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1. Full Text EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)
by Kowal-Bielecka, O and Landewé, R and Avouac, J and Chwiesko, S and Miniati, I and Czirjak, L and Clements, P and Denton, C and Farge, D and Fligelstone, K and Földvari, I and Furst, D E and Müller-Ladner, U and Seibold, J and Silver, R M and Takehara, K and Toth, B Garay and Tyndall, A and Valentini, G and van den Hoogen, F and Wigley, F and Zulian, F and Matucci-Cerinic, Marco and EUSTAR and EUSTAR Co-Authors and and the EUSTAR co-authors
Annals of the Rheumatic Diseases, ISSN 0003-4967, 05/2009, Volume 68, Issue 5, pp. 620 - 628
Purpose:The optimal treatment of systemic sclerosis (SSc) is a challenge because the pathogenesis of SSc is unclear and it is an uncommon and clinically... 
ORAL PROSTACYCLIN ANALOG | DOUBLE-BLIND TRIAL | INTRAVENOUS EPOPROSTENOL | PULMONARY-ARTERIAL-HYPERTENSION | RAYNAUDS-PHENOMENON SECONDARY | ENDOTHELIN-RECEPTOR ANTAGONIST | RENAL CRISIS | BACTERIAL OVERGROWTH | CONNECTIVE-TISSUE DISEASE | PLACEBO-CONTROLLED TRIAL | RHEUMATOLOGY | Lung Diseases, Interstitial - etiology | Evidence-Based Medicine - methods | Raynaud Disease - drug therapy | Humans | Gastrointestinal Diseases - etiology | Kidney Diseases - drug therapy | Treatment Outcome | Raynaud Disease - etiology | Lung Diseases, Interstitial - drug therapy | Gastrointestinal Diseases - drug therapy | Scleroderma, Systemic - complications | Hypertension, Pulmonary - drug therapy | Kidney Diseases - etiology | Hypertension, Pulmonary - etiology | Scleroderma, Systemic - drug therapy | Medical research | Care and treatment | Usage | Systemic scleroderma | Scleroderma (Disease) | Medicine, Experimental | Reports | Management | Evidence-based medicine | Disease | Antibiotics | Experts | Mortality | Ulcers | Quality | Committees | Rheumatism | Scleroderma | Patients
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2. Full Text Review: Frontiers of Antifibrotic Therapy in Systemic Sclerosis
by Distler, Jörg H. W and Feghali‐Bostwick, Carol and Soare, Alina and Asano, Yoshihide and Distler, Oliver and Abraham, David J
Arthritis & Rheumatology, ISSN 2326-5191, 02/2017, Volume 69, Issue 2, pp. 257 - 267
Scleroderma, Systemic - etiology | Drug Discovery | Humans | Scleroderma, Systemic - drug therapy | Fibrosis | Scleroderma, Systemic - pathology | Systemic sclerosis
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3. Full Text Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial
by Khanna, Dinesh, Prof and Denton, Christopher P, Prof and Jahreis, Angelika, MD and van Laar, Jacob M, Prof and Frech, Tracy M, MD and Anderson, Marina E, FRCP and Baron, Murray, MD and Chung, Lorinda, MD and Fierlbeck, Gerhard, MD and Lakshminarayanan, Santhanam, MD and Allanore, Yannick, Prof and Pope, Janet E, MD and Riemekasten, Gabriela, Prof and Steen, Virginia, Prof and Müller-Ladner, Ulf, Prof and Lafyatis, Robert, MD and Stifano, Giuseppina, MD and Spotswood, Helen, PhD and Chen-Harris, Haiyin, PhD and Dziadek, Sebastian, PhD and Morimoto, Alyssa, PhD and Sornasse, Thierry, PhD and Siegel, Jeffrey, MD and Furst, Daniel E, Prof
The Lancet, ISSN 0140-6736, 06/2016, Volume 387, Issue 10038, pp. 2630 - 2640
Summary Background Systemic sclerosis is a rare disabling autoimmune disease with few treatment options. The efficacy and safety of tocilizumab, an interleukin... 
