Predictors and Risk Factors for Recurrent Scleroderma Renal Crisis in the Kidney Allograft: Case Report and Review of the Literature

Abstract

Scleroderma renal crisis (SRC) can lead to end-stage renal disease (ESRD) and subsequent need for dialysisand/or renal transplantation. We review all reported cases of renal transplantations in scleroderma patients from PubMed search, present UNOS data on transplant outcomes, and identify predictors for allograft SRC. Of the five cases with recurrent SRC, all developed ESRD within a year of onset of native kidney SRC, whereas none of those who developed ESRD more than 1-2 years after the onset of SRC developed recurrence. Anemia preceded allograft SRC in two cases, pericardial effusion in one, and skin tightening in two others. UNOS data (October 1987-July 2004) documented 260 transplants performed for the renal diagnosis of scleroderma, with a 5-year graft survival rate of 56.7%. The risk for allograft SRC recurrence appears to correlate with early native renal function loss following the onset of SRC. Recurrent SRC in the allograft may be heralded by multiple clinical markers known to be predictive of severe scleroderma, including progression of diffuse skin thickening, new-onset anemia and cardiac complications.

Prognose und Therapie von entzündlich-rheumatischen Erkrankungen

“…Ansonsten ist die Nierentransplantation eine wichtige Behandlungsoption. Die 5-Jahres-Überlebensrate der Transplantate beträgt 56,7%, mit einem Rezidiv der Erkrankung nach Transplantation in 6,7% [40]. Calcineurininhibitoren können eine ARC begünstigen und werden daher bei transplantierten Patienten mit SyS nicht empfohlen.…”

Rekurrenz der Grundkrankheit nach Nierentransplantation

“…Am Anfang des Geschehens steht vermutlich eine autoantikörperindu-zierte Endothelschädigung. Rekurrenzen im Transplantat finden sich bei 3,5-45% und treten bereits in den ersten 1-2 Jahren nach Transplantation auf [26]. Risikofaktor für eine Rekurrenz ist eine rasche, progrediente Insuffizienz der Eigennieren (innerhalb von einem Jahr).…”

Scleroderma Renal Crisis

“…Post-transplant recurrence of SRC has been reported even after transplantation from a twin sister (Caplin, Dikman, Winston, Spiera, & Uribarri, 1999;Woodhall, McCoy, Gunnells, & Seigler, 1976). Pham et al (Pham, et al, 2005) suggested that SRC recurrence occurs early in the post-transplantation course. However, Cheung et al (Cheung, et al, 2005) challenged this view when describing a late SRC recurrence observed seven years post-transplantation.…”

“…In SRC, graft and patient survival post-transplantation is inferior to general renal transplant patient (Pham et al, 2005). Recurrence of scleroderma may participate in this poor outcome (Cheung, Gibson, Rush, Jeffery, & Karpinski, 2005).…”