Session Type: Abstract Submissions (ACR)
Scleroderma renal crisis (SRC) usually presenting with accelerated hypertension and acute kidney injury (AKI) is one of the most severe complications of Systemic Sclerosis (SSc). The presence of RNA-polymerase III auto-antibodies (ARA) is recognized as a strong risk factor for SRC but studies have not explored long-term outcomes in ARA positive cases compared to ARA negative cases.
Of more than 2000 SSc patients attending our institution between 1990-2013, 150 patients had a confirmed SRC. 80% patients had diffuse cutaneous SSc (dcSSc) and 20% patients had limited cutaneous SSc (dcSSc). ARA was measured by commercial ELISA or radio-immuno-precipitation. Patients were divided into two groups: ARA positive or ARA negative. Demographic and clinical parameters were compared between groups using Student’s t-test or Chi-squared analyses where appropriate.
61/150 (41%) patients were ARA positive and significantly more likely to have dcSSc (88.3% vs 73.8%, p=0.032) than lcSSc compared to ARA negative patients. There was no significant difference in age of onset of SRC (51.2 vs 51.9 years) or the number of females (73% vs 79%) between the two groups. 50.8% of ARA positive patients required dialysis compared to 29.2% of ARA negative patients (p=0.07). The mean time to recovery of renal function was significantly longer in ARA positive patients (14.25 vs 8.27 months, p=0.032). Significantly more ARA positive patients were able to discontinue dialysis compared to ARA negative patients (53.3% vs 25.5%, p=0.01). ARA positive patients had a significantly better survival outcome (figure 1).
In SRC, although more ARA positive patients required dialysis they also had significantly greater capacity for long-term recovery and survival compared to ARA negative patients.
Figure 1: Kaplan Meier survival estimate showing the effect of ARA positive auto-antibodies in patients with SRC
C. P. Denton,
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-prognosis-of-scleroderma-renal-crisis-in-rna-polymerase-iii-antibody-ara-positive-compared-to-ara-negative-patients/