What was the Macay-Wiggan et al theory of the pathogenesis 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

The following discussion is interesting more for historical purposes, because, since the identification of the nephrogenic systemic fibrosis–gadolinium link in 2006, the understanding of nephrogenic systemic fibrosis has changed radically.

Mackay-Wiggan et al [25]

In 2003, Mackay-Wiggan et al found that all patients in their series had anticardiolipin or antiphospholipid antibodies detected on testing. This implied a role for these antibodies in the development of nephrogenic systemic fibrosis. Mackay-Wiggan et al suggested that although these antibodies occur in 10-29% of patients with end-stage renal disease, the antibodies are more common in patients with nephrogenic systemic fibrosis.

Mackay-Wiggan et al also suggested that the lipid molecule of the antiphospholipid or anticardiolipin antibody may interact with a lipid substance in the dialysis procedure. How it interacts is uncertain. Possibly, it interacts with the dialysis machine's filter or the tubing to stimulate fibroblast or mucin production. This cause would not explain the occurrence of nephrogenic systemic fibrosis in the small subset of end-stage renal disease patients with an onset of the disorder before beginning hemodialysis.

Another theory of Mackay-Wiggan et al is that an accumulated substance intrinsic to acute or chronic renal failure may interact with the antiphospholipid antibody. Yet another theory of Mackay-Wiggan et al is that sudden, severe edema may trigger a fibrotic and mucinous cutaneous reaction that results in this progressive scleromyxedemalike illness. They speculate that perhaps edema coupled with immunosuppression in patients with antiphospholipid antibodies stimulate a physiologic response, resulting in the proliferation of fibroblastlike cells and mucin deposition in the dermis.


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