View clinical trials related to Nephrogenic Systemic Fibrosis.
Filter by:Phase 4, open-label, two-year, prospective, multi-center, follow-up study conducted at up 15 sites in USA. Approximately 1,000 patients with moderate-to-severe CKD will be enrolled and followed for up to 24 months.
The primary objective of this study is to determine any causative or associated factors for the development of Nephrogenic Systemic Fibrosis (NSF), Nephrogenic Fibrosing Dermopathy (NFD), or related diagnosis. Our primary focus will be on the previous administration of gadolinium to these patients, but we will also look at other postulated causes and risk factors. The secondary objective of this study is to assess tissue gadolinium (Gd) levels in five groups of subjects: - Those affected by NSF. - Those with normal kidney function who have undergone a medical imaging procedure using Gd-based contrast agent (GBCA) in the 2 years prior to a skin biopsy. - Those with normal kidney function who have never been exposed to GBCA and have had a skin biopsy. - Those on dialysis or with eGFR ≤ 30 ml/min/1.73 m2 who have had a medical imaging procedure using GBCA in the 2 years prior to skin biopsy. - Those on dialysis or with eGFR ≤ 30 ml/min/1.73 m2 who have never been exposed to GBCA and have had skin biopsy. We hypothesize that there is a correlation between the administration of Gd-containing agents usually associated with MRI procedures and the development of NSF in those with renal failure and some other predisposing condition. We also hypothesize that tissue Gd levels in those with NSF will be higher than in those who have been exposed to GBCA but do not have NSF. Of the two groups without NSF but with exposure to GBCA, we hypothesize that those with kidney dysfunction will have higher tissue Gd levels than those with normal kidney function. We hypothesize that in the two groups of subjects without exposure to GBCA, there will be no detectable levels of Gd, regardless of kidney function status.
The investigators will study the effect of imatinib mesylate (Glivec) in treatment of moderate to severe nephrogenic systemic fibrosis (NSF). So far there is no evidence of adequately effective treatment options of NSF. Various treatments have been tried to stop the progressing disease. Corticosteroids, which suppress the early inflammatory stage of the disease, fail to halt disease progression. Other immunosuppressive agents, photopheresis, and kidney transplantations are reported to be partly beneficial to the patients. It has not been possible to confirm these findings in further studies because in photopheresis, and kidney transplantation, such effects are generally unreproducible.
The Primary Aim of this study is to validate a questionnaire as a screening tool to identify subjects with symptoms suggestive of nephrogenic systemic fibrosis (NSF). The investigators believe that there will be difference between subjects with NSF and other skin conditions and normal skin.