Research Topics
| Fredrick M WigleySummaryAffiliation: Johns Hopkins University Country: USA Publications
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Detail Information
Publications
The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community-based rheumatologists (the UNCOVER study)Fredrick M Wigley
Johns Hopkins University, Baltimore, Maryland 21224, USA
Arthritis Rheum 52:2125-32. 2005..We sought to determine the point prevalence of undiagnosed PAH in community-based rheumatology practices...- Vascular disease in sclerodermaFredrick M Wigley
Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA
Clin Rev Allergy Immunol 36:150-75. 2009..This review will outline some of the evidence for the causes and consequences of scleroderma vascular disease... - Systemic sclerosis (scleroderma) is unique among our rheumatic diseases. PrefaceFredrick M Wigley
Johns Hopkins Scleroderma Center, John Hopkins University, 5200 Eastern Avenue, Suite 4100, Mason F. Lord Building, Center Tower, Baltimore, MD 21224, USA
Rheum Dis Clin North Am 34:xi-xiii. 2008 - Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung diseaseStephen C Mathai
Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
Arthritis Rheum 60:569-77. 2009..This study was undertaken to characterize determinants of survival among SSc patients with either type of PH who received PAH-specific therapy... - Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertensionAránzazu Campo
MD, Division of Pulmonary and Critical Care Medicine, 1830 E Monument St, Fifth Floor, Baltimore, MD 21205, USA
Am J Respir Crit Care Med 182:252-60. 2010..Objectives: To identify risk factors of mortality in a well-characterized cohort of patients with PAH related to systemic sclerosis (SSc-PAH)... - T cell polarization identifies distinct clinical phenotypes in scleroderma lung diseaseFrancesco Boin
Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21224, USA
Arthritis Rheum 58:1165-74. 2008..The aim of this study was to investigate whether a polarized T cell phenotype in SSc is associated with lung disease or other clinical manifestations of SSc... - Independent association of anti-beta(2)-glycoprotein I antibodies with macrovascular disease and mortality in scleroderma patientsFrancesco Boin
Johns Hopkins University School of Medicine, Baltimore, Maryland 21224, USA
Arthritis Rheum 60:2480-9. 2009..This study was undertaken to investigate whether anti-beta2GPI and aCL are correlated with macrovascular disease, including ischemic digital loss and PAH, in SSc patients... - Late-age onset systemic sclerosisRebecca L Manno
Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA
J Rheumatol 38:1317-25. 2011.... - Scleroderma patients with combined pulmonary hypertension and interstitial lung diseaseBetty Chang
Department of Medicine, Johns Hopkins University, Baltimore, Maryland, USA
J Rheumatol 30:2398-405. 2003..We studied the demographics, clinical features, and prognosis of individuals with both vascular and interstitial lung disease... - Age and risk of pulmonary arterial hypertension in sclerodermaLionel Schachna
Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA
Chest 124:2098-104. 2003..CONCLUSIONS: We identified increasing age at scleroderma onset as a risk factor for PAH. Vigilance among these high-risk patients may provide an opportunity to intervene prior to development of irreversible pulmonary vascular disease... - Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertensionAmi A Shah
Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Mason F Lord Building Center Tower, Suite 4100, Baltimore, MD 21224, USA
J Rheumatol 37:98-104. 2010..We investigated whether increased numbers of telangiectases in scleroderma associate with measures of pulmonary vascular disease... - Identification of candidate genes in scleroderma-related pulmonary arterial hypertensionDmitry N Grigoryev
Division of Allergy and Clinical Immunology, Johns Hopkins University, Baltimore, MD 21224, USA
