TOPIC DISCUSSION: NSF post transplant. theraputic challenge

A patient with history of Minimal Change disease and lupus nephritis who had renal transplant few years back. unfortunately immediately before transplant she had MRI with Gad. for years she had been complaining of a taught skin and immobility in her joints. recently she was seen by rhem and a skin biopsy showed NSF. her Cr 1.0. she is on prograf, cellcept prednison, what could we do to treat NSF? since we're able to diagnose it.

so NSF has been recently recognized as potentially a fatal disease. it is characterized by myofibroblasts depositions in internal organs such as liver, heart, kidneys etc as well as externally mainly the skin. it presents as painful,symmetrical thickening of the skin and the joints that can be misdiagnosed as scleroderma and alike,calciphylaxis, cellulitis. it follows a progressive unremitting coarse. major risk identified is exposure to gadolinium in dialysis patients or CKD patients with GFR<30. it maybe dose related.
all cases are retrospective, and all have been exposed to gad by history.

how?Free Gd3+ is poorly soluble, highly toxic, and can form precipitates with anions that tend to be elevated in renal failure. This has led to the hypothesis that excess exposure to free Gd3+ in patients with kidney disease leads to tissue damage.PD patient maybe at higher risk. Gad half life is dramatically increased to 1.3hrs in healthy to over 15 hours in CKD 5 patients.

Initiation of recombinant human erythropoietin (EPO) therapy or an increase in dose may be associated with NSF, but the true nature of the relationship between EPO and NSF remains incompletely understood.A case report described two patients without a history of exposure to gadolinium who developed NSF post-kidney transplantation ;the authors postulated that vascular manipulation or endothelial injury was a possible trigger.

treatment:case series. transplantation, Extracorporeal photopheresis ,Ultraviolet A1 (UV-A1) phototherapy, Plasmapheresis and more recently rapamune and gleevac have been proposed. all of these therapies have been inconsistent and disappointing.

back to the above case: options are either switching to rapamune based regimen. or addition to gleevac with special attention to infectious risks and significant edema developing in these patients.