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BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that requires frequent clinic and laboratory visits. However, patients with SLE, particularly those that are under-resourced, have unacceptably high rates of no-shows. OBJECTIVE: The objective of this study was to determine no-show rates associated with telemedicine visits during the COVID-19 pandemic in comparison to no show rates associated with contemporaneous and historic in-person. METHODS: We performed a retrospective cohort study in a publicly funded county hospital system in Houston, Texas. We identified a cohort of established SLE patients by International Classification of Diagnosis (ICD) codes that were independently confirmed as SLE by review of medical records. We identified patients who were seen from March to December in 2018, 2019, and 2020 (to reflect the height of the COVID-19 pandemic and account for seasonal changes in disease activity). Our primary outcome was percentage of no-shows for rheumatology clinic appointments. Our secondary outcome was laboratory utilization adherence, which was defined as lupus-specific blood and urine studies conducted within 30 days of the scheduled appointment. Covariates included age, gender, race, ethnicity, and SLE-related prescription drugs. RESULTS: We included 156 SLE patients in our analysis. Most were female (90.4%), Hispanic (49.3%) and had a median age of 43. In 2020, the no-show rate for telemedicine was 5.5% compared to a no-show rate of 16.2% for in-person visits (p=0.002). After multivariable adjustment for covariates, the odds of no-show was lower for telemedicine visits (OR 0.39, 95% CI 0.20-0.77). There were no differences in adherence to laboratory testing. CONCLUSIONS: Telemedicine visits had decreased odds of no-shows without difference in laboratory testing adherence after adjustment for covariates. More research is needed to determine the clinical impact of telemedicine on patients with SLE.
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OBJECTIVES: This study compared opioid prescribing among ambulatory visits with systemic autoimmune/inflammatory rheumatic diseases (SARDs) or without and assessed factors associated with opioid prescribing in SARDs. METHODS: This cross-sectional study used the National Ambulatory Medical Care Survey between 2006 and 2019. Adult (≥18 years) visits with a primary diagnosis of SARDs, including rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, or systemic lupus erythematosus were included in the study. Opioid prescribing was compared between those with vs without SARDs using multivariable logistic regression accounting for the complex survey design and adjusting for predisposing, enabling, and need factors within Andersen's Behavioral Model of Health Services Use. Another multivariable logistic regression examined the predictors associated with opioid prescribing in SARDs. RESULTS: Annually, an average of 5.20 million (95% confidence interval [CI] 3.58-6.82) visits were made for SARDs, whereas 780.14 million (95% CI 747.56-812.72) visits were made for non-SARDs. The SARDs group was more likely to be prescribed opioids (22.53%) than the non-SARDs group (9.83%) (adjusted odds ratio [aOR] 2.65; 95% CI 1.68-4.18). Among the SARDs visits, patient age from 50 to 64 (aOR 1.95; 95% CI 1.05-3.65 relative to ages 18-49) and prescribing of glucocorticoids (aOR 1.75; 95% CI 1.20-2.54) were associated with an increased odd of opioid prescribing, whereas private insurance relative to Medicare (aOR 0.50; 95% CI 0.31-0.82) was associated with a decreased odds of opioid prescribing. CONCLUSION: Opioid prescribing in SARDs was higher compared to non-SARDs. Concerted efforts are needed to determine the appropriateness of opioid prescribing in SARDs.
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OBJECTIVE: The objective is to determine cervical cancer screening rates and factors associated with decreased cervical cancer screening in women with systemic lupus erythematosus (SLE). METHODS: We conducted a cross-sectional study that enrolled consecutive women (age 21-64 years) with SLE. We collected demographics, clinical characteristics, constructs of the Health Beliefs Model (HBM) (ie, susceptibility, severity, barriers, benefits, cues to action, and self-efficacy), and self-reported cervical cancer screening (confirmed with the electronic medical record). The primary outcome was adherence to cervical cancer screening according to current guidelines. Multivariable logistic regression models were used to examine the association between SLE disease activity and cervical cancer screening and explore mediation effects from HBM constructs. RESULTS: We enrolled 130 women with SLE. The median age was 42 years (interquartile range 32-52 years). The cervical cancer screening adherence rate was 61.5%. Women with high SLE disease activity were less likely to have cervical cancer screening versus those with low disease activity (odds ratio 0.59, 95% confidence interval [CI] 0.39-0.89; P = 0.01), which remained statistically significant after adjusting for baseline demographics and drug therapy in a multivariable model (odds ratio 0.25, 95% CI 0.08-0.79; P = 0.02). Regarding the HBM constructs, increased perceived barriers to cervical cancer screening (r = -0.30, P < 0.01) and decreased self-efficacy (r = -0.21, P = 0.02) correlated with decreased cervical cancer screening. CONCLUSION: Patients with SLE with high disease activity undergo cervical cancer screening less frequently than those with low disease activity. Perceived barriers to cervical cancer screening are moderately correlated with decreased screening. These data highlight the need to develop strategies to increase cervical cancer screening in this high-risk patient population.
