, University Hospital, Uppsala, Sweden
    Scand J Rheumatol 32:348-55. 2003
    ..To study the gastric function in patients with diffuse and limited systemic sclerosis (SSc) in relation to the degree of upper gastrointestinal symptoms...
  41. pmc Alpha4beta1 and alpha4beta7 CD4 T cell numbers increase and CLA CD4 T cell numbers decrease in systemic sclerosis
    E Scala
    Istituto Dermopatico dell Immacolata, Istituto di Ricovero e Cura a Carattere Scientifico IDI IRCCS, Rome, Italy
    Clin Exp Immunol 139:551-7. 2005
    ....
  42. ncbi Antifibrotic effects of hepatocyte growth factor on scleroderma fibroblasts and analysis of its mechanism
    Ryoko Sherriff-Tadano
    Graduate School of Medical Science, Saga Medical School, Saga, Japan
    Mod Rheumatol 16:364-71. 2006
    ..The present study indicates that HGF may be a promising therapeutic agent for this intractable disease...
  43. ncbi Nursing problems of patients with systemic sclerosis
    M Sierakowska
    Department of General Nursing, Medical University of Białystok, Poland
    Adv Med Sci 52:147-52. 2007
    ..The most characteristic form SSc is limited and diffuse systemic sclerosis. The SSc is characterized by the progressive fibrosis of the skin and internal organs, leading to their failure, morphology and blood vessels disorders...
  44. ncbi Prevalence and clinical associations of anti-Ku antibodies in patients with systemic sclerosis: a European EUSTAR-initiated multi-centre case-control study
    B Rozman
    University Medical Centre, Department of Rheumatology, Vodnikova 62, SI 1000 Ljubljana, Slovenia
    Ann Rheum Dis 67:1282-6. 2008
    ..To determine the prevalence of anti-Ku antibodies in 625 patients with systemic sclerosis (SSc) from six European rheumatological centres and to evaluate their clinical and serological characteristics...
  45. ncbi Increased serum soluble CD40 levels in patients with systemic sclerosis
    Kazuhiro Komura
    Department of Dermatology, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan
    J Rheumatol 34:353-8. 2007
    ..To determine serum levels of soluble CD40 (sCD40) and clinical association in patients with systemic sclerosis (SSc)...
  46. pmc New therapeutic strategies for systemic sclerosis--a critical analysis of the literature
    Gisele Zandman-Goddard
    Department of Medicine B and Center for Autoimmune Diseases, Sheba Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Israel
    Clin Dev Immunol 12:165-73. 2005
    ..Novel therapies are currently being tested in the treatment of SSc and have the potential of modifying the disease process and overall clinical outcome. The evaluation of these studies is still a difficult process...
  47. pmc Intravenous immunoglobulins improve the function and ameliorate joint involvement in systemic sclerosis: a pilot study
    F Nacci
    Department of Medicine and Surgery, University of Florence, Italy
    Ann Rheum Dis 66:977-9. 2007
    ..In systemic sclerosis (SSc), joint involvement may reduce the functional capacity of the hands. Intravenous immunoglobulins have previously been shown to benefit patients with SSc...
  48. ncbi Prevalence and characteristics of moderate to severe pulmonary hypertension in systemic sclerosis with and without interstitial lung disease
    David Launay
    Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Regional University Hospital Claude Huriez Hospital, Lille, France
    J Rheumatol 34:1005-11. 2007
    ..To determine the prevalence and characteristics of moderate to severe pulmonary hypertension (PH) in patients with systemic sclerosis (SSc) with and without interstitial lung disease (ILD)...
  49. ncbi Analysis of the 5' flanking region of the interleukin 10 gene in patients with systemic sclerosis
    A Crilly
    Department of Medicine, Glasgow Royal Infirmary, Glasgow, UK
    Rheumatology (Oxford) 42:1295-8. 2003
    ..We postulated that there would be a difference in IL-10 polymorphisms in patients with limited (lSSc) and diffuse (dSSc) disease...
  50. 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...
