Moinzadeh, Pia, Kuhr, Kathrin, Siegert, Elise, Blank, Norbert, Sunderkoetter, Cord, Henes, Joerg, Krusche, Martin ORCID: 0000-0002-0582-7790, Schmalzing, Marc, Worm, Margitta, Schmeiser, Tim, Guenther, Claudia, Aberer, Elisabeth, Susok, Laura, Riemekasten, Gabriela, Kreuter, Alexander, Zeidler, Gabriele, Juche, Aaron, Hadjiski, Denitsa, Mueller-Ladner, Ulf, Gaebelein-Wissing, Noemi, Distler, Joerg H. W., Sardy, Miklos ORCID: 0000-0003-4306-5093, Krieg, Thomas and Hunzelmann, Nicolas (2020). Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication. J. Rheumatol., 47 (2). S. 241 - 249. TORONTO: J RHEUMATOL PUBL CO. ISSN 1499-2752

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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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Moinzadeh, PiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kuhr, KathrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Siegert, EliseUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Blank, NorbertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sunderkoetter, CordUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Henes, JoergUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Krusche, MartinUNSPECIFIEDorcid.org/0000-0002-0582-7790UNSPECIFIED
Schmalzing, MarcUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Worm, MargittaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schmeiser, TimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Guenther, ClaudiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Aberer, ElisabethUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Susok, LauraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Riemekasten, GabrielaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kreuter, AlexanderUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zeidler, GabrieleUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Juche, AaronUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hadjiski, DenitsaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mueller-Ladner, UlfUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gaebelein-Wissing, NoemiUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Distler, Joerg H. W.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sardy, MiklosUNSPECIFIEDorcid.org/0000-0003-4306-5093UNSPECIFIED
Krieg, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hunzelmann, NicolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-346092
DOI: 10.3899/jrheum.180582
Journal or Publication Title: J. Rheumatol.
Volume: 47
Number: 2
Page Range: S. 241 - 249
Date: 2020
Publisher: J RHEUMATOL PUBL CO
Place of Publication: TORONTO
ISSN: 1499-2752
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
SYSTEMIC-SCLEROSIS; DISEASE PROGRESSION; INVOLVEMENT; FREQUENCY; MANAGEMENT; DIAGNOSIS; SURVIVAL; DEATHMultiple languages
RheumatologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/34609

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