Nephrogenic Systemic Fibrosis Clinical Trial
Official title:
An Open Label Clinical Trial of Imatinib Mesylate(Glivec)in Patients With Moderate to Severe Nephrogenic Systemic Fibrosis
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.
NSF is a relatively newly defined fibrosing disease not described before 1997 where the
Gadolinium-based contrast agents (GBCAs) were introduced in patients with kidney disease.
The association between NSF and GBCA has in many studies shown to be very strong. Until
recently, radiologists believed that commercially available GBCAs were safe to use whether
the renal function was normal or not. Since the 1980s, >200 million patients have been given
these agents. Lately, the occurrence of NSF, a relatively new chronic disorder, has given
serious speculations about the safety of these drugs and has questioned their future use.
First identified in 1997, but not described until 2000, NSF has been reported only in
patients with acute or chronic severe renal insufficiency (with a glomerular filtration rate
<30 ml/min/1.73 m2).
Fibrosis in the subcutis means that the skin hardens and loses flexibility. Hard dermal
plaque changes often appear on legs, arms and abdomen together with dyspigmentation. As the
lesions involve the deep part of the subcutis the muscles are often affected. Involvement of
the joints leads to contractures and narrowing of movement. Patients with massive affection
of the joints often end up with a zimmer frame or in a wheelchair. The connecting tissue in
the inner vital organs may also be affected and NSF can accelerate the death of the patient.
The retained gadolinium in lesions of NSF can be found years after administration.
Interestingly, a case report suggests beneficial effects of imatinib mesylate in two
patients. Two other independent case reports also show promising results.
Imatinib mesylate inhibits several tyrosine kinases involved in the fibrotic reaction, which
is one of the main pathogenetic components of NSF.
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Allocation: Non-Randomized, Endpoint Classification: Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment
Status | Clinical Trial | Phase | |
---|---|---|---|
Completed |
NCT01135316 -
Prospective Evaluation of the Incidence of NSF in Patients With Kidney Disease Undergoing MR
|
N/A | |
Active, not recruiting |
NCT00677092 -
Pilot Study of Imatinib Mesylate to Treat Nephrogenic Systemic Fibrosis
|
Phase 2 | |
Completed |
NCT00869479 -
Validation of a Questionnaire to Identify Signs and Symptoms of Nephrogenic Systemic Fibrosis
|
N/A | |
Enrolling by invitation |
NCT01359345 -
Nephrogenic Systemic Fibrosis With Gadollinum
|
Phase 2/Phase 3 | |
Withdrawn |
NCT00811863 -
Evaluation of Nephrogenic Systemic Fibrosis (NSF) in Patients With Chronic Kidney Disease Following OptiMARK Injection
|
N/A | |
Terminated |
NCT01078987 -
Plasmapheresis for Nephrogenic Fibrosing Dermopathy (NFD)/Nephrogenic Systemic Fibrosis (NSF)
|
Phase 4 | |
Completed |
NCT01014754 -
Nephrogenic Systemic Fibrosis (NSF): Analysis of Tissue Gadolinium Levels
|
N/A |