RT Journal Article
SR Electronic
T1 Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 241
OP 248
DO 10.3899/jrheum.180582
VO 47
IS 2
A1 Pia Moinzadeh
A1 Kathrin Kuhr
A1 Elise Siegert
A1 Norbert Blank
A1 Cord Sunderkoetter
A1 Jörg Henes
A1 Martin Krusche
A1 Marc Schmalzing
A1 Margitta Worm
A1 Tim Schmeiser
A1 Claudia Günther
A1 Elisabeth Aberer
A1 Laura Susok
A1 Gabriela Riemekasten
A1 Alexander Kreuter
A1 Gabriele Zeidler
A1 Aaron Juche
A1 Denitsa Hadjiski
A1 Ulf Müller-Ladner
A1 Noemi Gaebelein-Wissing
A1 Jörg H.W. Distler
A1 Miklós Sárdy
A1 Thomas Krieg
A1 Nicolas Hunzelmann
YR 2020
UL http://www.jrheum.org/content/47/2/241.abstract
AB 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.