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| Name | Class |
|---|---|
| Research Centre for Natural Sciences | OTHER |
| University of Debrecen Dept. of Rheumatology | UNKNOWN |
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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Pyrophosphate | Experimental | Disodium pyrophosphate |
|
| Placebo | Placebo Comparator | Glucose |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Pyrophosphate | Dietary Supplement | 50 mg/kg bwt once daily in gelatine capsules. |
| |
| Measure | Description | Time Frame |
|---|---|---|
| Change in the size of calcinosis nodues. | Volumetric assessment with low energy CT | 52 weeks+ 16 weeks |
| Measure | Description | Time Frame |
|---|---|---|
| Change of the severity of sympthoms caused by calcinosis | Visual analogue scale (0-100) | 52 weeks+ 16 weeks |
| Change of the activity of calcinosis | Visual analogue scale (0-100) |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| László Kovács, MD, PhD | Contact | +36309081611 | kovacs.laszlo@med.u-szeged.hu | |
| Márta Bocskai, MD | Contact | +3662341520 | bocskai.marta@med.u-szeged.hu |
| Name | Affiliation | Role |
|---|---|---|
| László Kovács, MD, Ph.D. | Szeged University | Principal Investigator |
| András Váradi, PhD, DSc | Department of Enzymology, Research Centre for Natural Sciences | Study Director |
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| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 34152415 | Background | Hsu VM, Kozak E, Li Q, Bocskai M, Schlesinger N, Rosenthal A, McClure ST, Kovacs L, Balint L, Szamosi S, Szucs G, Carns M, Aren K, Goldberg I, Varadi A, Varga J. Inorganic pyrophosphate is reduced in patients with systemic sclerosis. Rheumatology (Oxford). 2022 Mar 2;61(3):1158-1165. doi: 10.1093/rheumatology/keab508. | |
| 28701330 |
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| ID | Term |
|---|---|
| D012595 | Scleroderma, Systemic |
| D045743 | Scleroderma, Diffuse |
| D002114 | Calcinosis |
| ID | Term |
|---|---|
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D012871 | Skin Diseases |
| D002128 | Calcium Metabolism Disorders |
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| ID | Term |
|---|---|
| C107241 | diphosphoric acid |
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| Placebo |
| Dietary Supplement |
Glucose in gelatine capsules. |
|
| 52 weeks+ 16 weeks |
| Change of the size of calcinosis by ultrasound | Volumetric assessment by ultrasound | 52 weeks+ 16 weeks |
| Dedinszki D, Szeri F, Kozak E, Pomozi V, Tokesi N, Mezei TR, Merczel K, Letavernier E, Tang E, Le Saux O, Aranyi T, van de Wetering K, Varadi A. Oral administration of pyrophosphate inhibits connective tissue calcification. EMBO Mol Med. 2017 Nov;9(11):1463-1470. doi: 10.15252/emmm.201707532. |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |