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Scleroderma renal crisis (SRC): risk factors for an increasingly rare organ complication.

J Rheumatol; 2019 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-30936287


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 here determined the frequency of SRC and identified risk factors for the prediction of SRC.


Based on regular follow-up data from the German Network for Systemic Scleroderma (DNSS), we used univariate and multivariate generalized estimating equations to explore the association between clinical parameters, SSc subsets, therapy (i.e., ACE-inhibitors, corticosteroids), and the occurrence of SRC.


Data of 2873 patients with 10425 visits were available for analysis with a mean number of registry visits of 3.6±2.8 and a mean time of follow-up of 3.6±3.8 years, respectively. In total, 70 patients developed a SRC (70/2873, 2.4%). Of these patients, 57.1% (40/70) were diagnosed with diffuse cutaneous SSc (dcSSc), 31.4% (22/70) with limited cutaneous SSc (lcSSc), and 11.4% (8/70) with SScoverlap syndromes, respectively. Predictive independent factors with the highest probability for SRC were positive anti-RNA polymerase (RNAP) autoantibodies, a history of proteinuria prior to SRC onset, diminished DLCO, and a history of hypertension. Interestingly, positive anti-topoisomerase autoantibodies did not predict a higher risk for SRC. Furthermore, patients with SRC were significantly more frequently treated with ACE-inhibitors and corticosteroids without being independently associated with SRC.


In this cohort, SRC has become a rare complication. By far the highest risk for SRC was associated with the detection of anti-RNAP antibodies and proteinuria.