Citations

Objective To provide evidence‐based and expert guidance for the treatment and management of non‐renal systemic lupus erythematosus (SLE); treatment and management of lupus nephritis are addressed in a separate guideline. Methods Clinical questions for treatment and management of SLE were developed in the PICO format (population, intervention, comparator, and outcome). Systematic literature reviews were developed for each PICO question, and the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology was used to assess evidence quality and formulate recommendations. The Voting Panel achieved a consensus of ≥70% agreement on the direction (for or against) and strength (strong or conditional) of each recommendation. Results We present recommendations and ungraded, consensus‐based good practice statements for the treatment and management of SLE that are applicable to pediatric and adult patients. Recommendations emphasize uniform treatment with hydroxychloroquine, limiting duration of glucocorticoid use, and early introduction of conventional and/or biologic immunosuppressive therapies to achieve and maintain control of SLE inflammation (remission or a low level of disease activity), reduce SLE‐related morbidity and mortality, and minimize medication‐related toxicities. Conclusion This guideline presents direction regarding treatment and management of SLE and provides a foundation for well‐informed, shared clinician–patient decision‐making. These recommendations should not be used to limit or deny access to therapies, as treatment decisions may vary due to the unique clinical situation and personal preferences of each person with SLE.

Importance The Breast Cancer Index (BCI) previously identified premenopausal patients with tumors in which the ratio of expression of HOXB13 relative to IL17BR (hereafter, BCI [H/I]–low tumors) as likely to derive greatest benefit from ovarian function suppression (OFS)–containing adjuvant therapy in the Suppression of Ovarian Function Trial (SOFT) trial. Objectives To assess BCI as a predictive biomarker of benefit from exemestane plus OFS vs tamoxifen plus OFS and to validate BCI as a prognostic biomarker for premenopausal patients. Design, Setting, and Participants This prognostic study used a prospective-retrospective translational design within the Tamoxifen and Exemestane (TEXT) and SOFT trials (enrolled November 2003 to April 2011). Blinded BCI testing in all available tumor samples was completed in March 2024. Premenopausal women with hormone receptor–positive breast cancer randomized to tamoxifen plus OFS or exemestane plus OFS who had BCI assessed were included. Analysis occurred from March to August 2024. Exposure 5 years of adjuvant tamoxifen plus OFS or exemestane plus OFS. Main Outcomes and Measures The primary outcomes were breast cancer-free interval (BCFI) for predictive analyses and distant recurrence-free interval (DRFI) for prognostic analyses after a median follow-up of 13 years in the TEXT cohort. Secondary objectives examined the predictive performance of BCI (H/I) in the combined TEXT and SOFT cohort overall and in prespecified clinical subgroups. Results Of 1782 patients in the TEXT study, 1034 (58.0%) had BCI (H/I)–low tumors; 915 (51.3%) of patients had N0 disease and 1077 (60.4%) were younger than 45 years. Patients with BCI (H/I)–low tumors had a 6.6% absolute benefit in 12-year BCFI (HR, 0.61; 95% CI, 0.44-0.85) for exemestane plus OFS vs tamoxifen plus OFS, while those with BCI (H/I)–high tumors had a 6.3% absolute benefit (HR, 0.78; 95% CI, 0.57-1.07; P for interaction = .29). Results were consistent in the combined TEXT plus SOFT cohort (2896 patients) and adjusting for clinicopathological variables. Clinical subgroup analyses consistently showed benefit of exemestane plus OFS vs tamoxifen plus OFS for BCI (H/I)–low tumors, and more variable relative treatment effects among BCI (H/I)–high tumors, including by age. Post hoc exploratory time-varying estimates suggested the treatment × BCI associations may differ in years 0 to 5 vs greater than 5 years. BCI and BCI N+ as continuous indices were prognostic for distant recurrence in N0 (HR, 1.27; 95% CI, 1.11-1.44; P < .001) and N1 (HR, 1.58; 95% CI, 1.21-2.05; P < .001) cancers. The 12-year DRFI was 96.3%, 90.3%, and 84.9% for BCI low-, intermediate-, and high-risk N0 cancers, respectively. Conclusions and Relevance In this study of premenopausal women with hormone receptor–positive breast cancer, BCI (H/I) status did not clearly predict greater benefit of adjuvant exemestane plus OFS vs tamoxifen plus OFS for women with BCI (H/I)–low tumors than for those with BCI (H/I)–high tumors; BCI continuous indices were reconfirmed as prognostic for premenopausal women. These findings support prior results of SOFT, which compared tamoxifen-alone vs OFS with either exemestane or tamoxifen, indicating premenopausal patients with BCI (H/I)–low tumors may benefit from more intensive endocrine therapy.

