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.

Sine Scleroderma, Limited Cutaneous and Diffused Cutaneous Systemic Sclerosis Survival and Predictors of Mortality

“…Other explanations could account for our SMR within the lower range of published data such as a lower number of diffuse cutaneous subtype of SSc (16.8% vs. 23.4% to 46%) [8][9][10][11]19,[43][44][45][46][47] , a lower number of patients with a SRC (2.4% compared to 2.9%-10%) [9][10][11]19,43,47], and a less extended level of cutaneous involvement (average Rodnan skin score of 6.8 for the patients in the present study vs. 9) [11]. Regarding SRC, we described the same incidence as in the recent German epidemiological study evaluating renal involvement on 2873 patients with SSc [48]. In addition, we kept in our analysis SSc sine scleroderma, which is often excluded from such studies that could explain our relatively low SMR.…”

Iatrogenic scleroderma renal crisis: A case report and mini literature review for renal crisis

“…The independent risk factor with the highest probability of SRC for autoantibodies is positive anti-RNA polymerase III antibodies. [5] In our case, ANA was positive in 1/1000 granular pattern; anti-topoisomerase I, anti-centromere, and anti RNA polymerase III were negative, and also regarding antiphospholipid antibodies, anti-cardiolipin IgG and IgM, anti-beta 2 glycoprotein IgA, IgM, and IgG were negative as well as lupus anticoagulant. When evaluated clinically, features related to kidney function were not helpful in predicting the renal crisis.…”

“…DcSSc was diagnosed in 57.1% of these patients, lcSSc in 31.4%, and overlap syndromes in 11.4%. [5] Pre-vious studies noted that patients with dcSSc had a higher risk of up to 25% with SRC. [6] SRC was observed in only 1% of patients with lcSSc.…”

Renal Involvement in Systemic Sclerosis: An Update

“…Rapidly progressive skin thickening appears to be the main risk factor for SRC, with most dcSSc patients developing SRC within 7.5 months to 4 years of disease onset [10, 11, 42]. According to Moinzadeh et al [43], development of proteinuria and/or hypertension, as well as positive anti-RNA polymerase III (anti-RNAP III) antibodies are the strongest risk factors for SRC. Terras et al [44] and Stochmal et al [45] showed that the presence of anti-RNAP III antibodies in patients with SSc correlates with renal crisis and severe cutaneous involvement.…”