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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, Vol. 47, No. 2, 02.2020, p. 241-248.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Moinzadeh, P, Kuhr, K, Siegert, E, Blank, N, Sunderkoetter, C, Henes, J, Krusche, M, Schmalzing, M, Worm, M, Schmeiser, T, Günther, C, Aberer, E, Susok, L, Riemekasten, G, Kreuter, A, Zeidler, G, Juche, A, Hadjiski, D, Müller-Ladner, U, Gaebelein-Wissing, N, Distler, JHW, Sárdy, M, Krieg, T & Hunzelmann, N 2020, 'Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication', J RHEUMATOL, vol. 47, no. 2, pp. 241-248. https://doi.org/10.3899/jrheum.180582

APA

Moinzadeh, P., Kuhr, K., Siegert, E., Blank, N., Sunderkoetter, C., Henes, J., Krusche, M., Schmalzing, M., Worm, M., Schmeiser, T., Günther, C., Aberer, E., Susok, L., Riemekasten, G., Kreuter, A., Zeidler, G., Juche, A., Hadjiski, D., Müller-Ladner, U., ... Hunzelmann, N. (2020). Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication. J RHEUMATOL, 47(2), 241-248. https://doi.org/10.3899/jrheum.180582

Vancouver

Moinzadeh P, Kuhr K, Siegert E, Blank N, Sunderkoetter C, Henes J et al. Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication. J RHEUMATOL. 2020 Feb;47(2):241-248. https://doi.org/10.3899/jrheum.180582

Bibtex

@article{df024751b26a4574a245e6f7fe05e3bf,
title = "Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication",
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.",
keywords = "Adult, Autoantibodies/immunology, DNA-Directed RNA Polymerases/immunology, Female, Follow-Up Studies, Humans, Hypertension/complications, Male, Middle Aged, Prognosis, Prospective Studies, Proteinuria/complications, Pulmonary Diffusing Capacity, Registries, Renal Insufficiency/etiology, Risk Factors, Scleroderma, Systemic/complications",
author = "Pia Moinzadeh and Kathrin Kuhr and Elise Siegert and Norbert Blank and Cord Sunderkoetter and J{\"o}rg Henes and Martin Krusche and Marc Schmalzing and Margitta Worm and Tim Schmeiser and Claudia G{\"u}nther and Elisabeth Aberer and Laura Susok and Gabriela Riemekasten and Alexander Kreuter and Gabriele Zeidler and Aaron Juche and Denitsa Hadjiski and Ulf M{\"u}ller-Ladner and Noemi Gaebelein-Wissing and Distler, {J{\"o}rg H W} and Mikl{\'o}s S{\'a}rdy and Thomas Krieg and Nicolas Hunzelmann",
year = "2020",
month = feb,
doi = "10.3899/jrheum.180582",
language = "English",
volume = "47",
pages = "241--248",
journal = "J RHEUMATOL",
issn = "0315-162X",
publisher = "Journal of Rheumatology",
number = "2",

}

RIS

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 -