Transl Res 151:197-207. 2008..Deciphering the role of genes involved in vascular remodeling and PAH development may reveal new treatment targets for this devastating disorder... - Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertensionLionel Schachna
Johns Hopkins University, Baltimore, Maryland 21224, USA
Arthritis Rheum 54:3954-61. 2006..Lung transplantation may represent a viable therapeutic option to consider for patients with end-stage lung disease due to scleroderma... - Long-term experience of mycophenolate mofetil for treatment of diffuse cutaneous systemic sclerosisElizabeth N Le
Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
Ann Rheum Dis 70:1104-7. 2011..Immunosuppressive therapy may potentially alter the natural disease course of scleroderma. There have been reports of using mycophenolate mofetil (MMF) for the treatment of scleroderma skin disease... - Evaluation of topoisomerase-1-specific CD8+ T-cell response in systemic sclerosisFrancesco Boin
Division of Rheumatology, The Johns Hopkins University School of Medicine, 5200 Eastern Ave, Mason F Lord Bldg, Center Tower, Ste 4100, Baltimore, MD 21224, USA
Ann N Y Acad Sci 1062:137-45. 2005..94%). This small-volume ex vivo assay may prove to be a sensitive and specific tool to assess disease activity and to monitor response to therapy in patients with scleroderma... - Efficacy and tolerability of a selective alpha(2C)-adrenergic receptor blocker in recovery from cold-induced vasospasm in scleroderma patients: a single-center, double-blind, placebo-controlled, randomized crossover studyRobert A Wise
Johns Hopkins School of Medicine, Baltimore, Maryland 21224, USA
Arthritis Rheum 50:3994-4001. 2004.... - Understanding, assessing and treating Raynaud's phenomenonFrancesco Boin
Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21224, USA
Curr Opin Rheumatol 17:752-60. 2005..SUMMARY: Remarkable progress towards a rational approach to the management and treatment of Raynaud's phenomenon is emerging... - Long-term outcome of bosentan treatment in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with the scleroderma spectrum of diseasesReda E Girgis
Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University, School of Medicine, Baltimore, Maryland, USA
J Heart Lung Transplant 24:1626-31. 2005..Scant data are available regarding PAH associated with the scleroderma spectrum of diseases (APAH-SSD). Here we review our experience with bosentan in these 2 groups... - Persistence of abnormal bronchoalveolar lavage findings after cyclophosphamide treatment in scleroderma patients with interstitial lung diseaseShikha Mittoo
Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Baltimore, MD 21224, USA
Arthritis Rheum 56:4195-202. 2007.... - Abnormalities in the regulators of angiogenesis in patients with sclerodermaLaura K Hummers
Department of Medicine, Johns Hopkins University, Baltimore, MD 21224, USA
J Rheumatol 36:576-82. 2009..To determine plasma levels of regulators of angiogenesis in patients with scleroderma and to correlate those levels with manifestations of scleroderma-related vascular disease... - Diffuse fasciitis with eosinophilia developing after local irradiation for breast cancerNoelle S Sherber
Department of Dermatology, Johns Hopkins Medical Institutions, 601 North Caroline Street, 6th Floor, Baltimore, MD 21205, USA
Clin Rheumatol 28:729-32. 2009.... - Recognition of Granzyme B-generated autoantigen fragments in scleroderma patients with ischemic digital lossLionel Schachna
Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
Arthritis Rheum 46:1873-84. 2002..To examine whether autoantibodies recognizing granzyme B (GB)-cleaved autoantigens are associated with ischemic digital loss (IDL) in limited systemic sclerosis (SSc)... - Clinical practice. Raynaud's PhenomenonFredrick M Wigley
Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
N Engl J Med 347:1001-8. 2002 - "Still" ill?Peter K Wung
Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA
Am J Med 121:491-3. 2008 - Raynaud's phenomenon in mixed connective tissue diseaseThomas Grader-Beck
