Introduction: This study aimed to develop a predictive model based on ultrasound variables which can be used to screen patients with psoriasis who are prone to progress to psoriatic arthritis (PsA) in clinical practice. Methods: This is a cross-sectional study conducted in a single center from October 2018 to
ObjectiveLight chain amyloidosis (AL) with cardiac involvement is associated with poor prognosis. The existing prognostic assessment system does not consider treatment-related factors, and there is currently no effective system for predicting the response. The purpose of this study was to build an individualized, dynamic assessment model for cardiac response and overall survival (OS) for AL patients with cardiac involvement.MethodsThe records of 737 AL patients with cardiac involvement were collected through cooperation with 18 hospitals in the Chinese Registration Network for Light-chain Amyloidosis (CRENLA). We used univariate and multivariate analyses to evaluate the prognostic factors for OS and cardiac response. Then, two nomogram models were developed to predict OS and cardiac response in AL patients with cardiac involvement.ResultsA nomogram including four independent factors from the multivariate Cox proportional hazards analysis—Mayo staging, courses of treatment, hematologic response, and cardiac response—was constructed to calculate the possibility of achieving survival by adding all the points associated with four variables. The higher the score, the more likely death would occur. The other nomogram model included the courses of treatment, hematological response, and different treatment regimens, and was correlated with cardiac response. The higher the score, the more likely a cardiac response would occur.ConclusionIn conclusion, based on the large Chinese cohort of patients with AL and cardiac involvement, we identified nomogram models to predict cardiac response and OS. These models are more individualized and dynamic, and therefore, they have important clinical application value.
Background: Light-chain (AL) amyloidosis frequently involves severe multiple end-organ damage, thus affecting prognosis. As the current disease staging system is based only on cardiac indicators, we propose a new staging system based on multiple organ indicators to supplement the existing system. Methods: Patients with AL amyloidosis (n=1,064) from 18 Chinese hospitals were enrolled and divided into test and validation cohorts (4:1). Multivariate analyses were performed to identify the clinical and laboratory factors for inclusion in the new staging system.Results: A score of 1 was assigned for each of the following-the difference between the involved and uninvolved free light chains ≥100 mg/L, estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m 2 , total bilirubin (Tbil) ≥18 µmol/L, cardiac troponin I ≥0.06 µg/L, and N-terminal pro-brain natriuretic peptide (NT-proBNP) ≥3,600 pg/mL-to divide the patients into five disease stages (0 to IV). There were 220 (20.7%), 291 (27.3%), 251 (23.6%), 178 (16.7%), and 124 (11.7%) patients with stage 0, I, II, III, and IV disease, respectively. Patients with stage II, III, and IV disease had a median overall survival (OS) of 56.9 months [95% confidence interval (CI), 33.9-not reached (NR)], 18.6 months (95% CI, 33.9-NR), and 6.5 months (95% CI, 8.0-24.6) (P<0.001), respectively. The 3-year survival estimates for patients with stages 0, I, II, III, and IV were 90.7%, 71.4%, 59.4%, 39.0%, and 22.1%, respectively.Conclusions: The new staging system has been developed that incorporates plasma cell-related characteristics in addition to cardiac, renal, and hepatic function parameters. It enhances the risk stratification of patients with AL amyloidosis and is useful when multiple organs are involved.
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