  51. ncbi Association of polymorphisms in the IL1B and IL2 genes with susceptibility and severity of systemic sclerosis
    Silvia Mattuzzi
    Dipartimento di Patologia, Universita di Verona, Verona, Italy
    J Rheumatol 34:997-1004. 2007
    ..To investigate possible associations of 9 single-nucleotide polymorphisms in the IL10, IL1B, IL1A, IL1RN, IL2, LTA, and IL6 genes with susceptibility to systemic sclerosis (SSc), and with clinical subtype of SSc patients...
  52. ncbi Natural remedies for scleroderma
    Alan R Gaby
    Altern Med Rev 11:188-95. 2006
    ..This article reviews several promising natural treatments for scleroderma, including para-aminobenzoic acid, vitamin E, vitamin D, evening primrose oil, estriol, N-acetylcysteine, bromelain, and an avocado/soybean extract...
  53. ncbi Autoantibodies in systemic sclerosis (scleroderma): clues for clinical evaluation, prognosis and pathogenesis
    Alfred Grassegger
    Dermatology, Phlebology and Laser Medicine, Salurner Strasse 15, Innsbruck, Austria
    Wien Med Wochenschr 158:19-28. 2008
    ..Anti-RNA polymerase I/III antibodies are associated with a high risk for renal involvement. Autoantibodies against the PDGF receptor and fibrillin-1 seem to play important roles in the pathogenetic process of systemic sclerosis...
  54. ncbi Pregnancy in scleroderma
    Virginia D Steen
    Department of Medicine, Georgetown University Medical Center, Washington, DC 20007, USA
    Rheum Dis Clin North Am 33:345-58, vii. 2007
    ..Women who have diffuse scleroderma are at a greater risk for developing serious cardiopulmonary and renal problems early in the disease so ..
  55. ncbi [Gastrointestinal involvement in systemic sclerosis]
    Isabelle Marie
    Departement de Medecine Interne, Inserm 644, Centre Hospitalier Universitaire, Rouen
    Presse Med 35:1952-65. 2006
    ..Anorectal involvement is frequent in SSc patients (50-70%) and causes fecal incontinence and rectal prolapse. Other digestive manifestations, including liver impairment, are less common in SSc...
  56. ncbi [Interstitial lung disease in systemic sclerosis]
    Luc Mouthon
    Service de Medecine Interne, Hopital Cochin, Centre de Référence pour les Vascularites et la Sclérodermie Systémique, AP HP, Paris
    Presse Med 35:1943-51. 2006
    ..A subgroup of patients with rapidly progressive ILD might benefit from intravenous cyclophosphamide pulses in association with 15 mg/d prednisone...
  57. ncbi Scleroderma in South Australia: further epidemiological observations supporting a stochastic explanation
    P J Roberts-Thomson
    Department of Immunology, Allergy and Arthritis, Flinders Medical Center, Adelaide, South Australia, Australia
    Intern Med J 36:489-97. 2006
    ..5-fold); however, the majority of the disease variance appears unexplained. A stochastic explanation based on genetic instability is favoured to explain this paradox...
  58. ncbi [Classification criteria of scleroderma]
    Jean Cabane
    Service de Medecine Interne, Hopital Saint Antoine, Paris
    Presse Med 35:1916-22. 2006
    ..The old term "CREST" tends to be abandoned due to its lack of specificity...
  59. ncbi The efficacy of self-administered stretching for finger joint motion in Japanese patients with systemic sclerosis
    Naoki Mugii
    Department of Dermatology and Division of Rehabilitation Science, Kanazawa University Hospital, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan
    J Rheumatol 33:1586-92. 2006
    ..To determine the efficacy of self-administered stretching of each finger in Japanese patients with systemic sclerosis (SSc)...
  60. ncbi Ischemic scleroderma wounds successfully treated with hyperbaric oxygen therapy
    Y Michael Markus
    Division of Rheumatology, Sunnybrook and Women s College Health Sciences Centre, Toronto, Canada
    J Rheumatol 33:1694-6. 2006
    ..4 ATA with resulting wound healing. This is the first reported successful use of HBOT to treat scleroderma ulcers, and may represent an unrecognized treatment option for these notoriously difficult chronic wounds...
