The Risk of Scleroderma Renal Crisis After Intravenous Corticosteroid Administered with Rituximab

Background/Objectives: This retrospective study determined whether high-dose intravenous (IV) corticosteroid administered during biologic therapy for systemic sclerosis (SSc) was associated with scleroderma renal crisis (SRC). Methods: The outcomes in SSc patients who received high-dose IV methylprednisolone were compared to non-corticosteroid SSc controls. Blood pressure (BP), serum creatinine and glucose, urine protein, and episodes of SRC were determined at baseline, 4 h, 2 weeks, and 24 weeks after IV corticosteroid. Results: The SSc corticosteroid infusion (37 subjects) and non-corticosteroid (120 subjects) cohorts were similar in age, race, disease duration, antinuclear and topoisomerase-1 antibody positivity, interstitial lung disease, and pulmonary artery hypertension (all p > 0.05). However, the corticosteroid group was characterized by a higher proportion of men (18.9 vs. 6.7%, p = 0.04), diffuse cutaneous SSc (75.7 vs. 45.9%, p < 0.001), and anti-RNA polymerase III positivity (21.6 vs. 10%, p = 0.04). No patients in the control group developed SRC. In the corticosteroid group, after a total of 827 infusions (21 ± 23 infusions/subject) with 135 mg ± 66 mg methylprednisolone/infusion and a cumulative dose of 2983 mg ± 5198 mg methylprednisolone, none developed SRC. All-cause mortality (37.8 vs. 19.2%, HR 2.23, 95% CI 1.14–4.37, p = 0.02) during the mean 11.6 ± 6.2-year disease duration was increased in the corticosteroid group; however, 5-year (92% vs. 91%, p > 0.05) survival rates were similar. In the high-risk anti-RNA polymerase III antibody subgroup, there were no episodes of SRC or significant changes in blood pressure or renal function at 4 h, 2 weeks, and 24 weeks after corticosteroid. Conclusions: These data suggest that intermittent high-dose IV corticosteroid premedication for biologic infusions may have a lower risk for SRC.