Introduction: Artificial intelligence (AI) and wider digital technologies are accelerating healthcare systems’ attempts to address the financial and moral imperatives of improving health disparities and thus wider population health outcomes. Yet adoption has thus far been slower in less common conditions including carcinoid, its syndrome, and related heart disease especially amid wider system shocks like the COVID-19 pandemic and its lingering effects. This is the first known AI-augmented nationally representative analysis of mortality and disparities for these associations. Methods: Quantum BAyesian Machine learning-augmented Propensity Score translational (QBAM-PS) statistics with multivariable regression was performed on the largest all payor US inpatient dataset, the National Inpatient Sample. Results: Of the 208,257,049 adult hospitalizations from 2016-2022, 124,954 (0.06%) had carcinoid tumors. Of the 71,402 hospitalizations with carcinoid from 2016-2019, 26,711 (37.41%) had metastases, 2,835 (3.97%) had acute heart failure, and 2,042 (2.86%) died. But of the 53,552 hospitalizations with carcinoid from 2020-2022, diagnosed metastases (38.04%) had increased, as did acute heart failure (4.21%) and mortality (3.73%), while 0.04% had diagnosed COVID. In all adult hospitalizations from 2016-2019 by QBAM-PS multivariable regression controlling for clinical confounders and severity, carcinoid and carcinoid syndrome did not significantly increase mortality, but carcinoid was significantly less likely to be diagnosed among Hispanics (OR 0.73) and Asians (OR 0.46) compared to Caucasians, in addition to Medicare (OR 0.71) and VA (OR 0.65) compared to private insurance, and more likely to be diagnosed in the highest versus lowest income quartile (OR 0.124) (all p<0.001). By 2020-2022, carcinoid and its syndrome did not significantly increase the odds of COVID diagnosis. But again carcinoid was significantly less likely to be diagnosed in Hispanics (OR 0.68), Asians (OR 0.61), Medicare (OR 0.78), and VA (OR 0.59), while the highest income again increased diagnosis odds (OR 1.19) (all p<0.01). There were no mortality disparities within carcinoid during either time period. Conclusions: This large multi-year analysis supports improvements in metastatic carcinoid detection and heart disease management, including through the COVID pandemic, though amid persistent racial, insurance, and income disparities.