  61. ncbi High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease
    Jonathan G Goldin
    David Geffen School of Medicine at UCLA, Department of Radiology, Los Angeles, CA 90095 1721, USA
    Chest 134:358-67. 2008
    ..The frequency, nature, and progression of interstitial lung disease seen on high-resolution CT (HRCT) scans in patients with diffuse SSc (dcSSc) compared with those with limited SSc (lcSSc) has not been well characterized...
  62. 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...
  63. ncbi Evaluating inflammatory joint disease: how and when can autoantibodies help?
    Olivier Meyer
    Rheumatology Department, Bichat Teaching Hospital, 46, rue Henri Huchard, 75018 Paris, France
    Joint Bone Spine 70:433-47. 2003
    ..In microscopic polyangiitis the ANCAs are peripheral and correspond by ELISA to antimyeloperoxidase antibodies. Tests for other antibodies are less often needed to evaluate inflammatory joint disease...
  64. pmc Correlation of endostatin and tissue inhibitor of metalloproteinases 2 (TIMP2) serum levels with cardiovascular involvement in systemic sclerosis patients
    Bozena Dziankowska-Bartkowiak
    Division of Immunodermatology, Medical University of Lodz, Poland
    Mediators Inflamm 2005:144-9. 2005
    ..The study group comprised 34 patients (19 with limited scleroderma (lSSc) and 15 with diffuse scleroderma (dSSc)). The control group consisted of 20 healthy persons, age and sex matched...
  65. ncbi Hypothalamus-hypophysis-thyroid axis, triiodothyronine and antithyroid antibodies in patients with primary and secondary Sjögren's syndrome
    A G Ozgen
    Ege University Endocrinology Department, Bornova, Izmir, Turkey
    Clin Rheumatol 20:44-8. 2001
    ..In conclusion, we found that T3 autoantibody levels in pSS, were not significantly higher than sSS and normal controls...
  66. ncbi Safety and efficacy of disease-modifying anti-rheumatic agents: focus on the benefits and risks of etanercept
    Roy Fleischmann
    Radiant Research Dallas, Dallas, Texas 75235, USA
    Drug Saf 25:173-97. 2002
    ..Preliminary data show that it may be well tolerated and effective in other rheumatic diseases in which there is over production of TNFalpha...
  67. ncbi Granulomatous anterior uveitis in a patient with CREST syndrome
    A Akman
    Department of Ophthalmology, Faculty of Medicine, Baskent University, Ankara, Turkey
    Ocul Immunol Inflamm 8:201-3. 2000
    ..While a small number of cases of uveitis have been reported in other variant forms of progressive systemic sclerosis, to date there have been no descriptions of uveitis associated with the CREST syndrome...
  68. ncbi Autoantibodies detectable in the sera of silicosis patients. The relationship between the anti-topoisomerase I antibody response and HLA-DQB1*0402 allele in Japanese silicosis patients
    A Ueki
    Department of Hygiene, Kawasaki Medical School, Matsushima, Kurashiki, Japan
    Sci Total Environ 270:141-8. 2001
    ..These findings will be useful for preventing occupational autoimmune diseases...
  69. ncbi Pregnancy in women with systemic sclerosis
    V D Steen
    Department of Medicine, Georgetown University, Washington, DC, USA
    Obstet Gynecol 94:15-20. 1999
    ..To determine pregnancy outcomes in women with systemic sclerosis...
  70. ncbi Clastogenic inosine nucleotide as components of the chromosome breakage factor in scleroderma patients
    C Auclair
    Institut Gustave Roussy Villejuif, Paris, CNRS, France
    Arch Biochem Biophys 278:238-44. 1990
    ..The induction of chromatid type damage by these substances in lymphocytes exposed in the G0 phase of their cell cycle and the prevention of this damage by superoxide dismutase are analogous to the observations with CF...
  71. 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
    ..Recent reports of scleroderma overlap with rheumatoid arthritis suggest distinct features of diffuse scleroderma with positive Scl-70, pulmonary fibrosis, and later seropositive erosive rheumatoid arthritis...
  72. ncbi [mRNA expression of procollagen and SMADs in lesional skin of progressive systemic sclerosis]
    Hai Yan Yu
    Department of Dermatology, Sir Run Run Shaw Hospital, Zhejiang University Medical College, Hangzhou 310016, China
    Zhonghua Yi Xue Za Zhi 87:546-9. 2007
    ..To investigate the mRNA expression of type I and type III procollagen and SMADs in the lesional skin of progressive systemic sclerosis (PSS) and analyze the role in pathogenesis of scleroderma thereof...
