scholarly journals POS1439 A MULTI-PREDICTOR MODEL TO PREDICT RISK OF SCLERODERMA RENAL CRISIS IN SYSTEMIC SCLEROSIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1004.1-1004
Author(s):  
D. Xu ◽  
R. Mu

Background: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[1].Objectives:We aimed to provide a personalized 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 optimize 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.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, anemia 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.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 personalized medicine.References:[1]Woodworth TG, Suliman YA, Li W, Furst DE, Clements P (2016) Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol 12 (11):678-91.Disclosure of Interests:None declared

2016 ◽  
Vol 12 (11) ◽  
pp. 678-691 ◽  
Author(s):  
Thasia G. Woodworth ◽  
Yossra A. Suliman ◽  
Wendi Li ◽  
Daniel E. Furst ◽  
Philip Clements

2020 ◽  
Vol 45 (4) ◽  
pp. 532-548
Author(s):  
Magdalena Chrabaszcz ◽  
Jolanta Małyszko ◽  
Mariusz Sikora ◽  
Rosanna Alda-Malicka ◽  
Anna Stochmal ◽  
...  

Background: Systemic sclerosis is an immune-mediated rheumatic disease characterized by vascular abnormalities, tissue fibrosis and autoimmune phenomena. Summary: Renal disease occurring in patients with systemic sclerosis may have a variable clinicopathological picture. The most specific renal condition associated with systemic sclerosis is scleroderma renal crisis, characterized by acute onset of renal failure and severe hypertension. Although the management of scleroderma renal crisis was revolutionized by the introduction of angiotensin-converting enzyme inhibitors, there is still a significant proportion of patients with poor outcomes. Therefore, research on establishing disease markers (clinical, ultrasonographical and serological) and clear diagnostic criteria, which could limit the risk of developing scleroderma renal crisis and facilitate diagnosis of this complication, is ongoing. Other forms of renal involvement in systemic sclerosis include vasculitis, an isolated reduced glomerular filtration rate in systemic sclerosis, antiphospholipid-associated nephropathy, high intrarenal arterial stiffness and proteinuria. Key Messages: Scleroderma renal crisis is the most specific and life-threatening renal presentation of systemic sclerosis, albeit with declining prevalence. In patients with scleroderma renal crisis, it is mandatory to control blood pressure early with increasing doses of angiotensin-converting enzyme inhibitors, along with other antihypertensive drugs if necessary. There is a strong association between renal involvement and patients’ outcomes in systemic sclerosis; consequently, it becomes mandatory to find markers that may be used to identify patients with an especially high risk of scleroderma renal crisis.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1440.1-1440
Author(s):  
D. Xu ◽  
J. He ◽  
T. LI ◽  
R. Mu

Background:Scleroderma renal crisis (SRC) is a rare but life-threatening complication in SSc, which defined as malignant hypertension and progressive renal failure [1]. Previous reports of the prevalence for scleroderma renal crisis (SRC) show variable outcomes.Objectives:We aim to find out the prevalence for SRC in Chinese, and conduct a meta-analysis to assess the prevalence of SRC in worldwide and investigate possible factors of variability.Methods:We evaluated the prevalence for SRC and compared the clinical characteristics in SSc patients with or without SRC. A systematic review on the prevalence of SRC was carried out in PubMed, Embase and Cochrane. Meta-analysis was performed using available data on the prevalence for SRC.Results:302 patients were enrolled in PKUPH-SSc cohort and the prevalence of SRC was 4.97%. In our cohort, SRC was found in 3.60% limited cutaneous SSc (lcSSc) and 7.75% diffused cutaneous SSc (dcSSc) patients. Twenty-nine studies including a total population of 22686 SSc patients were analyzed in the meta-analysis. The overall pooled prevalence of SRC was 4.0%, but heterogeneity among the studies was substantial (I2=86.1%, P <0.00001). Meta-regression revealed that subtype of SSc and geographic factors partially explained this heterogeneity. Sub-group analyses demonstrated that the prevalence of SRC in dcSSc and lcSSc were 9.0% and 2.0%. The SRC prevalence in SSc was 3.0% in Asia, 5.0% in Europe, and 3.0% in North America, respectively.Conclusion:Data on our new cohort showed the prevalence of SRC was around 5% in China, and meta-analysis confirmed that the prevalence of SRC varies among centers. The marked heterogeneity of SRC prevalence was partially explained by subtype of SSc and geographic origin of patients.References:[1]Woodworth TG, Suliman YA, Li W, Furst DE, Clements P (2016) Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol 12 (11):678-91.Disclosure of Interests:None declared


2014 ◽  
Vol 53 ◽  
pp. 203-205 ◽  
Author(s):  
Doug Redd ◽  
Tracy M. Frech ◽  
Maureen A. Murtaugh ◽  
Julia Rhiannon ◽  
Qing T. Zeng

