A multi-predictor model to predict risk of scleroderma renal crisis in systemic sclerosis: a multicentre, retrospective, cohort study

Abstract

Objective. Scleroderma renal crisis (SRC) is a life-threatening syndrome. The early identification of patients at risk is essential for timely treatment to improve the outcome. Therefore, it is of great interest to provide a personalised tool to predict risk of SRC in systemic sclerosis (SSc). Methods. We tried to set up a SRC prediction model based on the PKUPH-SSc cohort of 302 SSc patients. The least absolute shrinkage and selection operator (Lasso) regression was used to optimise disease features. Multivariable logistic regression analysis was applied to build a SRC prediction model incorporating the features of SSc selected in the Lasso regression. Then, a multi-predictor nomogram combining clinical characteristics was constructed and evaluated by discrimination and calibration, with further assessment by external validation in a validation cohort composed of 400 consecutive SSc patients from other 4 tertiary hospitals. Results. A multi-predictor nomogram for evaluating the risk of SRC was successfully developed. In the nomogram, four easily available predictors were contained, including disease duration <2 years, cardiac involvement, anaemia and corticosteroid >15mg/d exposure. The nomogram displayed good discrimination with an area under the curve (AUC) of 0.843 (95% CI: 0.797-0.882) and good calibration. High AUC value of 0.854 (95% CI: 0.690-1.000) could still be achieved in the external validation. The model is now available online for research use. Conclusion. The multi-predictor nomogram for SRC could be reliably and conveniently used to predict the individual risk of SRC in SSc patients, and be a step towards more personalised medicine.

Anemia Is an Indicator for Worse Organ Damage Trajectories in Patients with Systemic Sclerosis: A Retrospective Study

“…Several researchers have reported that the hemoglobin level was an independent risk factor for 3-to-5-year mortality in early dcSSc patients [ 36 ], in-hospital mortality [ 37 ], severe skin thickness [ 38 ], as well as higher healthcare costs related to organ damage [ 39 ] in SSc patients. In addition, patients with anemia also showed a more significant progression of organ damage during follow-up, which has been reported in our previous cohort study [ 20 ], and shows that anemia was a predictor for the renal involvement of SSc. In our results, baseline anemia was related to the progression of organ damage in the high-burden SCTC-DI group among lcSSc and dcSSc patients and in the low-burden SCTC-DI group of SSc patients with overlap syndrome.…”

“…We retrospectively identified two cohorts of patients who were diagnosed as SSc at the Department of Rheumatology and Immunology of Peking University Third Hospital (PKUTH) and Peking University People’s Hospital (PKUPH) from January 2001 to July 2021, as reported previously [ 20 ]. The Ethics Committee approved the study of Peking University Third Hospital ((2022) 142-01)) and Peking University People’s Hospital (2019PHB276-01).…”

The clinical characteristics and outcomes of patients with systemic sclerosis with myocardial involvement

“…Therefore, we should remain vigilant for MI in patients with positivity to anti-SCL70 antibody and wide skin sclerosis. The correlation between scleroderma renal crisis (SRC) and cardiac or myocardial involvement has been reported in previous studies [21,22]; severe myocardial damage resulted in reduced blood supply to the kidneys, thus aggravating renal ischemia.…”

Cirrhosis of the Liver: A Case Report and Literature Review of a Rare Case Presentation of Autoimmune Hepatitis With Systemic Sclerosis

“…Managing autoimmune hepatitis is difficult with diffuse cutaneous SSc patients, as these patients may develop a renal crisis with high-dose steroid therapy [ 19 , 20 ]. Future studies are required to determine the treatment outcome and complications of high-dose steroid therapy for combined autoimmune hepatitis and SSc.…”