  73. ncbi [Detection of circulating antibodies in the blood and immune complex precipitates of patients with autoimmune diseases (preliminary report)]
    M Kiss
    Borgyógyászati Klinika, Szent Györgyi Albert Orvostudományi Egyetem, Szeged
    Orv Hetil 133:1103-4, 1109-10. 1992
    ....
  74. ncbi [Chorea as the presenting form of progressive systemic sclerosis]
    M Seijo Martinez
    Servicio de Neurologia, Complexo Hospitalario de Pontevedra
    Neurologia 15:304-6. 2000
    ..Brain MR imaging confirmed an infarct in the left caudate and adjacent internal capsule. The study of this case supports the hypothesis of a direct primary cerebrovascular damage in progressive systemic sclerosis...
  75. ncbi Impact of thiopurine methyltransferase activity and 6-thioguanine nucleotide concentrations in patients with chronic inflammatory diseases
    Jörg Schedel
    Department of Internal Medicine I, Division of Rheumatology and Clinical Immunology, University Hospital of Regensburg, D 93042 Regensburg, Germany
    Ann N Y Acad Sci 1069:477-91. 2006
    ..Reduced activity of TPMT in patients with chronic inflammatory diseases requiring immunosuppressive therapy with azathioprine, especially below a distinct cutoff, appears to inherit a substantial risk for development of AE...
  76. ncbi Tight skin and limited joint movements as early presentation of Hutchinson-Gilford progeria in a 7-week-old infant
    Lieve Sevenants
    Department of Paediatrics, University Hospital Leuven, Herestraat 49, 3000 Leuven, Belgium
    Eur J Pediatr 164:283-6. 2005
    ..He presented with feeding difficulties and severe, diffuse scleroderma-like lesions, a faint peri-oral cyanosis and prominent scalp veins...
  77. ncbi Therapeutic plasma exchange in a single center: Ibni Sina experience
    Onder Arslan
    Ankara University Faculty of Medicine, Ibni Sina Hospital, Department of Hematology, Hemapheresis Unit and Blood Bank, Sihhiye 06100 Ankara, Turkey
    Transfus Apher Sci 30:181-4. 2004
    ..The number of therapeutic procedures is increasing steadily year by year with growing collaboration of departments other than Hematology. In the aim to demonstrate our single center activity we analyzed our data since four years...
  78. ncbi Autoantibodies to lipoprotein lipase and dyslipidemia in systemic lupus erythematosus
    Morris Reichlin
    Oklahoma Medical Research Foundation and Oklahoma University College of Medicine, Oklahoma City, OK 73104, USA
    Arthritis Rheum 46:2957-63. 2002
    ..To demonstrate the binding of bovine lipoprotein lipase (LPL) by IgG from sera obtained from patients with systemic lupus erythematosus (SLE) and other rheumatic diseases, and the relationship of anti-LPL to triglyceride levels in SLE...
  79. ncbi Successful autologous peripheral blood stem cell transplantation using thiotepa in a patient with systemic sclerosis and cardiac involvement
    Atsushi Komatsuda
    Third Department of Internal Medicine, Akita University School of Medicine, 1 1 1 Hondo, Akita City, Akita 010 8543, Japan
    Tohoku J Exp Med 209:61-7. 2006
    ..The present case report demonstrates that thiotepa can be employed as a conditioning regimen for auto-PBSCT in SSc patients with cardiac involvement in order to reduce cyclophosphamide-induced cardiotoxicity...
  80. ncbi Systemic sclerosis sine scleroderma associated with Wolff-Parkinson-White syndrome
    Y W Park
    Department of Rheumatology, Chonnam National University Medical School, University Hospital, 8 Hak Dong, Dong Ku, Gwangju 501 757, Republic of Korea
    Scand J Rheumatol 36:68-70. 2007
    ..Electrophysiological study confirmed WPW syndrome with left posterior bypass tract. This case highlights cardiac arrhythmia caused by WPW syndrome as a clinical manifestation of the heart in ssSSc...