Internal Medicine | INTERSTITIAL LUNG-DISEASE | MORTALITY | SURVIVAL | MEDICINE, GENERAL & INTERNAL | PLACEBO | LYSOPHOSPHATIDIC ACID | DIFFUSE SCLERODERMA | SKIN FIBROSIS | INTERLEUKIN-6 | EXPRESSION | SSC SKIN | Biomarkers - metabolism | Antibodies, Monoclonal, Humanized - therapeutic use | Double-Blind Method | Europe | Humans | Middle Aged | Scleroderma, Systemic - metabolism | Male | Treatment Outcome | United Kingdom | Canada | Vital Capacity | Antibodies, Monoclonal, Humanized - administration & dosage | Injections, Subcutaneous | Scleroderma, Systemic - complications | Interleukin-6 - physiology | Adult | Female | Aged | Scleroderma, Systemic - drug therapy | Medical colleges | Adults | Systemic scleroderma | Interleukins | Scleroderma (Disease) | Analysis | Tissue | Cytokines | Ulcers | Biomarkers | Fibroblasts | Clinical trials | Patient safety | Skin diseases | Skin | Health risk assessment | Scleroderma | Drug therapy | Pathogenesis | Interleukin | Medical services | Itching | Interleukin 6 | Cost-benefit analysis | Safety | Thickening | Effectiveness | Mortality | Lung diseases | Medical treatment | Rheumatology | Patients | Studies | Systemic sclerosis | Least squares | Autoimmune diseases
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4. Full Text Effects and safety of rituximab in systemic sclerosis: an analysis from the European Scleroderma Trial and Research (EUSTAR) group
by Jordan, Suzana and Distler, Jörg H W and Maurer, Britta and Huscher, Dörte and van Laar, Jacob M and Allanore, Yannick and Distler, Oliver and EUSTAR Rituximab Study Grp and EUSTAR Rituximab study group
Annals of the Rheumatic Diseases, ISSN 0003-4967, 06/2015, Volume 74, Issue 6, pp. 1188 - 1194
Objectives To assess the effects of Rituximab (RTX) on skin and lung fibrosis in patients with systemic sclerosis (SSc) belonging to the European Scleroderma... 
RHEUMATOID-ARTHRITIS | FIBROSIS | SCORE | B-CELL DEPLETION | Systemic Sclerosis | B cells | RHEUMATOLOGY | TIGHT-SKIN MOUSE | INTERSTITIAL LUNG-DISEASE | Treatment | RESEARCH GROUP DATABASE | AUTOIMMUNE-DISEASES | CD19/CD22 LOOP | DOUBLE-BLIND | Lung Diseases, Interstitial - etiology | Severity of Illness Index | Humans | Immunosuppressive Agents - therapeutic use | Middle Aged | Rituximab | Male | Lung - physiopathology | Lung Diseases, Interstitial - drug therapy | Case-Control Studies | Vital Capacity | Scleroderma, Systemic - complications | Fibrosis | Female | Lung Diseases, Interstitial - physiopathology | Scleroderma, Systemic - drug therapy | Skin - pathology | Antibodies, Monoclonal, Murine-Derived - therapeutic use | Antirheumatic Agents - therapeutic use | Cohort Studies | Databases, Factual | Complications and side effects | Treatment outcome | Care and treatment | Systemic scleroderma | Analysis | Scleroderma (Disease) | Risk factors | Studies | Disease | Cytokines | Biopsy | Lymphomas | Clinical medicine | RNA polymerase | Gene expression | Scleroderma | Data bases
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5. Systemic sclerosis: from pathogenesis to targeted therapy
by Denton, Christopher P and Denton, Christopher P
Clinical and experimental rheumatology, ISSN 0392-856X, 2015, Volume 33, Issue 4, pp. S3 - S7
Systemic sclerosis (scleroderma) leads to morbidity and mortality through a combination of inflammation, fibrosis and vascular damage leading to internal organ... 
Vasculopathy | Scleroderma | Systemic sclerosis | Cytokine | Fibrosis | Growth factor | cytokine | SURVIVAL | scleroderma | INJURY | RHEUMATOLOGY | INTERLEUKIN-6 | growth factor | INTERSTITIAL LUNG-DISEASE | systemic sclerosis | DEPENDENT MOUSE MODEL | fibrosis | vasculopathy | PULMONARY-FIBROSIS | Inflammation Mediators - immunology | Humans | Immunosuppressive Agents - therapeutic use | Scleroderma, Systemic - metabolism | Treatment Outcome | Scleroderma, Systemic - immunology | Molecular Targeted Therapy | Remission Induction | Scleroderma, Systemic - diagnosis | Animals | Signal Transduction - drug effects | Inflammation Mediators - metabolism | Immunosuppressive Agents - adverse effects | Scleroderma, Systemic - drug therapy
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6. Full Text Treatment of Systemic Sclerosis Complications: What to Use When First-Line Treatment Fails—A Consensus of Systemic Sclerosis Experts
by Walker, Kyle M., BMSc (Hons) and Pope, Janet, MD, MPH, FRCPC and CSRG and SCTC and Canadian Scleroderma Research Group (CSRG) and participating members of the Scleroderma Clinical Trials Consortium (SCTC)
Seminars in Arthritis and Rheumatism, ISSN 0049-0172, 2012, Volume 42, Issue 1, pp. 42 - 55
Objectives There is a need for standardization in systemic sclerosis (SSc) management. Methods SSc experts ( n = 117) were sent 3 surveys to gain consensus for... 
