Adjunct Treatment of Recalcitrant Hand Plaques in Nephrogenic Systemic Fibrosis After Imatinib Therapy
Main Article Content
Keywords
nephrogenic systemic fibrosis, imatinib, corticosteroids, treatment
Abstract
Nephrogenic systemic fibrosis (NSF) is a sclerotic disorder presenting with painful indurated plaques and skin thickening involving the trunk and extremities, which can lead to tethering and joint contractures. NSF most commonly affects patients with renal insufficiency who have been exposed to gadolinium. We present a case of NSF involving the bilateral hands, knees, and lower extremities developing over 10 years after gadolinium exposure. Initial improvement was noted in the lower extremities after initiation of imatinib mesylate therapy, but recalcitrant, thickened hand plaques caused persistent pain and functional limitation. Adjunct intralesional corticosteroid injections produced durable softening of the recalcitrant lesions with considerable functional improvement in hand mobility. Based on our experience, intralesional corticosteroid injections appear to be an effective adjunct treatment in patients with incomplete response to anti-fibrotic therapies.
References
2. Grobner, T. Gadolinium--a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis? Nephrol Dial 2006; 21: 1104–1108.
3. Wermuth, P. J. & Jimenez, S. A. Gadolinium Compounds Signaling through TLR 4 and TLR 7 in Normal Human Macrophages: Establishment of a Proinflammatory Phenotype and Implications for the Pathogenesis of Nephrogenic Systemic Fibrosis. J Immunol 2012; 189:318–327.
4. Distler, J. H. W. & Distler, O. Tyrosine kinase inhibitors for the treatment of fibrotic diseases such as systemic sclerosis: towards molecular targeted therapies. Ann Rheum Dis 2010; 69: i48–51.
5. Akhmetshina, A. et al. Treatment with imatinib prevents fibrosis in diferent preclinical models of systemic sclerosis and induces regression of established fibrosis. Arthritis Rheum 2009; 60: 219–224.
6. Elmholdt, T. R., Pedersen, M., Jørgensen, B., Ramsing, M. & Olesen, A. B. Positive effect of low-dose imatinib mesylate in a patient with nephrogenic systemic fibrosis. Acta Derm Venereol 2011; 91: 478–479.
7. Kay J, High WA. Imatinib mesylate treatment of nephrogenic systemic fibrosis. Arthritis Rheum 2008; 58: 2543–2548.
8. Clements, P. J. et al. Skin thickness score in systemic sclerosis: an assessment of interobserver variability in 3 independent studies. J Rheumatol 1993; 20: 1892–1896.
9. Larson KN, Gagnon AL, Darling MD, Patterson JW & Cropley TG. Nephrogenic systemic fibrosis manifesting a decade after exposure to gadolinium. JAMA 2015; 151: 1117-20.
10. Iudici, M., van der Goes, M. C., Valentini, G. & Bijlsma, J. W. J. Glucocorticoids in systemic sclerosis: weighing the benefits and risks - a systematic review. Clin Exp Rheumatol 2013; 31: 157–165.
11. Spiera RF, Gordon JK, Mersten JN, et al. Imatinib mesylate (Gleevec) in the treatment of diffuse cutaneous systemic sclerosis: results of a 1-year, phase IIa, single-arm, open-label clinical trial. Ann Rheum Dis 2011; 70: 1003–9.
Article Sidebar
Article Details
This work is licensed under a Creative Commons Attribution 4.0 International License.
Creative Commons Attribution (CC BY): lets others distribute and copy the article, to create extracts, abstracts, and other revised versions, adaptations or derivative works of or from an article (such as a translation), to include in a collective work (such as an anthology), to text or data mine the article, even for commercial purposes, as long as they credit the author(s), do not represent the author as endorsing their adaptation of the article, and do not modify the article in such a way as to damage the author's honor or reputation.
Authors retain copyright in their work and license the publisher to publish the content. The publisher is the National Society for Cutaneous Medicine, with production and publishing support provided by OJS, Open Journal Systems,see https://pkp.sfu.
Prior to January 2022, authors transferred copyright to the National Society for Cutaneous Medicine. However, all articles are freely available to anyone in the world. There are no subscription fees, page charges, or article processing charges.