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Updated: Jan 27, 2026
Direct Drug Delivery to Kidney via the Renal Artery
Published on: April 17, 2021
Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication
Pia Moinzadeh1,2, Kathrin Kuhr3,4, Elise Siegert3,4
1From the Department of Dermatology and Venereology, University Hospital Cologne; Institute of Medical Statistics and Computational Biology, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne; Department of Rheumatology, Charité Universitätsmedizin Berlin; Department of Dermatology and Allergology, Charité Universitätsmedizin Berlin; Department of Rheumatology, Immanuel Krankenhaus Berlin-Buch, Berlin; Department of Rheumatology, University Hospital Heidelberg, Heidelberg; Department of Dermatology and Venereology, University Hospital Muenster, Muenster; University Hospital Halle, Halle; Centre for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases; Department of Internal Medicine II (Oncology, Hematology, Immunology, Rheumatology, Pulmonology), University Hospital Tuebingen, Tuebingen; Department for Internal Medicine, Rheumatology, Immunology and Nephrology, Asklepios Clinic Altona, Hamburg; Department of Rheumatology, University Hospital Wuerzburg, Wuerzburg; Department of Rheumatology, Krankenhaus St. Josef; Department of Dermatology, HELIOS University Hospital Wuppertal, Wuppertal; Department of Dermatology, University Hospital Carl Gustav Carus, Dresden; Department of Dermatology and Venereology, Ruhr-University-Bochum, Bochum; Department of Rheumatology, University Medical Center-UKSH, Luebeck; Department of Dermatology, Venereology and Allergology, HELIOS St. Elisabeth Hospital Oberhausen, University Witten-Herdecke, Witten; Department of Rheumatology, Johanniter-Krankenhaus im Flaeming Treuenbrietzen, Treuenbrietzen; University Medical Center Freiburg, Freiburg; Department of Rheumatology, Justus Liebig University Giessen, Kerckhoff Clinic, Bad Nauheim; Department of Rheumatology, University Hospital Erlangen, Erlangen; Department of Dermatology and Allergology, Ludwig Maximilian University, Munich, Germany; Department of Dermatology, Medical University of Graz, Graz, Austria. pia.moinzadeh@uk-koeln.de.
Objective:
Scleroderma renal crisis (SRC) is a severe life-threatening manifestation in patients with systemic sclerosis (SSc). However, the knowledge about risk factors for SRC is limited. We determined here the frequency of SRC and identified risk factors for the prediction of SRC.
Methods:
Based on regular followup data from the German Network for Systemic Scleroderma, we used univariate and multivariate generalized estimating equations to analyze the association between clinical variables, SSc subsets, therapy [i.e., angiotensin-converting enzyme inhibitors (ACEi), corticosteroids], and the occurrence of SRC.
Results:
Data of 2873 patients with 10,425 visits were available for analysis with a mean number of registry visits of 3.6 ± 2.8 and a mean time of followup of 3.6 ± 3.8 years. In total, 70 patients developed SRC (70/2873, 2.4%). Of these patients, 57.1% (40/70) were diagnosed with diffuse cutaneous SSc, 31.4% (22/70) with limited cutaneous SSc, and 11.4% (8/70) with SSc-overlap syndromes. Predictive independent factors with the highest probability for SRC were positive anti-RNA polymerase antibodies (RNAP), a history of proteinuria prior to SRC onset, diminished DLCO, and a history of hypertension. Interestingly, positive antitopoisomerase autoantibodies did not predict a higher risk for SRC. Further, patients with SRC were significantly more frequently treated with ACEi and corticosteroids without being independently associated with SRC.
Conclusion:
In this cohort, SRC has become a rare complication. By far the highest risk for SRC was associated with the detection of anti-RNAP and proteinuria.
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