Journey of a patient with scleroderma from renal failure up to kidney transplantation

Abstract

The increased awareness of systemic sclerosis (SS) and its pathogenetic background made the management of this disease more amenable than previously thought. However, scleroderma renal crisis (SRC) is a rarely seen as an associated disorder that may involve 2%-15% of SS patients. Patients presented with earlier, rapidly progressing, diffuse cutaneous SS disease, mostly in the first 3-5 years after non-Raynaud clinical manifestations, are more vulnerable to develop SRC. SRC comprises a collection of acute, mostly symptomatic rise in blood pressure, elevation in serum creatinine concentrations, oliguria and thrombotic microangiopathy in almost 50% of cases. The advent of the antihypertensive angiotensin converting enzyme inhibitors in 1980 was associated with significant improvement in SRC prognosis. In a scleroderma patient maintained on regular dialysis; every effort should be exerted to declare any possible evidence of renal recovery. A given period of almost two years has been suggested prior to proceeding in a kidney transplant (KTx). Of note, SS patients on dialysis have the highest opportunity of renal recovery and withdrawal from dialysis as compared to other causes of end-stage renal disease (ESRD). KTx that is the best well-known therapeutic option for ESRD patients can also be offered to SS patients. Compared to other primary renal diseases, SS-related ESRD was considered for a long period of poor patient and allograft survivals. Pulmonary involvement in an SS patient is considered a strong post-transplant independent risk factor of death. Recurrence of SRC after transplantation has been observed in some patients. However, an excellent post-transplant patient and graft outcome have been recently reported. Consequently, the absence of extrarenal manifestations in an SS-induced ESRD patient can be accepted as a robust indicator for a successful KTx.

Keywords: Systemic sclerosis, Scleroderma renal crisis, Risk factors, Renal failure, Hemodialysis, Kidney transplant

Core Tip: The current progress in the management of systemic sclerosis has its impact on improving patient’s survival and quality of life. Patients developed scleroderma renal crisis have greatly managed after commencing angiotensin converting enzyme inhibitors. Moreover, scleroderma patient with kidney failure has a marvelous therapeutic option receiving a kidney transplant with a greatly improved extrarenal manifestations. However, patients with end stage kidney failure, maintained on regular dialysis, should have enough period permitting renal recovery before attempting the transplant procedures. This duration may be actually extended up to two years. Patients with scleroderma may show the highest rate of renal recovery among dialysis patients.