Background: Adults with congenital heart disease (CHD) face ongoing physical, emotional, and psychosocial challenges that impact long-term outcomes. Research is limited on how their health topic interests vary by levels of empowerment. The 15-item Gothenburg Empowerment Scale (GES) assesses CHD patient empowerment across five domains. Objective: Examine the association between patient empowerment levels and patient-prioritized health education topics among adults with CHD. Methods: This cross-sectional study included adults (≥18) with self-reported CHD enrolled in the Empower My Congenital Heart digital study. Participants completed the GES survey and responded to open-ended questions regarding information of interest to them in managing their healthcare. GES scores were divided into tertiles, and responses were grouped into patient-prioritized topics. Descriptive analysis examined associations between empowerment levels and topic preferences. Results: Of 292 eligible patients, mean age was 45.6 ± 14.7 years, 91.4% were Caucasian, and 66.4% were female. The median (25th, 75th) GES score was 62 (57, 68). The most frequently observed patient prioritized topic was CHD health knowledge (73%), followed by interest in building CHD community (52.7%), lifestyle management (23.6%), health care navigation (7.2%), research and representation (5.1%). Participants in the lower and middle GES tertiles more frequently expressed interest in foundational sub-topics such as understanding CHD and surgical history, finding ACHD specialists, exercise and nutrition. In contrast, participants in the highest GES tertile prioritized sub-topics related to aging with CHD, managing menopause, CHD research and legislative activities. Peer support was a common topic across all groups (Figure 1). Patient quotes were stratified by tertile and patient prioritized health topics and subtopics as summarized in table 1. Conclusion: Adults with CHD prioritized varied health education topics depending on their baseline empowerment levels. Those with lower GES scores prioritized foundational information and guidance, while those with higher scores prioritized community-oriented and research-related topics. These findings could help tailor CHD patient-centered education strategies based on individual empowerment profiles.

Background: Digital health offers a potential solution to support adults with congenital heart disease (CHD), filling the critical gap to help maintain recommended lifelong care with adult CHD specialists. However, its design and implementation require a rigorous scientific approach to make it scalable and usable for the target population. Objective: To pilot test the acceptability and feasibility of a digital health intervention to support lifelong healthcare for adults with CHD. Methods: Using a community-based participatory research and a behavioral theory-based approach, we developed and piloted a digital tool, Empower My Congenital Heart (EmpowerMyCH), within the web—and mobile-based Eureka Digital Research platform at UCSF. Intervention components within EmpowerMyCH include exhibits (expert and peer-developed learning modules on CHD-related topics such as finding specialists or navigating visits, tests, procedures, etc.), peer support, and a digital medical passport (Figure 1). Participants receive an exhibit with study measure surveys every two months, with a two-month completion window. Participant satisfaction with the digital tool is measured at six months using the Client Satisfaction Questionnaire-8 (CSQ-8), a validated survey with scores ranging from 8 to 32; higher scores indicate higher satisfaction with the digital tool. Results: Of the 519 participants recruited, 495 were eligible adults with CHD, 445 consented, and 6 withdrew. The median age was 43 (ranging from 20 to 85) years, and 30.1% were males. Survey completion rate was the highest at 88.7% during the first two months after enrollment and 53.5% at 8 months. Of the five exhibits delivered so far, over 87% of participants rated the exhibits to be helpful (Figure 2). Participants found the exhibits readable, easily accessible, motivational, useful, and educational (Figure 3). The median (25th, 75th) CSQ-8 score was 21 (18, 25) among 144 participants who completed the survey. Conclusions: EmpowerMyCH, the digital health tool, appears to be acceptable to most adults with CHD to support and educate them in navigating their healthcare. The tool and its contents are accessible, user-friendly, and responsive to the needs of the CHD community, resulting in high satisfaction rates. The feasibility rates and satisfaction scores are similar to or higher for EmpowerMyCH than those of prior digital health studies. Future hybrid intervention trials can demonstrate EmpowerMyCH‘s effectiveness.