  81. ncbi Scleroderma in South Australia: epidemiological observations of possible pathogenic significance
    P J Roberts-Thomson
    Department of Immunology, Allergy and Arthritis, Flinders Medical Centre, Adelaide, South Australia
    Intern Med J 31:220-9. 2001
    ..Scleroderma is an autoimmune disorder of unknown cause. Previous epidemiological studies have suggested some regional clustering and associations with occupations involving exposure to silica dusts and hydrocarbons...
  82. ncbi Treatment of systemic sclerosis
    V D Steen
    Department of Medicine, Georgetown University, Washington, DC, USA
    Am J Clin Dermatol 2:315-25. 2001
    ..They are likely to present with all the classic manifestations of scleroderma. Diffuse scleroderma patients have a much more acute systemic onset with marked whole hand swelling and may initially have only ..
  83. pmc Photopheresis: a new therapeutic concept
    R L Edelson
    Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut 06510
    Yale J Biol Med 62:565-77. 1989
    ..The specificity of the induced immunologic reaction probably results from the extremely disproportionate expansion of the pathogenic clone(s), relative to the several million other clones of normal T cells...
  84. ncbi Aortic aneurysm in patients with autoimmune diseases treated with corticosteroids
    N Ohara
    Department of Surgery, Faculty of Medicine, The University of Tokyo, Japan
    Int Angiol 19:270-5. 2000
    ..The purpose of this study was to clarify the characteristic clinical features and the pathological mechanism of aneurysmal formation in those patients treated with corticosteroids...
  85. ncbi Silica and renal diseases: no longer a problem in the 21st century?
    P Stratta
    Department of Internal Medicine, Nephrology Section of the Faculty of Medicine, S Giovanni Molinette Hospital, University of Turin, Italy
    J Nephrol 14:228-47. 2001
    ..An occupational history must be obtained for all renal patients, checking particularly for exposure to silica, heavy metals, and solvents...
  86. ncbi Improvement in skin thickening in systemic sclerosis associated with improved survival
    V D Steen
    Georgetown University Medical Center, Division of Rheumatology, Immunology and Allergy, Department of Medicine, Washington, DC 20007, USA
    Arthritis Rheum 44:2828-35. 2001
    The natural history of changes in skin thickening in diffuse scleroderma is quite variable, but the significance of these changes is not clear...
  87. pmc Risk of cancer in patients with scleroderma: a population based cohort study
    C L Hill
    Rheumatology Unit, The Queen Elizabeth Hospital, Woodville Rd, Woodville, South Australia 5011
    Ann Rheum Dis 62:728-31. 2003
    ..Previous studies have suggested an increased risk of cancer among patients with scleroderma...
  88. ncbi Porphyria cutanea tarda presenting as scleroderma
    Raquel Zemtsov
    University Dermatology Center, PC, Muncie, IN 47303, USA
    Cutis 85:203-5. 2010
    ..Upon initiation of therapeutic phlebotomy, fingertip ulcerations and sclerodactyly resolved, and there was a notable improvement of sclerodermatous skin changes of the face and forearms...
  89. ncbi Diagnosis and treatment of scleroderma
    Zainal Arifin Adnan
    Department of Internal Medicine, Sebelas Maret University Dr Moewardi Hospital, Jl Kolonel Sutarto 132 Surakarta
    Acta Med Indones 40:109-12. 2008
    ..in the United States, the synonyms from this disease including Progressive systemic sclerosis (PSS), or diffuse scleroderma. Scleroderma is a multisystem disorder characterized by skin thickening and vascular abnormalities...
  90. ncbi [Extracorporeal photopheresis in progressive systemic sclerosis: discrimination of responders and non-responders]
    Stefanie Reich
    Klinik für Dermatologie und Allergologie der Ruhr Universität, Bochum, Deutschland
    J Dtsch Dermatol Ges 1:945-51. 2003
    ..There is limited experience with the long-term use of ExP in PSS. The purpose of the present study was to distinguish between responders and non-responders by using ExP in PSS and to evaluate activation markers for PSS...
