Scleroderma

The Scleroderma (Systemic Sclerosis) Center at The Boston Medical Center (Boston University School of Medicine) is a collaboration of the Pulmonary and Rheumatology Departments. The Center is recognized Internationally as a leader in scleroderma, and provides for the diagnosis and treatment of patients with Systemic Sclerosis. The Center is closely linked to National Institute of Health and Industry sponsored clinical, translational and basic research, including the Scleroderma Lung Study (SLS), a collaboration of 14 clinical scleroderma centers in the United States. Our Mission is to provide patients with systemic sclerosis state-of-the-art patient care and the opportunity to participate in advanced therapeutic research which both improves patient quality of life and provides for a basis for understanding the pathogenesis of the disease.

Systemic sclerosis (SSc) is classified as a collagen vascular disease, but its most catastrophic involvement is in the lungs. Eighty percent of patients with SSc develop interstitial lung disease at some point during their life with mortality from this accounting for 25-40 % of all deaths. A smaller percentage of patients develop pulmonary hypertension. Recognition of the severity of lung involvement in SSc led to the development of The Scleroderma Center at The Boston University School of Medicine, which is comprised of members from the Rheumatology and Pulmonary Departments. The Departments work in close collaboration both to provide patient care and to recruit subjects for clinical and basic science research.

Pulmonary Division involvement in the Scleroderma Center is directed by Arthur C. Theodore, Associate Professor of Medicine at Boston University School of Medicine who has an international reputation in the diagnosis, treatment, and investigation of interstitial lung disease in Systemic Sclerosis and is a member of the American Association for the Advancement of Science.

Patients with Systemic Sclerosis are referred to The Scleroderma Center from within Boston Medical Center, throughout New England, the Continental United States and Internationally. Some patients come only for an initial evaluation, second opinion or establishment of the diagnosis of lung disease while others may be followed for years. Approximately 50-100 new referrals per year are seen at The Scleroderma Center which provides patient care based on state-of-the-art clinical practice and translational/clinical research with extensive Research and Clinical programs in Molecular and Cell Biology, Immunology, Biochemistry, and Epi-demiology. All clinical care is provided at Boston Medical Center, where a full range of invasive and non-invasive Medical, Surgical and Radiologic subspecialists are available. Research is supported by grants from the NIH and private donations from pharmaceutical companies and organiz-ations committed to finding the cause and treating patients with systemic sclerosis.

The Scleroderma Center has been at the forefront in the treatment of alveolitis with intravenous cyclophosphamide. Initially pioneered by the late Dr. Joseph H. Korn, Chief of Rheumatology, the Center has experience in the treatment of several hundred patients with SSc alveolitis. The center provides for a full range of diagnostic and therapeutic capabilities which include:

  • Diagnosis and Initial Assessment of patients with Systemic Sclerosis including high resolution chest CT scanning, pulmonary function testing, bronchoalveolar lavage and pulmonary artery catheterization
  • Secondary and Tertiary Referrals from the Western Hemisphere for management of complex cases of systemic sclerosis
  • Ongoing treatment for multi-system Systemic Sclerosis, including pulmonary interstitial, pulmonary vascular, gastrointestinal, skin, cardiac and renal involvement
  • Use of immunosuppressive, receptor antagonist and transplantation (e.g. cyclphosphamide, azathioprine, Bosentan, and bone marrow transplantation) for initial and maintenance treatment
  • Participation in clinical trials and on-going Investigator-initiated and Industry-sponsored research with the goal of establishing efficacy and advancing knowledge about the pathogenesis and treatment of Systemic Sclerosis
  • Innovative therapies for recurrent and/or refractory disease

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