Which antibodies may have a role in the pathophysiology of nephrogenic systemic fibrosis (NSF)?

Updated: May 22, 2018
  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Parsons et al [21] performed immunohistochemical studies using antibodies to transglutaminase-2, factor XIIIa, transglutaminase isopeptide, and the histiocyte marker CD68 on 5 archived skin biopsy specimens of nephrogenic systemic fibrosis. Parsons et al found that dermal fibroblasts and histiocytes of nephrogenic systemic fibrosis expressed transglutaminase-2, CD68, factor XIIIa, and transglutaminase isopeptide. They posited that this represented increased expression, activation, or concomitant activation and expression of transglutaminases in nephrogenic systemic fibrosis.

Edward et al [22] found that fibroblasts derived from skin affected by nephrogenic systemic fibrosis synthesize elevated levels of sulphated glycosaminoglycans, in particular hyaluronan, compared with normal control samples, while serum from the one patient with dermatomyositis and from the 2 patients with nephrogenic systemic fibrosis stimulated sulphated glycosaminoglycans synthesis, including hyaluronan synthesis, by both control and patient fibroblasts. Metformin use might influence nephrogenic systemic fibrosis, but this is not proven. [23]

Iron metabolism shapes the development of nephrogenic systemic fibrosis in a mouse model. [24]


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