TY - JOUR T1 - Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication JF - The Journal of Rheumatology JO - J Rheumatol SP - 241 LP - 248 DO - 10.3899/jrheum.180582 VL - 47 IS - 2 AU - Pia Moinzadeh AU - Kathrin Kuhr AU - Elise Siegert AU - Norbert Blank AU - Cord Sunderkoetter AU - Jörg Henes AU - Martin Krusche AU - Marc Schmalzing AU - Margitta Worm AU - Tim Schmeiser AU - Claudia Günther AU - Elisabeth Aberer AU - Laura Susok AU - Gabriela Riemekasten AU - Alexander Kreuter AU - Gabriele Zeidler AU - Aaron Juche AU - Denitsa Hadjiski AU - Ulf Müller-Ladner AU - Noemi Gaebelein-Wissing AU - Jörg H.W. Distler AU - Miklós Sárdy AU - Thomas Krieg AU - Nicolas Hunzelmann Y1 - 2020/02/01 UR - http://www.jrheum.org/content/47/2/241.abstract N2 - 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. ER -