Division of Rheumatology, Johns Hopkins School of Medicine, 5200 Eastern Avenue, Suite 4100, Baltimore, MD 21224, USA
Rheum Dis Clin North Am 31:465-81, vi. 2005..Therapeutic regimens include the traditional use of calcium channel blockers and novel vascular therapies... - Resolution of pansclerotic morphea after treatment with antithymocyte globulinPhilip Song
Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA
Nat Rev Rheumatol 5:513-6. 2009..He also reported difficulties with swallowing and a 20 kg weight loss... - Responsiveness of the SF-36 and the Health Assessment Questionnaire Disability Index in a systemic sclerosis clinical trialDinesh Khanna
Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, Los Angeles, California, USA
J Rheumatol 32:832-40. 2005.... - Targeting mediators of vascular injury in sclerodermaLionel Schachna
Division of Rheumatology, Departments of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
Curr Opin Rheumatol 14:686-93. 2002..Finally, the authors look at the potential role of biomarkers as surrogate indicators of active vascular disease in scleroderma... - Autoantibodies against B23, a nucleolar phosphoprotein, occur in scleroderma and are associated with pulmonary hypertensionDanielle B Ulanet
Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
Arthritis Rheum 49:85-92. 2003..The demonstration that antibodies to B23 are associated with an increased prevalence of pulmonary hypertension points to anti-B23 antibodies as a possible marker of a specific phenotype in scleroderma... - Treatment of scleroderma: an updateSangeeta D Sule
Division of Rheumatology, Johns Hopkins University, Baltimore, MD 21205, USA
Expert Opin Investig Drugs 12:471-82. 2003..More experience in well-designed clinical trials is needed to define the role of these agents in treating scleroderma... - Digital necrosis related to carboplatin and gemcitabine therapy in systemic sclerosisMegan E B Clowse
Division of Rheumatology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
J Rheumatol 30:1341-3. 2003..This case illustrates the risk of severe digital ischemia and digital loss in patients with scleroderma during chemotherapy with carboplatin and gemcitabine... - Management of Raynaud's phenomenon and digital ischemic lesions in sclerodermaLaura K Hummers
Division of Rheumatology, Johns Hopkins University, Baltimore, MD 21205, USA
Rheum Dis Clin North Am 29:293-313. 2003..Critical ischemia can cause deep tissue injury and digital loss. An aggressive approach to reversing an ischemic crisis is essential for preventing these severe events... - Disease severity as a predictor of outcome in sclerodermaAllan C Gelber
Division of Molecular and Clinical Rheumatology, Johns Hopkins University, Baltimore, MD 21205, USA
Lancet 359:277-9. 2002 - Scleromyxedema: a case series highlighting long-term outcomes of treatment with intravenous immunoglobulin (IVIG)Marissa Blum
Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA
Medicine (Baltimore) 87:10-20. 2008..We discuss the potential therapeutic benefit of IVIG in this condition, where the pathophysiology of the disease is poorly understood, and underscore new data on the potential mechanism of action of IVIG therapy... - Often forgotten manifestations of systemic sclerosisAmi A Shah
Division of Rheumatology, Johns Hopkins University, 5200 Eastern Avenue, Mason F Lord Building, Center Tower, Suite 4100, Baltimore, MD 21224, USA
Rheum Dis Clin North Am 34:221-38; ix. 2008.... - Occurrence of an activated, profibrotic pattern of gene expression in lung CD8+ T cells from scleroderma patientsIrina G Luzina
Research Service, Veterans Affairs Maryland Health Care System, Baltimore VA Medical Center, Room 3C-125, 10 North Greene Street, Baltimore, MD 21201, USA
Arthritis Rheum 48:2262-74. 2003.... - Gene expression in bronchoalveolar lavage cells from scleroderma patientsIrina G Luzina
Research Service, Veterans Affairs Maryland Health Care System and Department of Medicine, University of Maryland School of Medicine, Baltimore, Maryland 21201, USA
