Prospective Registry of Early Systemic Sclerosis (PRESS)- Phenotypic, Serologic, and Biomarker/Genetic Characteristics of Early Diffuse Cutaneous Scleroderma

Institutional Review Board, Hospital for Special Surgery

October 06, 2016

The safety of study participants is our top priority. The trial is approved and periodically reviewed by an Institutional Review Board (IRB), which includes doctors, administrators, ethicists, and members of the general public. The safety of clinical trials is reviewed by the U.S. Food and Drug Administration.

Before enrolling in a clinical trial, the investigator will explain the purpose of the trial, its expected benefits, any possible risks or side effects, and what your role will be. This is the time to ask questions! If you want to join the trial, you must sign the informed consent documents. You can leave a clinical trial at any time without penalty.

For further information, see Understanding Clinical Trials.

Principal Investigator

Jessica K. Gordon, MD

Co-Investigators

Robert F. Spiera, MD
Horatio Wildman, MD 
Annel Fernandez
Emily Bakaj
Alexandra Morquette

Summary

Systemic sclerosis is an autoimmune condition with an unknown cause. Diffuse cutaneous Systemic Sclerosis (DcSSc) is the most severe subtype of this disease and treatment options are emphasized for patients in the early stages of DcSSc. Yet there are many questions about the natural history of disease in people with DcSSc. The PRESS study is a natural history study that collects data and biological material such as optional blood samples and tissue biopsies.  This study is being done to try to find out the causes of scleroderma and how baseline tests (such as radiographs, clinical examination, quality of life questionnaires) predict future outcome. This multi-center registry is open to enrollment. The enrollment period is ongoing, as is the duration of follow-up. Follow up visits occur every 6 months. ECHO, PFT, HRCT test are performed yearly as part of clinical care. Currently there are 26 patients enrolled in the registry at the Hospital for Special Surgery.

Inclusion/Exclusion Criteria

Inclusion Criteria:

• Patients 18 years of age and older.
• Patients who meet ACR criteria for Systemic Sclerosis with diffuse subtype by LeRoy criteria.

AND

• Disease duration less than 2 years based on time from initial symptom referable to scleroderma.

OR

• Patients with swollen hands or sclerodactly for less than or equal to 2 years, which is associated with one or more of the following:
•  anti-SCL-70 or anti-RNA polymerase III antibodies; and/or
•  Skin thickening involving upper arms, thighs, or torso; and/or
• Presence of tendon friction rubs

Exclusion Criteria:

• Inability to render informed consent in accordance with institutional guidelines.
•  Anti-centromere antibody positive, or limited Scleroderma positive.
• Diagnostic criteria for Systemic Sclerosis not met.
•  Have had Scleroderma for more than 2 years.
•  Patients under 18 years of age.
• Pregnant women (however, women already enrolled in the study who become pregnant will not be excluded)
•  Patients who do not read or write English proficiently.

Contact Information

Annel Fernandez
Research Coordinator
212.774.2123
fernandeza@hss.edu