Citations

Objective Systemic sclerosis (SSc) is an autoimmune disease marked by immune dysregulation, leading to progressive fibrosis and multiorgan dysfunction. Autologous hematopoietic cell transplantation (autoHCT) has demonstrated superiority over conventional immunosuppression in randomized trials yet remains underused in the United States. We report outcomes following implementation of autoHCT as a standard‐of‐care strategy at our institution. Methods We performed a retrospective analysis of consecutive patients with severe SSc undergoing autoHCT between April 1, 2020, and December 31, 2023. Eligibility required progressive disease despite standard immunosuppressive therapy. Primary endpoints were overall survival (OS) and event free survival (EFS). Secondary endpoints included cumulative incidence of relapse and nonrelapse mortality (NRM), organ‐specific outcomes, and transplant‐related complications. Results Twenty‐five patients underwent autoHCT. Median age was 46 years; 80% were female. At a median follow‐up of 23 (interquartile range 16.4–31.9) months, two‐year OS was 91.67% (95% confidence interval [CI] 81.25–100.0) and EFS was 74.51% (95% CI 58.78–94.45). Two‐year cumulative incidence of relapse was 23.93% (95% CI 4.27 –43.59), and NRM was 8.33% (95% CI 2.97–19.64). Significant improvement in Modified Rodnan Skin Score was observed at day +90 and +180 ( P < 0.05), whereas pulmonary function stabilized. Infectious complications occurred in 56% of patients, primarily viral reactivations. Conclusion Implementation of autoHCT for severe SSc in a multidisciplinary care model was associated with favorable survival and meaningful improvement in skin disease, with acceptable toxicity. Earlier referral and optimization of conditioning strategies may further reduce relapse and treatment‐related morbidity.

Background: Older adults with severe aortic stenosis (AS) are at high risk of mortality unless treated by aortic valve replacement (AVR). The Society of Thoracic Surgery (STS) score is primarily used clinically to determine mortality risk from surgical AVR. Due to geriatric comorbidities unique to older adults, traditional methods of risk stratification with STS may have significant shortcomings. Administratively derived risk scores that have prognostic significance in the geriatric population may prove valuable. Methods: We reviewed patients at our hospital system with AS undergoing AVR (surgical and transcatheter) and administratively derived an Elder Risk Assessment (ERA) score from the health record. The ERA score is a composite of age, marital status, number of hospitalized days in previous 2 years, and comorbidities (diabetes, coronary artery disease (CAD), heart failure, stroke, pulmonary disease, cancer, and dementia). Kaplan-Meier method was used to estimate survival by ERA score tertile. Cox regression was used to determine association between ERA and survival after accounting for STS predicted risk. Results: There were a total of 228 patients that underwent AVR (TAVR: 59 (26.1 %); SAVR 167 (73.9%) and had ERA scores derived. The average age was 79.6 (SD 7.6) years) and there were 93 (40.8%) women. Patients were stratified by ERA score tertiles: low (≤6), mid (6-13), or high (≥14). Over a mean follow up of 5.8 (SD 3.1) years, lower ERA scores were associated with better long-term survival (p<0.001). After accounting for age, sex, and STS score, mid ERA (HR 1.61; 95% CI 1.00-2.59; p<0.001) and high (HR 2.90; 95% CI 1.79-4.69; p<0.001) were associated with mortality (reference: low ERA). For every 10-point increase in the ERA, there was a 2-fold increase in adjusted mortality risk (HR 2.14; 95% CI 1.58-2.90; p<0.001). Conclusions: Older adults undergoing AVR for severe AS have specific geriatric comorbidities that may be better characterized using the ERA score. This administratively derived score was independently associated with survival after accounting for the widely used STS risk score.