Rheumatology | treatment | guidelines | systemic sclerosis | management | scleroderma | algorithm | Treatment | Management | Scleroderma | Algorithm | Systemic sclerosis | Guidelines | PLACEBO | SILDENAFIL | RISK | CONVERTING ENZYME-INHIBITORS | RHEUMATOLOGY | RAYNAUDS-PHENOMENON RESISTANT | TRIAL | RECOMMENDATIONS | DOUBLE-BLIND | Algorithms | Angiotensin-Converting Enzyme Inhibitors - therapeutic use | Humans | Scleroderma, Systemic - complications | Treatment Failure | Calcium Channel Blockers - therapeutic use | Adrenergic alpha-Antagonists - therapeutic use | Disease Management | Drug Therapy, Combination | Angiotensin Receptor Antagonists - therapeutic use | Health Care Surveys - statistics & numerical data | Scleroderma, Systemic - drug therapy | Complications and side effects | Care and treatment | Systemic scleroderma | Scleroderma (Disease)
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7. Full Text Integrin-modulating therapy prevents fibrosis and autoimmunity in mouse models of scleroderma
by Gerber, Elizabeth E and Gallo, Elena M and Fontana, Stefani C and Davis, Elaine C and Wigley, Fredrick M and Huso, David L and Dietz, Harry C
Nature, ISSN 0028-0836, 2013, Volume 503, Issue 7474, pp. 126 - 130
In systemic sclerosis (SSc), a common and aetiologically mysterious form of scleroderma (defined as pathological fibrosis of the skin), previously healthy... 
PLASMACYTOID DENDRITIC CELLS | PATHOGENESIS | TGF-BETA | MULTIDISCIPLINARY SCIENCES | DISEASE | MICE | SKIN | SYSTEMIC-SCLEROSIS | EXPRESSION | FIBROBLASTS | I INTERFERON | Fibrosis - drug therapy | Antibodies, Antinuclear - immunology | Scleroderma, Systemic - pathology | Skin Diseases, Genetic - drug therapy | Male | Integrins - metabolism | Mutation, Missense - genetics | Contracture - drug therapy | Plasma Cells - drug effects | T-Lymphocytes, Helper-Inducer - drug effects | Antibodies, Neutralizing - immunology | Autoimmunity - immunology | Antibodies, Neutralizing - therapeutic use | Integrins - drug effects | Transforming Growth Factor beta - antagonists & inhibitors | Dendritic Cells - drug effects | Female | Contracture - prevention & control | Microfilament Proteins - metabolism | Skin Diseases, Genetic - prevention & control | Contracture - immunology | Fibrosis - prevention & control | Microfilament Proteins - genetics | Transforming Growth Factor beta - immunology | Microfilament Proteins - chemistry | Fibrillin-1 | Skin Diseases, Genetic - immunology | Contracture - pathology | Skin Diseases, Genetic - pathology | Antibodies, Neutralizing - pharmacology | Fibrillins | Scleroderma, Systemic - immunology | Animals | Amino Acid Substitution - genetics | Mice | Fibrosis - pathology | Amino Acid Motifs - genetics | Autoimmunity - drug effects | Scleroderma, Systemic - drug therapy | Scleroderma, Systemic - prevention & control | Autoimmunity | Cell interaction | Systemic scleroderma | Scleroderma (Disease) | Research | Prevention | Complications and side effects | Fibrosis | Physiological aspects | Dosage and administration | Drug therapy | Molecular biology | Health aspects | Integrins | Flow cytometry | Cell culture | Cytokines | Rodents | Collagen | Mutation | Scleroderma | Age
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8. Full Text Incident Vertebral Fractures and Risk Factors in the First Three Years Following Glucocorticoid Initiation Among Pediatric Patients With Rheumatic Disorders
by LeBlanc, Claire MA and Ma, Jinhui and Taljaard, Monica and Roth, Johannes and Scuccimarri, Rosie and Miettunen, Paivi and Lang, Bianca and Huber, Adam M and Houghton, Kristin and Jaremko, Jacob L and Ho, Josephine and Shenouda, Nazih and Matzinger, Mary Ann and Lentle, Brian and Stein, Robert and Sbrocchi, Anne Marie and Oen, Kiem and Rodd, Celia and Jurencak, Roman and Cummings, Elizabeth A and Couch, Robert and Cabral, David A and Atkinson, Stephanie and Alos, Nathalie and Rauch, Frank and Siminoski, Kerry and Ward, Leanne M and Canadian STeroid Associated Osteop and Canadian STeroid-Associated Osteoporosis in Pediatric Population (STOPP) Consortium
Journal of Bone and Mineral Research, ISSN 0884-0431, 09/2015, Volume 30, Issue 9, pp. 1667 - 1675
ABSTRACT Vertebral fractures are an important yet underrecognized manifestation of osteoporosis in children with chronic, glucocorticoid‐treated illnesses. Our... 
