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



Abbas, Fedaey, El Kossi, Mohsen, Shaheen, Ihab Sakr, Sharma, Ajay and Halawa, Ahmed ORCID: 0000-0002-7305-446X
(2021) Journey of a patient with scleroderma from renal failure up to kidney transplantation. World journal of transplantation, 11 (9). pp. 372-387.

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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.

Item Type: Article
Uncontrolled Keywords: Hemodialysis, Kidney transplant, Renal failure, Risk factors, Scleroderma renal crisis, Systemic sclerosis
Divisions: Faculty of Health and Life Sciences
Faculty of Health and Life Sciences > Institute of Life Courses and Medical Sciences
Faculty of Health and Life Sciences > Institute of Life Courses and Medical Sciences > School of Medicine
Depositing User: Symplectic Admin
Date Deposited: 08 Nov 2022 14:40
Last Modified: 18 Jan 2023 19:43
DOI: 10.5500/wjt.v11.i9.372
Related URLs:
URI: https://livrepository.liverpool.ac.uk/id/eprint/3166053