Scleroderma renal crisis: Risk factors for an increasingly rare organ complication

Research output: Contribution to journalResearch articleContributedpeer-review

Contributors

  • Pia Moinzadeh - , University of Cologne (Author)
  • Kathrin Kuhr - , University of Cologne (Author)
  • Elise Siegert - , Charité – Universitätsmedizin Berlin (Author)
  • Norbert Blank - , Heidelberg University  (Author)
  • Cord Sunderkoetter - , University of Münster (Author)
  • Jörg Henes - , University of Tübingen (Author)
  • Martin Krusche - , Asklepios Clinic Altona (Author)
  • Marc Schmalzing - , University of Würzburg (Author)
  • Margitta Worm - , Charité – Universitätsmedizin Berlin (Author)
  • Tim Schmeiser - , Krankenhaus St. Josef (Author)
  • Claudia Günther - , Department of Dermatology, University Hospital Carl Gustav Carus Dresden (Author)
  • Elisabeth Aberer - , Medical University of Graz (Author)
  • Laura Susok - , Ruhr University Bochum (Author)
  • Gabriela Riemekasten - , University Hospital Schleswig-Holstein Campus Kiel (Author)
  • Alexander Kreuter - , Witten/Herdecke University (Author)
  • Gabriele Zeidler - , Evangelical Clinics of Bonn (Author)
  • Aaron Juche - , Fresenius AG (Author)
  • Denitsa Hadjiski - , University Medical Center Freiburg (Author)
  • Ulf Müller-Ladner - , Justus Liebig University Giessen (Author)
  • Noemi Gaebelein-Wissing - , Helios University Hospital Wuppertal (Author)
  • Jörg H.W. Distler - , Friedrich-Alexander University Erlangen-Nürnberg (Author)
  • Miklós Sárdy - , Ludwig Maximilian University of Munich (Author)
  • Thomas Krieg - , University of Cologne (Author)
  • Nicolas Hunzelmann - , University of Cologne (Author)

Abstract

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.

Details

Original languageEnglish
Pages (from-to)241-248
Number of pages8
JournalJournal of rheumatology
Volume47
Issue number2
Publication statusPublished - 1 Feb 2020
Peer-reviewedYes

External IDs

PubMed 30936287
ORCID /0000-0002-4330-1861/work/151982038

Keywords

Keywords

  • German network for systemic scleroderma, Renal crisis, Scleroderma, Scleroderma renal crisis, Systemic sclerosis