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

Publikation: Beitrag in FachzeitschriftForschungsartikelBeigetragenBegutachtung

Beitragende

  • Pia Moinzadeh - , Universität zu Köln (Autor:in)
  • Kathrin Kuhr - , Universität zu Köln (Autor:in)
  • Elise Siegert - , Charité – Universitätsmedizin Berlin (Autor:in)
  • Norbert Blank - , Universität Heidelberg (Autor:in)
  • Cord Sunderkoetter - , Westfälische Wilhelms-Universität Münster (Autor:in)
  • Jörg Henes - , Eberhard Karls Universität Tübingen (Autor:in)
  • Martin Krusche - , Asklepios Klinik Altona (Autor:in)
  • Marc Schmalzing - , Julius-Maximilians-Universität Würzburg (Autor:in)
  • Margitta Worm - , Charité – Universitätsmedizin Berlin (Autor:in)
  • Tim Schmeiser - , Krankenhaus St. Josef (Autor:in)
  • Claudia Günther - , Klinik und Poliklinik für Dermatologie, Universitätsklinikum Carl Gustav Carus Dresden (Autor:in)
  • Elisabeth Aberer - , Medizinische Universität Graz (Autor:in)
  • Laura Susok - , Ruhr-Universität Bochum (Autor:in)
  • Gabriela Riemekasten - , Universitätsklinikum Schleswig-Holstein Campus Kiel (Autor:in)
  • Alexander Kreuter - , Witten/Herdecke University (Autor:in)
  • Gabriele Zeidler - , Evangelical Clinics of Bonn (Autor:in)
  • Aaron Juche - , Fresenius AG (Autor:in)
  • Denitsa Hadjiski - , Universitätsklinikum Freiburg (Autor:in)
  • Ulf Müller-Ladner - , Justus Liebig University Giessen (Autor:in)
  • Noemi Gaebelein-Wissing - , Helios Universitätsklinikum Wuppertal (Autor:in)
  • Jörg H.W. Distler - , Friedrich-Alexander-Universität Erlangen-Nürnberg (Autor:in)
  • Miklós Sárdy - , Ludwig-Maximilians-Universität München (LMU) (Autor:in)
  • Thomas Krieg - , Universität zu Köln (Autor:in)
  • Nicolas Hunzelmann - , Universität zu Köln (Autor:in)

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

OriginalspracheEnglisch
Seiten (von - bis)241-248
Seitenumfang8
FachzeitschriftJournal of rheumatology
Jahrgang47
Ausgabenummer2
PublikationsstatusVeröffentlicht - 1 Feb. 2020
Peer-Review-StatusJa

Externe IDs

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

Schlagworte

Schlagwörter

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