  91. pmc Coexistence of antitopoisomerase I and anticentromere antibodies in patients with systemic sclerosis
    T Dick
    Rheumaklinik u Rheumaforschungsinstitut, Aachen, Germany
    Ann Rheum Dis 61:121-7. 2002
    ..Antibodies targeting DNA topoisomerase I (ATA) or centromere proteins (ACA) are associated with clinical subsets of patients with systemic sclerosis (SSc). The occurrence of those autoantibodies is considered to be mutually exclusive...
  92. 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...
  93. pmc Over-expression of the decoy receptor 3 (DcR3) gene in peripheral blood mononuclear cells (PBMC) derived from silicosis patients
    T Otsuki
    Department of Hygiene, Kawasaki Medical School, Kurashiki, Okayama, Japan
    Clin Exp Immunol 119:323-7. 2000
    ..It will be necessary to study these patients further, establish an in vitro model of human T cells exposed recurrently to silica compounds, and resolve whether the increase in DcR3 mRNA expression is a cause or consequence of disease...
  94. ncbi Fluorescence microlymphography: diagnostic potential in lymphedema and basis for the measurement of lymphatic pressure and flow velocity
    A Bollinger
    Clinic of Angiology, University Hospital of Zurich, Zurich, Switzerland
    Lymphology 40:52-62. 2007
    ....
  95. ncbi [Clinical study of interstitial lung disease in mixed connective tissue disease]
    G Colin
    Service de Pneumologie et Immunoallergologie, Centre de compétence des maladies pulmonaires rares, Clinique des Maladies Respiratoires, hôpital A Calmette, CHRU de Lille, 59037 Lille Cedex, France
    Rev Mal Respir 27:238-46. 2010
    ..MCTD-ILD characteristics are not specific. When systemic sclerosis manifestations are present, MCTD-ILD seems to associate more frequently pulmonary hypertension and progressive ILD...
  96. ncbi Paraspinal calcinosis associated with progressive systemic sclerosis. Case report
    M S Arginteanu
    Department of Neurosurgery, Mount Sinai Medical Center, New York, New York 10029, USA
    J Neurosurg 87:761-3. 1997
    ..Internal fixation was indicated to correct the instability and decompress the spinal canal. Medical therapy was instituted to arrest or reverse the ongoing calcinosis...
  97. ncbi Effects of cisapride on oesophageal transit of solids in patients with progressive systemic sclerosis
    S J Wang
    Department of Nuclear Medicine, Taichung Veterans General Hospital, Taiwan
    Clin Rheumatol 21:43-5. 2002
    ..Each PSS patient was given cisapride 10 mg or placebo orally three times a day in a random, double-blind, crossover fashion. The results show that cisapride does not seem to have any impact on oesophageal transit in patients with PSS...
  98. ncbi [Prevalence and clinical significance of cathepsin G antibodies in systemic sclerosis]
    P Grypiotis
    Cattedra e U O di Reumatologia, Universita degli Studi di Padova, Padova, Italia
    Reumatismo 55:256-62. 2003
    ..To evaluate the prevalence and clinical significance of cathepsin G antibodies in patients affected with systemic sclerosis (SSc, scleroderma)...
  99. ncbi Esophageal hypomotility in systemic sclerosis: close relationship with pulmonary involvement
    K Kinuya
    Department of Radiology, Tonami General Hospital, Toyama, Japan
    Ann Nucl Med 15:97-101. 2001
    ....
  100. ncbi [Clinical characteristics of juvenile dermatomyositis]
    Tamas Constantin
    Semmelweis Egyetem, Általános Orvostudományi Kar, II sz Gyermekgyógyászati Klinika, Budapest
    Orv Hetil 144:1245-50. 2003
    ....
  101. ncbi Effects of cisapride on colonic transit in patients with progressive systemic sclerosis
    S J Wang
    Department of Nuclear Medicine, Taichung Veterans General Hospital, Taichung, Taiwan
    Clin Rheumatol 21:271-4. 2002
    ..The results showed acceleration in colonic transit in response to cisapride. We conclude that cisapride is effective in the treatment of constipation in patients with PSS...

Research Grants16

  1. Cost Effective Designs for Practitioners
    WENG WONG; Fiscal Year: 2007
    ..Such a website does not exist now. Our website will educate, inform and encourage practitioners to incorporate more optimal design ideas in their work, thereby saving substantial cost in their studies. [unreadable] [unreadable]..
