End-stage kidney disease due to scleroderma—outcomes in 127 consecutive ANZDATA registry cases

• Published in: Nephrology, dialysis, transplantation Vol. 26; no. 10; pp. 3165 - 3171
• Main Authors: SIVA, Brian, MCDONALD, Stephen P, HAWLEY, Carmel M, ROSMAN, Johan B, BROWN, Fiona G, WIGGINS, Kathryn J, BANNISTER, Kym M, CAMPBELL, Scott B, JOHNSON, David W
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.10.2011
• Subjects: Adult; Aged; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Australia - epidemiology; Biological and medical sciences; Cohort Studies; Comorbidity; Emergency and intensive care: renal failure. Dialysis management; Female; Follow-Up Studies; Humans; Intensive care medicine; Kidney Failure, Chronic - epidemiology; Kidney Failure, Chronic - etiology; Kidney Failure, Chronic - mortality; Kidney Transplantation - mortality; Male; Medical sciences; Middle Aged; Nephrology. Urinary tract diseases; Nephropathies. Renovascular diseases. Renal failure; Peritoneal Dialysis; Prevalence; Prognosis; Registries; Renal failure; Scleroderma, Systemic - complications; Survival Rate; Australia; Kidney disease; Immunopathology; Connective tissue disease; Skin disease; end-stage renal failure; Urinary system disease; Renal function; Prognosis; renal function recovery; Hemodialysis; kidney failure; Autoimmune disease; Terminal stage; Extrarenal dialysis; outcomes; Systemic disease; Renal failure; progressive systemic sclerosis; Scleroderma; chronic
• ISSN: 0931-0509; 1460-2385
• DOI: 10.1093/ndt/gfq861

Scleroderma is an uncommon cause of end-stage kidney disease (ESKD) which carries significant morbidity and mortality risks. The aim of this study was to determine the prevalence, treatment and outcomes of scleroderma patients with ESKD. A study was conducted of all ESKD patients enrolled in the ANZDATA registry, who commenced dialysis between 15 May 1963 and 31 December 2005, and remained on dialysis for at least 90 days. Of the 40 238 patients who commenced dialysis during the study period, 127 (0.3%) patients had ESKD secondary to scleroderma. Scleroderma ESKD patients were more likely than other ESKD patients to be female (72% versus 43%, P < 0.001), Caucasian (98% versus 79%, P < 0.001) and of lower BMI (22.7 ± 4.7 versus 26.0 ± 5.9, P < 0.001) with a higher prevalence of chronic lung disease (36 versus 14%, P < 0.001) and lower prevalence of diabetes mellitus (10% versus 32%, P < 0.001) and coronary artery disease (23% versus 35%, P = 0.01). Median survival was significantly shorter in scleroderma ESKD (2.43 years, 95% confidence interval (CI) 1.75-3.11 years) than other ESKD (6.02 years, 95% CI 5.89-6.14 years, log-rank score 55.7, P < 0.001). Renal recovery was more likely in scleroderma patients (10% versus 1%, P < 0.001) with a shorter time to recovery. Scleroderma was found to be an independent predictor for mortality (HR 2.47, 95% CI 1.99-3.05) and renal recovery (HR 11.1, 95% CI 6.37-19.4). Five year deceased donor and live donor renal allograft survival rates of recipients with scleroderma were 53 and 100%, respectively. Scleroderma is an uncommon cause of ESKD, which is associated with increased risks of both spontaneous renal recovery and mortality.