Publications

Leaders in the Field of Scleroderma Research

Publications from work done at the Scleroderma Center:

Published Manuscripts

  1. Blum M, Wigley FM, Hummers LK. Scleromyxedema: a case series highlighting long-term outcomes of treatment with intravenous immunoglobulin (IVIG). Medicine. 2008 Jan;87(1):10-20.
  2. Soong TK, Barouch LA, Champion HC, Wigley FM, Halushka MK. New clinical and ultrastructural findings in hydroxychloroquine-induced cardiomyopathy – a report of 2 cases. Hum Pathol. 2007 Dec; 38(12):1858-63.
  3. Distler O, Behrens F, Pittrow D, Huscher D, Denton CP, Foeldvari I, Humbert M, Matucci-Cerinic M, Nash P, Opitz CF, Rubin LJ, Seibold JR, Furst DE for the other coauthors of the EPOSS-OMERACT group.  Defining appropriate outcome measures in pulmonary arterial hypertension related to systemic sclerosis:  Delphi consensus study with cluster analysis.  Arthritis Care Res, in press.
  4. Mittoo S, Wigley FM, Wise R, Xiao H, Hummers L. Persistence of an abnormal BAL after cyclophosphamide treatment in scleroderma interstitial lung disease. Arthritis Rheum. 2007 Dec; 56(12):4195-202.
  5. Heinberg LJ, Kudel I, White B, Kwan A, medley K, Wigley F. Assessing body image in patients with systemic sclerosis (scleroderma): validation of the adapted Satisfaction with Appearance Scale. Body Image. 2007 Mar;4(1):79-86.
  6. Boin F, De Fanis U, Bartlett SJ, Wigley FM, Rosen A, Casolaro V. T cell polarization identifies distinct clinical phenotypes in scleroderma lung disease. Arthritis Rheum, in press. Renda ST, Halushka M, Barouch L, Wigley FM.  New clinical and ultrastructural findings in hydroxychloroquine-induced cardiomyopathy – a report of two cases.  Human Pathol, in press.
  7. Tehlirian CV, Hummers LK, White B, Brodsky RA, Wigley FM.  High dose cyclophosphamide without stem cell rescue in scleroderma.  Ann Rheum Dis. 2007 Nov 1; [Epub ahead of print].
  8. Strange C, Bolster MB, Roth MD, Silver RM, Theodore A, Goldin J, Clements P, Chung J, Elashoff RM, Suh R, Smith EA, Furst DE, Tashkin DP, The Scleroderma Lupus Study Research Group.  Brochoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease.  Am J Respir Crit Care Med.  2007 Sep 27; [Epub ahead of print].
  9. Tashkin DP, Elashoff R, Clements PJ, Roth MD, Furst DE, Silver RM, Goldin J, Arriola E, Strange C, Bolster MB, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel D, Theodore A, Simms R, Wise R, Wigley F; Scleroderma Lung Study Research Group.  Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease.  Am J Respir Crit Care Med.  2007 Nov 15; 176(10):1026-34.
  10. Mulligan-Kehoe MJ, Drinane MC, Mollmark J, Casciola-Rosen L, Hummers LK, Hall A, Rosen A, Wigley FM, Simons M.  Antiangiogenic plasma activity in patients with systemic sclerosis.  Arthritis Rheum.  2007 Oct; 56(10):3448-58.
  11. Lam GK, Hummers LK, Woods A, Wigley F.  Efficacy and safety of Etanercept in the treatment of scleroderma-associated joint disease.  J Rheumatol.  2007 Jul; 34(7):1636-7.
  12. Walker UA, Tyndall A, Czirjak L, Denton CP, Bancel DF, Kowal-Bielecka O, Muller-Ladner U, Bocelli-Tyndall C, Matucci-Cerinic M; EULAR Scleroderma Trials.  Clinical risk assessment of organ manifestations in systemic sclerosis – a report from the EULAR Scleroderma Trials and Research (EUSTAR) group data base.  Ann Rheum Dis.  2007 Jun; 66(6):754-63.
  13. Khanna D, Yan X, Tashkin DP, Furst DE, Elashoff R, Roth MD, Silver R, Strange C, Bolster M, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel DE, Theodore A, Simms R, Wise R, Wigley F, White B, Steen V, Read C, Mayes M, Parsley E, Mubarak K, Connolly MK, Golden J, Olman M, Fessler B, Rothfield N, Metersky M, Clements PJ; Scleroderma Lung Study Group.  Impact of oral cyclophosphamide on health-related quality of life in patients with active scleroderma lung disease:  results from the scleroderma lung study.  Arthritis Rheum.  2007 May; 56(5):1676-84.
  14. Schachna L, Medsger TA, Dauber JH, Wigley FM, Braunstein NA, White B, Steen VD, Conte JV, Yang SC, McCurry KR, Borja MC, Plaskon DE, Orens JB, Gelber AC. Lung transplantation in scleroderma compared to idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum 2006 Dec; 54(12):3954-61.
