Bosentan Treatment of Digital Ulcers Related to Systemic Sclerosis
Condition: Systematic Sclerosis; Digital Ulcer; Scleroderma
MeSH terms: Skin Ulcer; Sclerosis; Ulcer
Study type: Observational [Patient Registry]

Summary: 
      GRegistry is a significant collection of data and information on the clinical practice of
      systemic scleroderma finger ulcers.

      The GRegistry study is a nationwide (Greece) prospective study recording, which includes
      consecutive patients with SC-DU diagnosis in clinical management departments of different
      hospitals, selected in such a way as to reflect a representation of the country.

      The use of Bosentan in the treatment of patients suffering from systemic sclerosis with
      digital ulcer disease is supported by data from large, placebo-controlled, randomized
      studies. The beneficial effect of Bosentan in digital ulcers, a vascular event of systemic
      sclerosis consistent with its beneficial effect on pulmonary arterial hypertension associated
      with connective tissue disease and its beneficial effect on vascular remodeling.
    

Detailed description: 
      The existence and use of Patient Registries, represents one of the foundations of the
      progressing medical science, both for studying the natural history of diseases as well as to
      critically evaluate the therapeutic methods, the collection and analysis of the data from a
      large number of hospital units, is essential. Furthermore, the assessment of the
      effectiveness and adequacy of health services should be based on long-term monitoring of
      large groups of patients, and the assessment of the efficient functioning of the individual
      units, is only possible only with the availability of information for the patients of these
      units.

      Moreover, the record of health services distribution data is a prerequisite for the design of
      their future development. The above led to growth of many national and international patient
      logs, diseases and specific treatments, the operation of which has contributed to the
      advancement of medical science.

      Scleroderma or Systemic Sclerosis (SC) is a chronic autoimmune disease of the connective
      tissue with an incidence of 1: 10000- 1: 30000. In Greece it is estimated that there are
      approximately 2,500 patients. The disease usually affects women aged 40-60 years, and the
      incidence is four women to one man. The pathogenesis of the disease is unknown, while the
      main causes are considered genetic and environmental factors.

      Two of the most critical complications of the disease, are the Pulmonary Arterial
      Hypertension, which occurs in patients with SC approximately 8-12% of patients, being the
      main cause of death, and digital ulcers that occur in 30-60 % of patients and are painful
      sores on the fingers. Digital ulcers, i.e. painful sores on the fingers, as a consequence of
      the limited blood supply of vessels are seen in approximately 30-60 % of patients with
      scleroderma. The digital ulcers adversely affect the quality of life of patients, even the
      simple daily activities and can lead to serious complications, such as amputation. The
      symptoms of scleroderma can vary between patients and may not be typical of this disease, at
      least in the early stages, making it difficult to diagnose. Two of the early symptoms are
      disturbance of blood supply to the extremities by a color change of the skin of the fingers,
      alternately pallor-cyanation-redness (Raynaud phenomenon) and rigid edematous fingers.
    

Intervention name(s): Bosentan group
Intervention type(s): Drug
Intervention MeSH terms: Bosentan
Observational model: Other
Time perspective: Prospective
Sampling method: Non-Probability Sample
Study population: 
        Patients with Digital Ulcers caused by Systematic Scleroderma according to ACR 2013
        criteria