  2. Outcomes Research in Rheumatic Diseases
    Dinesh Khanna; Fiscal Year: 2013
    ..oral cyclophosphamide in 150 patients with active SSc-ILD. 2. To develop an early diffuse scleroderma observational registry through the coordinated efforts of several young investigators at large U.S...
  3. Mineralization/Anti-Mineralization Networks in the Skin
    Jouni Uitto; Fiscal Year: 2013
    ....
  4. Retinoic Acid Modulation for Scleroderma
    BERT J W M OEHLEN; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Systemic sclerosis (SSc) or diffuse scleroderma is a complex, chronic, autoimmune, connective tissue disease which primarily causes skin thickening and hardening in addition to interstitial fibrosis ..
  5. Targeting Adiponectin Signaling: Novel Peptide Therapy for Scleroderma
    John Varga; Fiscal Year: 2013
    ..Levels of ADP were significantly reduced in skin biopsies and in serum from patients with early diffuse scleroderma. These observations suggest a novel paradigm for fibrosis linking impaired adipogenesis, reduced ADP ..
  6. Developing Clinical Risk Predictions Rules in Early Diffuse Schleroderma
    ROBYN THERESE DOMSIC; Fiscal Year: 2013
    ..proposal is to develop both a two- and five-year mortality risk prediction rule for patients with early diffuse scleroderma while using state of the art methodologic guidelines and a true inception cohort from a large, well- ..
  7. 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...
  8. VASCULOPATHY, APOPTOSIS AND AUTOIMMUNITY
    JOSEPH AHEARN; Fiscal Year: 2003
    ..It has also been recently demonstrated that several of the autoantigens targeted in diffuse scleroderma are uniquely susceptible to cleavage by metal-catalyzed oxidation reactions similar to what may occur ..
  9. Provisional Combined Response Index For Patients With Diffuse Systemic Sclerosis
    Daniel Furst; Fiscal Year: 2009
    ..Lay Language: We propose to develop a response criteria for clinical trials in diffuse scleroderma that can facilitate drug development and hopefully, can lead to effective treatments for scleroderma.
  10. BIOLOGY OF HUMAN INTRAEPITHELIAL LYMPHOCYTES
    Ellen Ebert; Fiscal Year: 2002
    ..These studies will determine the mechanism of IEL chemotaxis toward secreted products of ECs, why IL-15 is more potent than IL-2 in inducing LAK activity by IELs, and how IL-10 and IL-12 augment this activity. ..
  11. Cardiopulmonary Exercise Testing: Pulmonary Hypertension
    Ronald Oudiz; Fiscal Year: 2007
    .._l.Center. PHS 398/2590(Rev.05/01) Page _, LJ ContinuationFormatPage NumberpagesconsecutivelyatthebottomthroughoutheapplicationD. o no_u_stesuffixesuchas3a, 3b. ..
  12. Alveolitis and Fibrosis in Scleroderma Lung Disease
    Jonathan Goldin; Fiscal Year: 2004
    ..These findings will potentially improve our understanding of the relationship between alveolitis and fibrosis and to the pathogenesis of fibrosis in SLD. ..
  13. A New Antioxidant Prevents Toxicity of HIV Proteins with Methamphetamine
    Nuran Ercal; Fiscal Year: 2007
    ..unreadable] [unreadable] [unreadable]..
  14. Oklahoma Specialized Center of Research in SLE
    Morris Reichlin; Fiscal Year: 2006
    ....
  15. Molecular Mechanism of PARC-Mediated Lung Fibrosis
    Sergei Atamas; Fiscal Year: 2007
    ..4. Analyze the effects of pulmonary adenovector-mediated gene transfer of PARC on lung fibrosis in intact mouse lung and bleomycin-treated mouse lung. ..
  16. CD40 Regulation of IL-4 and IFN-g Effects on Fibroblasts
    Sergei Atamas; Fiscal Year: 2003
    ..immune cells. The proposed study may contribute to our understanding of the role T cells in the development of fibrosis and lead to novel therapeutic approaches in these diseases. ..