Scleroderma

Goal of Program:

The Scleroderma Program at Thomas Jefferson University is a collaboration of the Scleroderma Center of Thomas Jefferson University, and the Divisions of Rheumatology and Pulmonary of the Department of Medicine. Our goal is to provide state-of-the-art care that improves the quality of life for patients with scleroderma.

Who We Are:

  • Sandra Weibel, MD, Assistant Professor of Medicine, specializes in treatment of interstitial lung disease and has been a leader in treatment of scleroderma lung disease at Thomas Jefferson University for over 15 years.
  • Ross Summer, MD, Associate Professor of Medicine, physician-scientist who specializes in treatment of interstitial lung disease and engages in laboratory investigation to better understand the molecular mechanisms driving the development of lung fibrosis.
  • Michael Scharf, MD. Associate Professor of Medicine, specializes in treatment of pulmonary hypertension and has extensive experience with treatment scleroderma-related PAH.
  • Sergio Jimenez, MD, Professor of Medicine, Director of the Scleroderma Center has been committed to the study of Scleroderma and to the treatment of patients with Scleroderma for over 30 years.
  • Fabian A. Mendoza-Ballesteros, MD, Assistant Professor of Medicine, Department of Rheumatology, specializes in treatment of all aspects of scleroderma.
  • Jennifer Sloan, MD - Assistant Professor of Medicine, Department of Rheumatology, specializes in treatment of all aspects of scleroderma.
  • Joel Rosenbloom, PhD- Professor of Medicine, world-renown leader in area of fibrosis research.

Background:

Scleroderma is a connective tissue disease that leads to hardening and tightening of the skin and fibrosis (scarring) of numerous internal organs. While almost any organ is affected by this condition lung manifestations including interstitial lung disease and pulmonary hypertension are often its most serious organ complications.  In fact, recent estimates indicate that eighty percent of patients with scleroderma have some lung involvement and 25-40 % of all deaths are attributable to pulmonary complications. At Thomas Jefferson University we are dedicated to changing these statistics and improving the lives of patients with scleroderma. The Scleroderma Center at Thomas Jefferson is led by a team of clinicians and researchers that work closely together to provide patients with latest and most proven therapies for this condition.

Clinical Activities:

Thomas Jefferson has been a leader in the field of scleroderma research and clinical care for over 3 decades. In the pulmonary section, we provide a full range of diagnostic and therapeutic capabilities and we do so with a patient-centered focus. Patients are referred to our section for any of the following reasons:

  • Diagnosis and Initial Assessment of scleroderma lung disease.
  • Secondary and Tertiary Referral from complicated scleroderma lung disease
  • Full complement of diagnostic testing including high resolution chest CT scanning, pulmonary function studies, bronchoalveolar lavage and pulmonary artery catheterization.
  • State-of-the-art treatment for interstitial lung disease and pulmonary vascular hypertension.
  • Management and monitoring of drug efficacy and toxicity (immunosuppresants and pulmonary hypertension medications).
  • Participation in clinical trials and on-going Investigator-initiated and Industry-sponsored research
  • Innovative therapies for refractory disease

Research Activities:

Research on scleroderma is focused on developing a better understanding of the pathogenesis of scleroderma in order to develop more disease- and patient-specific treatments.

Publications: (selected publications)

  1. Haley, S., Shah, D., Romero, F., Summer, R. “Scleroderma Related Lung Disease: Is there a pathogenic role for adipokines?” Curr Rheumatol Rep. 2013 Dec;15(12):381.
  2. Rosenbloom J, Mendoza FA, Jimenez SA. Strategies for anti-fibrotic therapies. Biochim Biophys Acta. 2013 Jul;1832(7):1088-103
  3. Fang F, Liu L, Yang Y, Tamaki Z, Wei J, Marangoni RG, Bhattacharyya S, Summer RS, Ye B, Varga J. The adipokine adiponectin has potent anti-fibrotic effects mediated via adenosine monophosphate-activated protein kinase: novel target for fibrosis therapy. Arthritis Res Ther. 2012 Oct 23;14(5):R229
  4. Mendoza FA, Nagle SJ, Lee JB, Jimenez SA.A prospective observational study of mycophenolate mofetil treatment in progressive diffuse cutaneous systemic sclerosis of recent onset. J Rheumatol. 2012 Jun;39(6):1241-7.
  5. Mendoza FA, Keyes-Elstein LL, Jimenez SA. Systemic sclerosis disease modification clinical trials design: quo vadis?. Arthritis Care Res (Hoboken). 2012 Jul;64(7):945-54.
  6. Rudnicka L, Varga J, Christiano AM, Iozzo RV, Jimenez SA, Uitto J. Elevated expression of type VII collagen in the skin of patients with systemic sclerosis. Regulation by transforming growth factor-beta. J Clin Invest. 1994 Apr;93(4):1709-15

Useful links on the Web:

Scheduling an Appointment:

Call 1 800-JEFFNOW and ask the operator to schedule a new patient visit with Drs. Summer, Scharf or Dr. Weibel for an evaluation of ”scleroderma-related lung disease”.  Alternatively, one can either call our pulmonary offices directly by dialing  (215) 955-5161, option 2 or by e-mailing one of our physicians at Ross.Summer@jefferson.edu or Sandra.Wiebel@Jefferson.edu.

How to Get Here