  15. Roberts CGP, Hummers LK, Ravich WJ, Wigley FM, Hutchins GM. A case-controlled study of the pathology of oesophageal disease in systemic sclerosis (scleroderma). GUT 2006 Dec; 55(12):1697-703.
  16. Sherber NS, Wigley FM, Anhalt GJ. Bullous pemphigoid in a patient with systemic sclerosis (scleroderma). J Rheum. 2006 Oct; 33(10:2098.
  17. Khanna D, Furst DE, Hays RD, Park GS, Wong WK, Seibold JR, Mayes MD, White B, Wigley FM, Weisman M, Barr W, Moreland L, Medsger TA, Steen VD, Martin RW, Collier D, Weinstein A, Lally EV, Varga J, Weiner SR, Andres B, Abeles M, Clements PJ. Minimally important difference in diffuse systemic sclerosis: results from the D-Penicillamine Study. Ann Rheum Dis 2006 Oct; 65(10):1325-9.
  18. Edwards RR, Goble L, Kwan A, Kudel I, McGuire L, Heinberg L, Wigley FM, Haythornthwaite J. Catastrophizing, pain, and social adjustment in scleroderma: relationships with educational level. Clin J Pain. 2006 Sep; 22(7):639-46.
  19. Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L, Krishnan JA, Wigley FM, Hassoun PM. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum. 2006 Sep; 54(9):3043-50.
  20. Kamen DL, Wigley FM, Brown AN. Antineutrophil cytoplasmic antibody-positive crescentic glomerulonephritis in scleroderma—a different kind of renal crisis. J Rheumatol. 2006 Sep; 33(9):1886-8.
  21. Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, Arriola E, Silver R, Strange C, Bolster M, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel DE, Theodore A, Simms R, Wise R, Wigley FM, White B, Steen V, Read C, Mayes M, Parsley E, Mubarak K, Connolly K, Golden J, Olman M, Fessler B, Rothfield N, Metersky M, for the Scleroderma Lung Study Research Group. Cyclophosphamide versus placebo in scleroderma lung disease.  N Engl J Med 2006 Jun; 354(25)2655-66.
  22. Cope KA, Solga SF, Hummers LK, Wigley FM, Diehl AM, Risby TH. Abnormal exhaled ethane concentrations in scleroderma. Biomarkers 2006;11(1):70-84.
  23. Chang B, Schachna L, White B, Wigley FM, Wise RA. Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma. J Rheumatol 2006;33(2):269-74.
  24. Gelber AC, Pillemer SR, Baum BJ, Wigley FM, Hummers LK, Morris S, Rosen A, Casciola-Rosen LA. Distinct recognition of antibodies to centromere proteins in primary Sjogren's syndrome compared with limited scleroderma. Ann Rheum Dis 2006;65(8):1028-32.
  25. Wigley FM, Lima JA, Mayes M, McLain D, Chapin JL, Ward-Able C. Reply. Arthritis Rheum 2006;54(1):380-1.
  26. Boin F, Wigley FM, Schneck JP, Oelke M, Rosen A. Evaluatoin of topoisomerase-1-specific CD8+ T-Cell response in systemic sclerosis. Ann N Y Acad Sci 2005;1062:137-45.
  27. Liu X, Zhu S, Wang T, Hummers L, Wigley FM, Goldschmidt-Clermont PJ, Dong C. Paclitaxel modulates TGFbeta signaling in scleroderma skin grafts in immunodeficient mice. PLoS Med 2005;2(12):e354.
  28. Girgis RE, Mathai SC, Krishnan JA, Wigley FM, Hassoun PM. Long-term outcome of bosentan treatment in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with the scleroderma spectrum of diseases. J Heart Lung Transplant 2005;24(10):1626-31.
  29. Wigley FM, Lima JA, Mayes M, McLain D, Chapin JL, Ward-Able C. 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). Arthritis Rheum 2005;52(7):2125-32.
  30. Khanna D, Clements PJ, Furst DE, Chon Y, Elashoff R, Roth MD, Sterz MG,Chung J, FitzGerald JD, Seibold JR, Varga J, Theodore A, Wigley FM, Silver RM,Steen VD, Mayes MD, Connolly MK, Fessler BJ, Rothfield NF, Mubarak K, Molitor J, Tashkin DP; Scleroderma Lung Study Group. 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 Study. Arthritis Rheum.2005;52(2):592-600.
  31. Clements PJ, Seibold JR, Furst DE, Mayes M, White B, Wigley FM, Weisman MD, Barr W, Moreland L, Medsger TA Jr, Steen V, Martin RW, Collier D, Weinstein A, Lally E, Varga J, Weiner SR, Andrews B, Abeles M, Wong WK. High-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial: lessons learned. Semin Arthritis Rheum 2004;33(4):249-63.
  32. Wise RA, Wigley FM, White B, Leatherman G, Zhong J, Krasa H, Kambayashi J, Orlandi C, Czerwiec FS. 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 study. Arthritis Rheum 2004;50(12):3994-4001.