Background: It is currently unknown whether patient engagement in adult congenital heart disease (CHD) care is influenced by social determinants of health (SDOH) such as race/ethnicity, education, and income. Adapting from the chronic care model, patient engagement, defined as the knowledge, skills, ability, and willingness to manage one’s health, can minimize lapses in care and improve CHD care outcomes. Methods: This cross-sectional study analyzes data from patients aged 18 and older with a self-reported diagnosis of CHD enrolled in a digital health research study called Empower My Congenital Heart. Patient engagement was measured using the Gothenburg Empowerment Scale (GES). We compared mean GES scores across demographic categories (race/ethnicity, education, and income), using independent samples t-tests or one-way ANOVA as appropriate. Additionally, we conducted a multivariate linear regression analysis to evaluate the impact of race/ethnicity, education, and income on GES scores, adjusting for age, gender, marital status, parental status, and employment status. Results: Of 323 adult CHD patients, the mean age was 45.6 ±15 years, 32.5% were males, 89.9% were non-Hispanic White, 29.7% had less than a Bachelor's degree, and 43.5% had less than $100,000 annual household income. The mean GES score was 61.8 ± 8, with significantly higher scores observed for non-Hispanic White identifying individuals (p=0.006) and those with a Bachelor's or higher level of education (p=0.005), while no difference across income levels (p = 0.394) (Figure 1). Linear regression analysis demonstrated that race and ethnicity remained significantly associated with GES scores (Table 1). Conclusions: Race/ethnicity and education level (in the bivariate analysis only), but not income, are the SDOH variables significantly associated with how empowered a CHD patient is in managing their health care. Future interventions that target enhancing patient engagement and self-management skills should take into consideration the influence of these demographic and social factors.

Abstract Understanding tumor cell dynamics can improve prognosis and treatment but remains limited for lung adenocarcinoma in people who have never smoked (NS-LUAD). With RNA sequencing data from 684 NS-LUAD cases and validation in an independent dataset, we identified three subtypes with distinct phenotypic traits and cell compositions. Additional genomic and histologic data further characterized the subtypes. “Steady,” marked by low proliferation, high alveolar cell fraction, moderate-to-well differentiation, and fewer driver gene alterations, is linked to prolonged survival and low immune evasion. “Proliferative” shows high proliferation markers, TP53 mutations, and gene fusions. “Chaotic,” with high epithelial-to-mesenchymal transition markers, has the worst prognosis, even within stage I tumors. Lacking known molecular or histologic characteristics, this aggressive subtype is solely identified by transcriptomic data. A 60-gene signature recapitulates the classification and predicts survival even within subgroups based on tumor stage or known genomic features, emphasizing its potential for improving early-stage NS-LUAD prognostication in clinical settings. Significance: The transcriptome of 684 NS-LUAD identifies three subtypes with different cellular dynamics and genomic and morphologic features. A 60-gene signature accurately stratifies subjects for mortality risk, even in stage I, offering a potential clinically applicable tool for treatment decision-making in patients with NS-LUAD. See related commentary by Azizi et al., p. 423

ABSTRACT We conducted a USA claims‐based cohort study in 20,705 patients with inflammatory bowel diseases (IBD) on advanced therapies to estimate the overall and comparative risk of tuberculosis between advanced therapies using inverse probability of treatment weighted Cox proportional hazards model. The incidence rate (IR, per 100,000 person‐years) of tuberculosis with TNF antagonists ( n = 9378) and non‐TNF‐targeting biologics (anti‐integrins [ n = 5877], anti‐interleukins [ n = 5450]) was similar (38 [95% CI, 8–69] vs. 33[7–59]). Adjusting for covariates, risk of tuberculosis was similar (HR, 1.16 [0.41–3.31]). In low incidence regions like USA, the incidence of tuberculosis after starting advanced therapies for IBD is very low, and comparable across advanced therapies.

ABSTRACT The American Society for Apheresis (ASFA) Attending Physician Subcommittee of the Physicians' Committee performed an annual review of articles published in 2024 related to apheresis medicine. The 10 seminal apheresis articles selected by the subcommittee members are summarized in this review. PubMed was used to identify manuscripts published in 2024 in four areas of interest: donor apheresis, therapeutic apheresis, apheresis education, and apheresis for cellular therapy. Only full length, peer‐reviewed manuscripts in English with data from human subjects were included. Case reports, review articles, and meta‐analyses were excluded. Articles were considered seminal if they met at least one of the following previously established criteria: novel finding(s), practice‐altering outcomes, international in scope, randomized‐controlled trial, relevant to current clinical practice, and/or provide evidence for category III or IV indications based on the ASFA 9th special issue of the Guidelines on the Use of Therapeutic Apheresis in Clinical Practice‐Evidence‐Based Approach.