Kidney Transplantation in a Patient with Scleroderma

Area of Science:

• Rheumatology
• Nephrology
• Autoimmune Diseases

Background:

• Kidney involvement is frequent in systemic sclerosis (SSc), affecting about 50% of patients.
• Scleroderma renal crisis (SRC), a severe kidney complication, impacts roughly 20% of SSc patients, particularly those with diffuse cutaneous SSc (dcSSc) and anti-RNA polymerase III antibodies.
• The introduction of angiotensin-converting-enzyme inhibitors (ACE-i) has decreased SRC prevalence and mortality.

Purpose of the Study:

• To review the epidemiology and outcomes of kidney involvement in systemic sclerosis.
• To highlight the impact of scleroderma renal crisis (SRC) and its management.
• To discuss the progression to end-stage renal disease (ESRD) and renal replacement therapies (RRTs) in SSc patients.

Main Methods:

• Literature review focusing on kidney manifestations in systemic sclerosis.
• Analysis of data regarding scleroderma renal crisis (SRC) prevalence, mortality, and treatment.
• Examination of end-stage renal disease (ESRD) and renal transplant rates in SSc.

Main Results:

• SRC mortality has decreased from 78% to 30-36% post-ACE inhibitor introduction.
• Despite improved outcomes, progression to end-stage renal disease (ESRD) requiring renal replacement therapies (RRTs) is common.
• Renal transplant rates in SSc patients with ESRD remain relatively low (4-17%).

Conclusions:

• Systemic sclerosis frequently involves the kidneys, with SRC being a major concern.
• While ACE inhibitors have improved SRC outcomes, long-term renal function and RRTs remain critical issues for SSc patients.
• Further research is needed to optimize renal care and transplantation strategies for SSc patients.