Book Chapters, Review Articles

  1. Henness S, Wigley FM.  Current drug therapy for scleroderma and secondary Raynaud’s phenomenon:  evidence-based review.  Curr Opin Rheumatol.  2007 Nov; 19(6):611-8.
  2. Sherber NS, Wigley FM, Scher RK.  Autoimmune disorders:  nail signs and therapeutic approaches.  Dermatol Therapy.  2007 Jan-Feb; 20(1):17-30.
  3. Boin F, Wigley FM. Understanding, assessing and treating Raynaud's phenomenon. Curr Opin Rheumatol 2005; 17(6):752-60.
  4. Grader-Beck T, Wigley FM. Raynaud's phenomenon in mixed connective tissue disease. Rheum Dis Clin North Am 2005;31(3):465-81, vi. Review.
  5. Varga J, Wigley F. Systemic Sclerosis. In: Rich, Shearer, Fleisher, Schroeder, Weyand, Frew, editors. Clinical Immunology (3rd Ed). London: Elsevier.
  6. Huston KK, Stone JH, Wigley FM. Digital ischemia and Raynaud's phenomenon, In: Ball GV, Bridges Jr SL, editors. Vasculitis. London: Oxford University Press.
  7. Wigley FM. Scleroderma (Systemic Sclerosis). In: Goldman L, Ausiello D, editors. Cecil Textbook of Medicine (22nd Ed). Philadelphia: Saunders.
  8. Boin F, Wigley FW. Systemic Sclerosis. In: Bartlett SJ, Bingham C, Marcic M, editors. Clinical Care in the Rheumatic Diseases (3rd Ed). ACR.
  9. Sule S. Pediatric Rheumatic Diseases. In: Bartlett SJ, Bingham C, Marcic M, editors. Clinical Care in the Rheumatic Diseases (3rd Ed). ACR.
  10. Hummers LK, Wigley FM. Scleroderma. In: Bartlett SJ, Bingham C, Marcic M, editors. Clinical Care in the Rheumatic Diseases (3rd Ed). ACR.

Submitted Manuscripts

  1. Blum M, Hummers LK, Wigley F. Scleromyxedema: A case series describing clinical features and long-term management with IVIG.
  2. Hummers LK, Dugowson CE, Dechow FJ, Wise RA, Wener M, Bartolucci A, Wigley F. A multi-center, blinded, randomized, placebo-controlled study of MQX-503, a topical gel formulation of nitroglycerin, in patients with Raynaud’s phenomenon.
  3. Hummers LK, Hall A, Wigley FM, Simons M. Abnormalities in the regulators of angiogenesis in patients with scleroderma.
  4. Gelber AC, Hummers LK, Wang NY, Wigley FM. Race and independent association with disease subtype, organ system involvement and serologic status in scleroderma.
  5. Sule SD, Rosen A, Wigley FM. Characterization of transforming growth factor (TGF)- β secretion from monocytes in systemic sclerosis.
  6. Sule SD, Hummers L, Wigley FM. A longitudinal study of estimated right ventricular systolic pressure (RVSP) by Doppler Echocardiography (ECHO) as a predictor of mortality in systemic sclerosis (scleroderma).
  7. Wigley FM, Wise RA, Czerwiec FS and the REWARM Study. A Multi-center, Blinded, Randomized, Placebo-controlled Study of OPC-28326, a selective alpha-adrenergic antagonist, in Patients with Raynaud’s Phenomenon.
  8. White B, Choi J, Luzina IG, Atamas SP, Vaishnaw A, Wigley FM, Wise R. Selective T cell reduction in scleroderma lung disease, using lfa3/igg1 human fusion protein alefacept.
  9. Huston KK, Casciola-Rosen L, Hummers L, Wigley F, Wise R, Rosen A. Fibrilin-1 is associated with alveolitis in scleroderma.