GLUCOCORTICOIDS | BONE DENSITY | VERTEBRAL FRACTURES | RHEUMATIC DISORDERS | ADOLESCENTS | CHILDREN | rheumatic disorders | glucocorticoids | adolescents | children | vertebral fractures | bone density | INDUCED OSTEOPOROSIS | SYSTEMIC-LUPUS-ERYTHEMATOSUS | BONE HEALTH | JUVENILE IDIOPATHIC ARTHRITIS | DISEASE-ACTIVITY | THERAPY | GROWTH | ENDOCRINOLOGY & METABOLISM | COMPRESSION FRACTURES | Glucocorticoids - therapeutic use | Lupus Erythematosus, Systemic - complications | Rheumatic Diseases - drug therapy | Scleroderma, Localized - complications | Humans | Male | Osteoporosis - drug therapy | Dermatomyositis - complications | Arthritis, Juvenile - drug therapy | Incidence | Systemic Vasculitis - complications | Dermatomyositis - drug therapy | Scleroderma, Systemic - complications | Lupus Erythematosus, Systemic - drug therapy | Female | Child | Glucocorticoids - adverse effects | Spinal Fractures - diagnosis | Scleroderma, Localized - drug therapy | Bone Density | Risk Factors | Proportional Hazards Models | Spinal Fractures - epidemiology | Systemic Vasculitis - drug therapy | Spinal Fractures - chemically induced | Adolescent | Arthritis, Juvenile - complications | Rheumatic Diseases - complications | Scleroderma, Systemic - drug therapy | Cohort Studies | Pediatrics | Fractures | Corticosteroids | Bones | Children | Health aspects | Density | Risk factors | Steroids
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9. Full Text A multicenter, open-label, comparative study of B-cell depletion therapy with Rituximab for systemic sclerosis-associated interstitial lung disease
by Daoussis, Dimitrios and Melissaropoulos, Konstantinos and Sakellaropoulos, Georgios and Antonopoulos, Ioannis and Markatseli, Theodora E and Simopoulou, Theodora and Georgiou, Panagiotis and Andonopoulos, Andrew P and Drosos, Alexandros A and Sakkas, Lazaros and Liossis, Stamatis-Nick
Seminars in Arthritis and Rheumatism, ISSN 0049-0172, 04/2017, Volume 46, Issue 5, pp. 625 - 631
Rituximab (RTX) may favorably affect lung function and skin fibrosis in patients with systemic sclerosis (SSc). We aimed to assess long-term efficacy and... 
Rituximab | B cells | Scleroderma | Interstitial lung disease | Systemic sclerosis | Fibrosis | CYCLOPHOSPHAMIDE | MECHANISMS | RHEUMATOLOGY | FIBROBLASTS | PATHOGENESIS | AUTOIMMUNITY | SKIN INVOLVEMENT | GENE-EXPRESSION | EXPERIENCE | MYCOPHENOLATE-MOFETIL | LYMPHOCYTES | Humans | Middle Aged | Male | Lymphocyte Depletion | Case-Control Studies | Time Factors | Scleroderma, Systemic - complications | Adult | Female | Retrospective Studies | Immunosuppressive Agents - administration & dosage | Skin - pathology | Rituximab - adverse effects | Lung - pathology | Kaplan-Meier Estimate | Lung Diseases, Interstitial - complications | Lung Diseases, Interstitial - drug therapy | Rituximab - administration & dosage | B-Lymphocytes - drug effects | B-Lymphocytes - immunology | Lung - drug effects | Immunosuppressive Agents - adverse effects | Aged | Scleroderma, Systemic - drug therapy | Skin - drug effects | Systemic scleroderma | Comparative analysis | Lung diseases | Scleroderma (Disease) | Medical research | Medicine, Experimental | Mycophenolate mofetil | Methotrexate
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10. Full Text Cyclophosphamide versus Placebo in Scleroderma Lung Disease
by Tashkin, Donald P and Elashoff, Robert and Clements, Philip J and Goldin, Jonathan and Roth, Michael D and Furst, Daniel E and Arriola, Edgar and Silver, Richard and Strange, Charlie and Bolster, Marcy and Seibold, James R and Riley, David J and Hsu, Vivien M and Varga, John and Schraufnagel, Dean E and Theodore, Arthur and Simms, Robert and Wise, Robert and Wigley, Fredrick and White, Barbara and Steen, Virginia and Read, Charles and Mayes, Maureen and Parsley, Ed and Mubarak, Kamal and Connolly, M. Kari and Golden, Jeffrey and Olman, Mitchell and Fessler, Barri and Rothfield, Naomi and Metersky, Mark and Scleroderma Lung Study Res Grp and Scleroderma Lung Study Research Group
The New England Journal of Medicine, ISSN 0028-4793, 06/2006, Volume 354, Issue 25, pp. 2655 - 2666
Interstitial lung disease is a common complication of systemic sclerosis, but there is no widely accepted treatment. In this randomized, placebo-controlled... 
