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.
ISSN 2220-3230, 2220-3230
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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: | 06 Dec 2024 20:42 |
| DOI: | 10.5500/wjt.v11.i9.372 |
| Related URLs: | |
| URI: | https://livrepository.liverpool.ac.uk/id/eprint/3166053 |
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