Abstract # 32: Imatinib Mesylate Treatment Improves Skin Changes of Nephrogenic Systemic Fibrosis
Authors:
J. Kay
Background
This study is a report of 2 patients with Nephrogenic Systemic Fibrosis (NSF) treated with the tyrosine kinase inhibitor, imatinib.
Methods:
Two patients on dialysis with NSF were treated with imatinib (Gleevec) 400 mg PO daily and had 15 weeks of follow up data. The patients were assessed using the modified Rodnan skin score (mRSS). Range of motion of the elbow and knee were also assessed and pain measured on a 10 cm visual analog scale.
Results:
Both patients had a >60% improvement in mRSS at 15 weeks. One patient had significant reduction in pain (from 5/10 to 0/10 on visual analog scale) and a twenty degree reduction in flexion contractures of the knees. Both patients had trouble with fluid retention.
Editorial Comment:
Nephrogenic systemic fibrosis (or nephrogenic fibrosing dermopathy) is a fibrosing skin condition associated with exposure to gadolinium in patients with renal insufficiency/failure. This condition has been extremely difficult to treat. There has been a significant interest in the use of tyrosine kinase inhibitors for fibrosing diseases, namely systemic sclerosis. Imatinib could potentially modify the fibrotic process via the PDGF pathway or via TGF-beta signaling through the Abl pathway. Data published in the New England Journal of Medicine in 2006 suggested that anti-PDGFR antibodies may play a role in the fibrotic process in scleroderma (Baroni et al. NEJM, 2006 ). In addition several investigators have shown inhibition of fibrosis in several mouse models (Distler et al., Arthritis Rheum, 2006 ). There is no clinical data to show benefit in scleroderma yet, but multiple studies are underway or planned. This limited case series does suggest, however, that this strategy may prove to be beneficial for this and other fibrosing skin diseases and should be further pursued.