ABSTRACT Background The association between transjugular intrahepatic portosystemic shunt TIPS and waitlist outcomes in simultaneous liver‐kidney (SLK) transplant recipients remains unclear. Methods Between 2003 and 2023, we examined the scientific registry of transplant recipients. All adult candidates with overlapping waiting lists for liver and kidney transplants ( N = 15 087) were analyzed (entire cohort). Candidates without TIPS ( n = 13 980) and with ( n = 1107) were followed through November 30, 2023, and the following outcomes were analyzed: death, SLK transplant, liver‐ or kidney‐alone transplant, or removal from the list. The Aalen–Johansen method was used to calculate the cumulative incidence rates of the outcomes, where the outcomes were considered competing risks. Mixed effects Cox proportional hazard models were used to compare the outcomes by TIPS status. We separately analyzed dialysis‐dependent candidates ( n = 10 370). Results In the entire cohort, the 1‐year cumulative incidence of death in those without TIPS was higher than in those with (12.5% vs. 9%). In the dialysis‐dependent cohort, the 1‐year death incidence was 13.3% versus 8.8% in those without TIPS and those with, respectively. In the multivariable Cox proportional hazard models, TIPS was associated with a 29% lower mortality risk in the entire cohort (HR = 0.71, 95% CI: 0.60–0.84, p < 0.001) and a 33% lower risk of mortality in the dialysis‐dependent cohort. The likelihood of receiving SLK was lower for recipients with TIPS in the univariable analysis. However, this association was attenuated after adjustment. The causes of removal were similar irrespective of TIPS status in both cohorts. Conclusion In this large cohort of SLK candidates, those with TIPS had a better waitlist survival, irrespective of their dialysis requirements before transplantation. Prospective studies are needed to validate these findings.

BACKGROUND Rotational vertebral artery occlusion, or bow hunter’s syndrome (BHS), is a rare but clinically important cause of vertebrobasilar insufficiency. Extrinsic compression of the artery is usually caused by osteophytes, fibrous bands, or lateral disc herniation and typically occurs in the setting of anatomical variations, leading to dynamic compromise of the posterior circulation. Neoplastic causes of BHS are rare. OBSERVATIONS The authors present the case of a 72-year-old man with a left vertebral artery–dominant posterior circulation and isolated small right vertebral artery terminating in the posterior inferior cerebellar artery (PICA). He developed progressively worsening dynamic insufficiency of his right PICA circulation due to compression of the right vertebral artery at C2 from an intradural-extradural dumbbell-shaped lesion. This patient was successfully treated via surgical debulking of the lesion and cervical spine fixation and experienced no neurological deficits postoperatively. LESSONS This neoplastic change represents an exceedingly rare presentation for BHS, which warrants further study on its etiology and treatment. https://thejns.org/doi/10.3171/CASE24415

Background: Previous data suggested that the outcomes for liver-alone transplant recipients following transjugular intrahepatic portosystemic shunt (TIPS) insertion were comparable to those without TIPS. This study investigates the association between TIPS and outcomes among simultaneous liver–kidney (SLK) recipients in the United States. Methods: Utilizing the Scientific Registry for Transplant Recipients (SRTR) standard analysis file from 2003 to 2022, we examined 9717 adult SLK recipients, among whom 858 had undergone TIPS before transplantation. Kaplan–Meier curves were generated to assess recipient and death-censored liver and kidney graft survival. Mixed-effects Cox proportional hazard models were employed to analyze the association between TIPS and the outcomes of interest, where the transplant center was treated as a random effect. The models were adjusted for recipient age, sex, MELD score, diabetes, duration of listing, induction, steroid maintenance, hepatitis C status, donor age, donor sex, cold ischemia time, local vs. shipped organs, and allocation era. Results: Overall, the two groups were comparable, with minor differences. Notably, the median liver waiting time was significantly longer in the TIPS group compared to the non-TIPS group (4.1 vs. 2 months, p < 0.001). One-year rejection rates for liver and kidney allografts did not differ significantly between groups. Univariable Cox regression analyses demonstrated no association between TIPS and worse outcomes for recipient, liver, and kidney survival (p = 0.65, p = 0.22, and p = 0.54, respectively). TIPS did not emerge as a predictor of recipient or death-censored liver or kidney graft survival in multivariable models. Conclusion: In this extensive national cohort of SLK transplant recipients, pre-transplant TIPS was not linked to adverse outcomes for recipients or their allografts.