Abstracts (Presented at the American College of Rheumatology Annual Scientific Meeting, 2007)

  1. Sule SD, Hummers L, Shapiro L, Fiorentino D, Chung L, Baron M, Shanahan J, Hsu V, Rothfield N, Steen V, Martin R, Dugowson C, Smith E, Mayes M, Simms R, Pope J, Kahaleh B, Csuka ME, Gruber B, Collier D, Sweiss N, Klaff L, Dechow FJ, Gregory J, Wigley FM.  Safety and Tolerability of MQX-503, a Novel Investigational Topical Formulation of Nitroglycerin for the Treatment of Raynaud’s Phenomenon.
  2. Shah AA, Hummers LK, Schwartz P, Le B, Dellinger W, Stott D, Wise RA, Wigley FM.   Ambulatory Raynaud’s Monitor:  A Novel Measurement Devise to Assess Raynaud’s Phenomenon in an Ambulatory Setting.
  3. Chung L, Baron M, Collier D, Csuka ME, Fiorentino D, Gruber B, Hsu V, Kahaleh B, Martin R, Mayes M, Pope J, Rothfield N, Shanahan J, Shapiro L, Simms R, Smith E, Steen V, Sule S, Sweiss N, Matthews E, Gilbert A, Dechow FJ, Wigley FM.   A Multi-Center Placebo-Controlled “In-Life” Study of MQX-503 in Patients with Raynaud’s Phenomenon.
  4. Chung L, Shapiro L, Fiorentino D, Baron M, Shanahan J, Sule S, Hsu V, Rothfield N, Steen V, Martin R, Smith E, Mayes M, Simms R, Pope J, Kahaleh B, Csuka ME, Gruber B, Collier D, Sweiss N, Matthews E, Gilbert A, Dechow FJ, Gregory J, Wigley FM.  MQX-503, a Novel Topical Nitroglycerin Formulation, Improves Severity of Raynaud’s phenomenon in a Heterogeneous Patient Population.
  5. Kowal-Bielecka O, Landewe R, Avouac J, Chwiesko S, Miniati I, Czirjak L, Clements P, Denton C, Farge D, Fligelstone K, Foldvari I, Furst D, Muller-Ladner U, Seibold J, Silver R, Takehara K, Toth BG, Tyndall A, Valentini G, van den Hoogen F, Wigley F, Zulian F, Matucci-Cerinic M, on behalf of EUSTAR centers.  EULAR/EUSTAR Recommendations for the Treatment of Systemic Sclerosis (SSc).
  6. Denton CP, Fiorentino D, Herrick A, Sule S, Rothfield N, Belch J, Shapiro L, Scheja A, Shanahan J, Steen V, Emery P, Chung L, Gregory J, Batson E, Dooley T, Gilbert A, Dechow F, Wigley FM.  A Multi-center Crossover Study of MQX-503, a Topical Formulation of Nitroglycerin, in Patients with Raynaud’s Phenomenon.

Abstracts (Presented at the American College of Rheumatology Annual Scientific Meeting, 2006)

  1. Christopher V. Tehlirian et al.: High dose cyclophosphamide without stem cell transplantation in systemic sclerosis.
  2. Shikha Mitto et al.: Long term pulmonary function and survival outcomes of scleroderma patients treated with cyclophosphamide (CYC) for active interstitial lung disease (ILD).
  3. Laura Hummers et al.: A multi-center, blinded, randomized, placebo-controlled study of MQX-503, a topical gel formulation of nitroglycerin, in patients with Raynaud’s phenomenon.
  4. Francesco Boin et al.: Diverging T cell polarization identifies distinct clinical phenotypes in systemic sclerosis.
  5. Sangeetta D. Sule et al.: Characterization of transforming growth factor (TGF)- β secretion from monocytes in systemic sclerosis.

Abstracts (Presented at the American College of Rheumatology Annual Scientific Meeting, 2005)

  1. Gordon K. Lam, Adrianne Woods, Laura K. Hummers, Fredrick M. Wigley. Efficacy and safety of etanercept in scleroderma (SSc) ioint disease.
  2. Allan C. Gelber, Laura K. Hummers, Nae Y. Wang, Fredrick M. Wigley.  Race and independent association with disease subtype, organ system involvement and serologic status in scleroderma.
  3. Kwas Huston, Livia Casciola-Rosen, Laura Hummers, Fred Wigley, Robert Wise, Antony Rosen. Fibrillin-1 predicts alveolitis in patients with scleroderma.
  4. Keary A. Cope, Steven F. Solga, Laura K. Hummers, Fredrick M. Wigley, Anna Mae Diehl, Terence H. Risby. Abnormal breath ethane concentrations in patients with systemic sclerosis.
  5. Laura K. Hummers, Fredrick M. Wigley. Scleromyxedema: a case series describing clinical features and long-term management with IVIG.