Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication
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Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication. / Moinzadeh, Pia; Kuhr, Kathrin; Siegert, Elise; Blank, Norbert; Sunderkoetter, Cord; Henes, Jörg; Krusche, Martin; Schmalzing, Marc; Worm, Margitta; Schmeiser, Tim; Günther, Claudia; Aberer, Elisabeth; Susok, Laura; Riemekasten, Gabriela; Kreuter, Alexander; Zeidler, Gabriele; Juche, Aaron; Hadjiski, Denitsa; Müller-Ladner, Ulf; Gaebelein-Wissing, Noemi; Distler, Jörg H W; Sárdy, Miklós; Krieg, Thomas; Hunzelmann, Nicolas.
in: J RHEUMATOL, Jahrgang 47, Nr. 2, 02.2020, S. 241-248.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication
AU - Moinzadeh, Pia
AU - Kuhr, Kathrin
AU - Siegert, Elise
AU - Blank, Norbert
AU - Sunderkoetter, Cord
AU - Henes, Jörg
AU - Krusche, Martin
AU - Schmalzing, Marc
AU - Worm, Margitta
AU - Schmeiser, Tim
AU - Günther, Claudia
AU - Aberer, Elisabeth
AU - Susok, Laura
AU - Riemekasten, Gabriela
AU - Kreuter, Alexander
AU - Zeidler, Gabriele
AU - Juche, Aaron
AU - Hadjiski, Denitsa
AU - Müller-Ladner, Ulf
AU - Gaebelein-Wissing, Noemi
AU - Distler, Jörg H W
AU - Sárdy, Miklós
AU - Krieg, Thomas
AU - Hunzelmann, Nicolas
PY - 2020/2
Y1 - 2020/2
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.
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.
KW - Adult
KW - Autoantibodies/immunology
KW - DNA-Directed RNA Polymerases/immunology
KW - Female
KW - Follow-Up Studies
KW - Humans
KW - Hypertension/complications
KW - Male
KW - Middle Aged
KW - Prognosis
KW - Prospective Studies
KW - Proteinuria/complications
KW - Pulmonary Diffusing Capacity
KW - Registries
KW - Renal Insufficiency/etiology
KW - Risk Factors
KW - Scleroderma, Systemic/complications
U2 - 10.3899/jrheum.180582
DO - 10.3899/jrheum.180582
M3 - SCORING: Journal article
C2 - 30936287
VL - 47
SP - 241
EP - 248
JO - J RHEUMATOL
JF - J RHEUMATOL
SN - 0315-162X
IS - 2
ER -