RHEUMATOID-ARTHRITIS | SURVIVAL | MEDICINE, GENERAL & INTERNAL | MANAGEMENT | NORMAL VALUES | ALVEOLITIS | INTRAVENOUS CYCLOPHOSPHAMIDE | FOLLOW-UP | PULMONARY-FUNCTION | SYSTEMIC-SCLEROSIS | HEALTH-ASSESSMENT QUESTIONNAIRE | Humans | Immunosuppressive Agents - therapeutic use | Middle Aged | Bronchoalveolar Lavage Fluid - immunology | Male | Cyclophosphamide - adverse effects | Cyclophosphamide - therapeutic use | Vital Capacity - drug effects | Leukopenia - chemically induced | Aged, 80 and over | Adult | Female | Lung Diseases, Interstitial - physiopathology | Autoimmune Diseases - drug therapy | Double-Blind Method | Administration, Oral | Treatment Outcome | Scleroderma, Systemic - immunology | Lung Diseases, Interstitial - drug therapy | Lung Diseases, Interstitial - immunology | Regression Analysis | Immunosuppressive Agents - adverse effects | Aged | Respiratory Function Tests | Scleroderma, Systemic - drug therapy | Systemic scleroderma | Drug therapy | Lung diseases | Scleroderma (Disease) | Quality of life | Hypertension | Lungs | Scleroderma | Raynaud disease | Mortality
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11. Full Text Nintedanib inhibits fibroblast activation and ameliorates fibrosis in preclinical models of systemic sclerosis
by Huang, Jingang and Beyer, Christian and Palumbo-Zerr, Katrin and Zhang, Yun and Ramming, Andreas and Distler, Alfiya and Gelse, Kolja and Distler, Oliver and Schett, Georg and Wollin, Lutz and Distler, Jörg H. W
Annals of the Rheumatic Diseases, ISSN 0003-4967, 05/2016, Volume 75, Issue 5, pp. 883 - 890
Background Nintedanib is a tyrosine kinase inhibitor that has recently been shown to slow disease progression in idiopathic pulmonary fibrosis in two replicate... 
C-ABL | KINASE | Systemic Sclerosis | PREVENTION | EXPERIMENTAL DERMAL FIBROSIS | ESTABLISHED FIBROSIS | RHEUMATOLOGY | VERSUS-HOST-DISEASE | GROWTH-FACTOR-BETA | SCLERODERMA | Treatment | Fibroblasts | IDIOPATHIC PULMONARY-FIBROSIS | RECEPTORS | Fibroblasts - physiology | Humans | Scleroderma, Systemic - pathology | Male | Indoles - administration & dosage | Dose-Response Relationship, Drug | Bleomycin | Mice, Mutant Strains | Female | Indoles - pharmacology | Skin - pathology | Disease Models, Animal | Drug Evaluation, Preclinical - methods | Cells, Cultured | Fibroblasts - pathology | Cell Movement - drug effects | Protein Kinase Inhibitors - administration & dosage | Animals | Protein Kinase Inhibitors - therapeutic use | Fibroblasts - drug effects | Fibrosis | Graft vs Host Disease - prevention & control | Indoles - therapeutic use | Cell Proliferation - drug effects | Mice, Inbred BALB C | Protein Kinase Inhibitors - pharmacology | Scleroderma, Systemic - drug therapy | Skin - drug effects | Clinical trials | Complications and side effects | Usage | Care and treatment | Pulmonary fibrosis | Sclerosis
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