Calcinosis, i.e. crystal-like nodules are troublesome complication of systemic sclerosis, an autoimmune disease. Pyrophosphate inhibits its formation is laborytory. We would like to test if orally administered pyrophosphate prevents calcinosis formation.
Calcinosis, the formation of hydroxyapatite subcutaneous nodules, often complicates systemic sclerosis. There is no standard treatment for it. Based upon our preclinical experienc, we would like to test it orally administered pyrophosphate inhibits calciosis.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
SINGLE
Enrollment
60
50 mg/kg bwt once daily in gelatine capsules.
Glucose in gelatine capsules.
Change in the size of calcinosis nodues.
Volumetric assessment with low energy CT
Time frame: 52 weeks+ 16 weeks
Change of the severity of sympthoms caused by calcinosis
Visual analogue scale (0-100)
Time frame: 52 weeks+ 16 weeks
Change of the activity of calcinosis
Visual analogue scale (0-100)
Time frame: 52 weeks+ 16 weeks
Change of the size of calcinosis by ultrasound
Volumetric assessment by ultrasound
Time frame: 52 weeks+ 16 weeks
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