Irish Raynaud's & Scleroderma Society

Research Updates

ISN Profile Update—Dr. James R. Seibold

In February 2010, Dr. James R. Seibold, who is the Chair of our ISN Medical Advisory Board, became Professor and Chief of the Division of Rheumatology at the University of Connecticut Health Center. He had been on the faculty of UMDNJ from 1980-2004 where he had served as Chief of the Division of Rheumatology, Director of the Clinical Research Center and as the W.H. Conzen Chair of Clinical Pharmacology.  In July 2004, he became Professor and Director of the University of Michigan Scleroderma Program, and in February 2010 he joined UCHC. For appointment inquiries with Dr. Seibold, please call 1-860-679-3605.  Progress through Collaboration in Scleroderma by James R. Seibold, M.D., ISN Medical Advisory Board Chair Dr. James R. Seibold James R. Seibold, M.D. Chair, ISN Medical Advisory Board

The Pace of Research in Ssc

The pace of research in scleroderma is accelerating and the quality of data is ever increasing. As a 30-year veteran of scleroderma research, I have watched in awe as the field advances and have tried to “diagnose” the reasons. I think it’s the spirit of collaboration and it all traces back to the U.S. study of D-penicillamine. Uncontrolled case series from the 1970s and early ‘80s had suggested this agent had benefits, but reliable data from a controlled trial were lacking.  Several attempts to get funding from the National Institute of Health had been unsuccessful largely because the evidence was so weak. A group of committed U.S. clinical researchers decided to do a study anyway, and organized a trial with minimal funding but maximal passion.

EUSTAR and EULAR

While the trial was quite negative, it did demonstrate that we could all pull together in an effective fashion. The success led to formation of the Scleroderma Clinical Trials Consortium and later to EUSTAR, the EULAR Scleroderma Trials and Research Group. International collaboration grew rapidly, nourished by the understanding by study sponsors that trials were needed and that they could be done with quality and efficacy.

Meanwhile, promising young academic talent is being drawn to the field which has further benefited from the large number of successful drug development projects in pulmonary hypertension. One need only browse http://clinicaltrials.gov under scleroderma to assess the breadth and quality of modern scleroderma research. There are currently 116 separate studies. We move forward as a group with agents that modify the overall disease, treatments for the lung including pulmonary hypertension and interstitial disease, Raynaud phenomenon and digital ulcers and agents aimed at enhancing quality of life. A journey of 1,000 miles begins with a first step. Perhaps it was the D-penicillamine study, perhaps not but all should be aware that healthy collegial collaborative projects continue to proliferate. The research community has justified optimism.

 

Information for Medical Professionals

Very Early Diagnosis of Systemic Sclerosis (VEDOSS)

Scleroderma (Systemic Sclerosis) can be treated early only if diagnosed early. The VEDOSS programme, led by EUSTAR, calls for all GPs to refer people with two early symptoms to a rheumatologist for further investigation. While Raynaud’s (lack of circulation in the fingers and toes when chilled, leading to white, numb digits) is a common, mild condition, it may prove to be the precursor of systemic scleroderma, and it is one of the symptoms that call for surveillance. A second symptom is hard puffy fingers. Where both symptoms are present, an ANA blood test should be conducted. Examination by a rheumatologist familiar with systemic sclerosis is the next step. Early diagnosis of scleroderma saves lives and leads to control of the disease.

“The challenge of early systemic sclerosis for the EULAR Scleroderma Trial and Research group (EULAR) community.  It is time to cut the Gordian knot and develop a prevention or rescue strategy”  This is the title of an article in the Annals of Rheumatic Diseases 2009.  Click here to read it, or download it from the link at the bottom of the page.

Scleroderma Awareness Day

On June 29 2009, in addition to launching VEDOSS, we celebrated the life and death of Paul Klee and all those who struggle with scleroderma. We continue to campaign for a world in which equal rights, treatments, and care are offered to people with scleroderma, and in which such rare diseases are not forgotten, but afforded the consideration and attention of other more common diseases.

Information Leaflets

If you need information leaflets to hand out to patients you can order them by phone (01 2020184) or email.

Booklet for GPs

We have prepared a booklet for GPs with information on diagnosis and treatment of Raynaud’s amd scleroderma.  Click on GPs booklet link below - see under DOCUMENT DOWNLOADS.  If you would like a hard copy of this book please email us with your name and postal address and we will send it to you.

Cost of Management of Ssc

The cost of the management of systemic sclerosis (SSc) increases enormously with the increase of disease duration and the damage that the disease causes to cutaneous, cardiovascular, pulmonary, and GI systems.  Indeed, the goal of treatment is now to block disease evolution, maintain quality of life, and prevent organ damage. Therefore, early diagnosis is mandatory to achieve these tasks. Unfortunately, late diagnosis of SSc allows, in the majority of cases, development and spread of organ damage leading eventually to functional failure.

Early Treatment of Ssc

Early SSc has recently become the main target of care and treatment. Recognition of the disease through its early symptoms is therefore of paramount importance in order to reach an early diagnosis and to fight the disease appropriately. Raynaud’s (RP) is the earliest sign of SSc suggesting an underlying endothelial and microvascular dysfunction. RP can precede disease onset and development by several months to years. Therefore, a program designed to increase awareness and further investigation of RP by primary care physicians is essential. The identification of early microvascular changes is today possible through the recognition of nailfold capillaroscopic patterns. In addition, autoantibodies (ANA, anti centromere, anti topoisomerase I) are very helpful hematological signs, as they are already present in the earliest pre-sclerodermatous phase.
Thus, in presence of RP, a careful clinical assessment together with the presence of an altered capillaroscopic pattern and positive antibody results may allow for a diagnosis of very early SSc.

Click here to read “VEDOSS unrolling” or download this document from the link at the bottom of the page.

The objectives of the VEDOSS programme

• To track SSc through identification of patients with Raynaud’s.

• To develop regional networks to disseminate and implement the awareness of systemic sclerosis and its early diagnosis and classification in Europe.

• To develop clinics devoted to Raynaud’s in order to detect SSc early.

• To develop information systems to facilitate and support the early diagnosis and treatment of the disease.

• To develop postgraduate education programmes to support the above objectives