Am J Respir Cell Mol Biol 26:549-57. 2002.... - Paclitaxel modulates TGFbeta signaling in scleroderma skin grafts in immunodeficient miceXialin Liu
Division of Cardiology, Department of Medicine, Duke University Medical Center, Durham, North Carolina, United States of America
PLoS Med 2:e354. 2005.... - Measuring disease activity and functional status in patients with scleroderma and Raynaud's phenomenonPeter A Merkel
Harvard Medical School, Boston, Massachusetts, USA
Arthritis Rheum 46:2410-20. 2002.... - Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in sclerodermaBetty Chang
Division of Pulmonary and Critical Care Medicine, University of New Mexico, Albuquerque, 87131, USA
J Rheumatol 33:269-74. 2006..To determine risk factors for developing pulmonary hypertension (PH) in patients with scleroderma (SSc, systemic sclerosis)... - Deficient Smad7 expression: a putative molecular defect in sclerodermaChunming Dong
Division of Cardiology, Duke University Medical Center, 7504 Duke Hospital North, Box 3845, Durham, NC 27710, USA
Proc Natl Acad Sci U S A 99:3908-13. 2002..These results suggest that alterations in the Smad pathway, including marked Smad7 deficiency and Smad3 up-regulation, may be responsible for TGF-beta hyperresponsiveness observed in scleroderma... - Increased circulating concentrations of the counteradhesive proteins SPARC and thrombospondin-1 in systemic sclerosis (scleroderma). Relationship to platelet and endothelial cell activationRichard F Macko
Department of Neurology, VA Maryland Health Care Center, Baltimore 21201, USA
J Rheumatol 29:2565-70. 2002.... - Correlation of the degree of dyspnea with health-related quality of life, functional abilities, and diffusing capacity for carbon monoxide in patients with systemic sclerosis and active alveolitis: results from the Scleroderma Lung StudyDinesh Khanna
David Geffen School of Medicine, University of California, Los Angeles, USA
Arthritis Rheum 52:592-600. 2005.... - Current drug therapy for scleroderma and secondary Raynaud's phenomenon: evidence-based reviewSheridan Henness
Wolters Kluwer Health Adis, Auckland, New Zealand
Curr Opin Rheumatol 19:611-8. 2007..Some data suggest some benefits to the use of the phosphodiesterase inhibitor sildenafil, the serotonin uptake inhibitor fluoxetine and the angiotensin receptor inhibitor losartan... - Antineutrophil cytoplasmic antibody-positive crescentic glomerulonephritis in scleroderma--a different kind of renal crisisDiane L Kamen
Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, South Carolina 29425, USA
J Rheumatol 33:1886-8. 2006..We suggest that the presence of ANCA be routinely evaluated when faced with renal failure in the setting of SSc... - Bullous pemphigoid in a patient with systemic sclerosis (scleroderma)Noelle S Sherber
J Rheumatol 33:2098. 2006 - Autoimmune disorders: nail signs and therapeutic approachesNoelle S Sherber
Columbia University Medical Center, New York, New York 10032, USA
Dermatol Ther 20:17-30. 2007..Whereas oral calcium channel blockers remain the most convenient therapy, oral endothelin receptor antagonists and intravenous prostaglandins may be important therapeutic advances for ischemic digital vascular lesions... - Efficacy and safety of etanercept in the treatment of scleroderma-associated joint diseaseGordon K Lam
J Rheumatol 34:1636-7. 2007 - Antiangiogenic plasma activity in patients with systemic sclerosisMary Jo Mulligan-Kehoe
Angiogenesis Research Center, Dartmouth Medical School, Lebanon, New Hampshire 03756, USA
Arthritis Rheum 56:3448-58. 2007..The aim of this study was to investigate the presence of antiangiogenic factors in patients with SSc... - The impact of pain and symptoms of depression in sclerodermaLisa M Benrud-Larson
Mayo Clinic, Rochester, MN, USA
Pain 95:267-75. 2002..Increased attention to effective management of pain and symptoms of depression in scleroderma will likely lead to improved functioning and quality of life...