2018 ◽  
Vol 14 (2) ◽  
pp. 137-137 ◽  
Author(s):  
Thasia G. Woodworth ◽  
Yossra A. Suliman ◽  
Wendi Li ◽  
Daniel E. Furst ◽  
Philip Clements

2021 ◽  
Vol 8 (3) ◽  
pp. 4
Author(s):  
Gülşah Boz ◽  
Samet Karahan ◽  
Nurdan Yüksek

Background: Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin and organs, marked changes in microvascular structure, cellular and humoral immune disorders. Renal involvement is more frequent and mainly characterized by moderate proteinuria, elevated serum creatinine levels, and hypertension. The most common kidney involvement in SSc is scleroderma renal crisis (SRC) that is fatal without prompt intervention.Case report: A 52-year-old Caucasian male with known diffuse cutaneous systemic sclerosis was hospitalized with communityacquired pneumonia. On the fifth day after appropriate antibiotic therapy and 60 mg/day methylprednisolone, decreased urine output, arterial hypertension, decreased renal function and pulmonary edema developed. The patient was diagnosed with a scleroderma renal crisis. Emergency hemodialysis was applied to the patient, and captopril 6 × 25 mg/day and nifedipine 2*60 mg/day treatment were given. He received a routine hemodialysis program for about three months. The hemodialysis program was terminated when the patient’s urine quality and quantity increased.Conclusions: SRC, characterized by malignant hypertension, azotemia, microangiopathic hemolytic anemia, and kidney failure, is one of the most important complications of systemic sclerosis with a poor prognosis without prompt intervention. Steroid use is one of the important risk factors that precipitate SRC development. With angiotensin-converting enzyme inhibitors, survival increased after SRC, the need for dialysis decreased, and usually allowed the discontinuation of dialysis treatment within about 6-18 months. Suspicion of SRC in the presence of the above-mentioned findings in patients with a diagnosis or suspected systemic sclerosis can be considered the most important treatment step.


2021 ◽  
Vol 15 ◽  
Author(s):  
Lina Zhao ◽  
Yunying Wang ◽  
Zengzheng Ge ◽  
Huadong Zhu ◽  
Yi Li

Objective: The study aims to develop a mechanical learning model as a predictive model for predicting the appearance of sepsis-associated encephalopathy (SAE).Materials and Methods: The prediction model was developed in a primary cohort of 2,028 sepsis patients from June 2001 to October 2012, retrieved from the Medical Information Mart for Intensive Care (MIMIC III) database. Least absolute shrinkage and selection operator (LASSO) regression model was used for data dimension reduction and feature selection. The model was developed using multivariable logistic regression analysis. The performance of the nomogram has been evaluated in terms of calibration, discrimination, and clinical utility.Results: There were nine particular features in septic patients that were significantly associated with SAE. Predictors of individualized prediction nomograms included age, rapid sequential evaluation of organ failure (qSOFA), and drugs including carbapenem antibiotics, quinolone antibiotics, steroids, midazolam, H2-antagonist, diphenhydramine hydrochloride, and heparin sodium injection. The area under the curve (AUC) was 0.743, indicating good discrimination. The prediction model showed calibration curves with minor deviations from the ideal predictions. Decision curve analysis (DCA) suggested that the nomogram was clinically useful.Conclusion: We propose a nomogram for the individualized prediction of SAE with satisfactory performance and clinical utility, which could aid the clinician in the early detection and management of SAE.


Cancers ◽  
2021 ◽  
Vol 13 (11) ◽  
pp. 2808
Author(s):  
Tzong-Yun Tsai ◽  
Jeng-Fu You ◽  
Yu-Jen Hsu ◽  
Jing-Rong Jhuang ◽  
Yih-Jong Chern ◽  
...  

(1) Background: The aim of this study was to develop a prediction model for assessing individual mPC risk in patients with pT4 colon cancer. Methods: A total of 2003 patients with pT4 colon cancer undergoing R0 resection were categorized into the training or testing set. Based on the training set, 2044 Cox prediction models were developed. Next, models with the maximal C-index and minimal prediction error were selected. The final model was then validated based on the testing set using a time-dependent area under the curve and Brier score, and a scoring system was developed. Patients were stratified into the high- or low-risk group by their risk score, with the cut-off points determined by a classification and regression tree (CART). (2) Results: The five candidate predictors were tumor location, preoperative carcinoembryonic antigen value, histologic type, T stage and nodal stage. Based on the CART, patients were categorized into the low-risk or high-risk groups. The model has high predictive accuracy (prediction error ≤5%) and good discrimination ability (area under the curve >0.7). (3) Conclusions: The prediction model quantifies individual risk and is feasible for selecting patients with pT4 colon cancer who are at high risk of developing mPC.


2021 ◽  
Vol 16 (S2) ◽  
Author(s):  
Eric Hachulla ◽  
Christian Agard ◽  
Yannick Allanore ◽  
Jerome Avouac ◽  
Brigitte Bader-Meunier ◽  
...  

AbstractSystemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis.


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