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OBJECTIVE: To define and select rheumatoid arthritis (RA)-specific core domain set for Longitudinal Observational Studies (LOS) within the Outcome Measures in Rheumatology (OMERACT) framework. METHODS: A three-round online Delphi exercise, including patient research partners (PRPs) and other community partners in healthcare, was conducted. Domains scored 7-9 (i.e., critically important to include) by ≥ 70 % of participants in both groups were included. Items were consolidated in a subsequent dedicated meeting. RESULTS: Nineteen domains scored ≥ 70 % consensus in both groups. The focus group refined these into a list of twelve domains. CONCLUSION: The achieved consensus will inform the next steps of developing the core domain set for LOS in RA.
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Artritis Reumatoide , Reumatología , Humanos , Consenso , Estudios Longitudinales , Evaluación de Resultado en la Atención de SaludRESUMEN
PURPOSE: To conduct a systematic review with meta-analysis to determine the effects of immunosuppression on Group 1 Pulmonary Arterial Hypertension in patients with systemic lupus erythematosus (SLE). METHODS: We searched Medline, Embase, Web of Science, Clinicaltrials.gov, and Cochrane Central Register of Controlled Trials (CENTRAL) with a search strategy developed by a medical librarian. We included retrospective, cross-sectional, case-control, prospective studies, and randomized controlled trials (RCTs) in our analysis and only included studies that contained data for patients with SLE. We included any immunosuppressive agents (including but not limited to cyclophosphamide, glucocorticoids, mycophenolate mofetil, azathioprine, and rituximab) We assessed for risk of bias and certainty of evidence. Outcomes included hemodynamics (as measured by pulmonary arterial hypertension), functional status, 6 minute walk test (6MWT), quality of life, mortality, and serious adverse events. RESULTS: We included three studies. One RCT and two single-arm interventional observational studies. The RCT had a high risk of bias whereas the two single-arm interventional studies were graded as fair quality. Meta-analysis could not be conducted because of insufficient data. The RCT showed significant improvements in hemodynamics (as measured by pulmonary arterial pressures) and functional status. One observational study showed improvements in hemodynamics, functional status, and 6MWT. There were insufficient data for serious adverse events, mortality, and quality of life. CONCLUSIONS: Despite a high prevalence and with a poor prognosis, there is a paucity of data for the role of immunosuppression in the treatment of Group 1 Pulmonary Arterial Hypertension in SLE. More high-quality studies are needed, especially to investigate serious adverse events and quality of life.
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Hipertensión Pulmonar , Lupus Eritematoso Sistémico , Hipertensión Arterial Pulmonar , Humanos , Inmunosupresores/efectos adversos , Lupus Eritematoso Sistémico/inducido químicamente , Terapia de Inmunosupresión , Estudios Observacionales como AsuntoRESUMEN
OBJECTIVES: We compared the outcomes of patients with or without systemic lupus erythematosus (SLE) who were diagnosed with coronavirus disease 19 (COVID-19) and evaluated factors within patients with SLE associated with severe outcomes. METHODS: This retrospective cohort study used the deidentified Optum COVID-19 electronic health record dataset to identify patients with COVID-19 from 1/1/2020 to 31/12/2020. Cases with SLE were matched with general controls at a ratio of 1:10 by age, sex, race and ethnicity and COVID-19 diagnosis date. Outcomes included 30-day mortality, mechanical ventilation, hospitalisation and intensive care unit admission. We evaluated the relationship between COVID-19-related outcomes and SLE using multivariable logistic regression. In addition, within SLE cases, we examined factors associated with COVID-19 related outcomes, including disease activity and SLE therapy. RESULTS: We included 687 patients matched with 6870 controls. Unadjusted rates of outcomes for patients with SLE were significantly worse than for matched controls including mortality (3.6% vs 1.8%), mechanical ventilation (6% vs 2.5%) and hospitalisation (31% vs 17.7%) (all p<0.001). After multivariable adjustment, patients with SLE had increased risks of mechanical ventilation (OR 1.81, 95% CI 1.16 to 2.82) and hospitalisation (OR 1.32, 95% CI 1.05 to 1.65). Among patients with SLE, severe disease activity was associated with increased risks of mechanical ventilation (OR 5.83, 95% CI 2.60 to 13.07) and hospitalisation (OR 3.97, 95% CI 2.37 to 6.65). Use of glucocorticoids, mycophenolate and tacrolimus before COVID-19 was associated with worse outcomes. CONCLUSION: Patients with SLE had increased risk of severe COVID-19-related outcomes compared with matched controls. Patients with severe SLE disease activity or prior use of corticosteroids experienced worse outcomes.
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COVID-19 , Lupus Eritematoso Sistémico , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/diagnóstico , Estudios Retrospectivos , Prueba de COVID-19 , COVID-19/complicaciones , Inmunosupresores/uso terapéuticoRESUMEN
OBJECTIVE: Patients with cancer and systemic lupus erythematosus (SLE) may have worse outcomes than those without SLE, given their comorbidities. We examined survival in elderly women with breast cancer (BC) and SLE and hypothesized that survival would be decreased compared with women with BC but without SLE. METHODS: We identified patients with BC and SLE and patients with BC without SLE in the Texas Cancer Registry and Surveillance, Epidemiology, and End Results, linked to Medicare claims. Overall survival (OS) was estimated after matching (age and cancer stage) and in multivariable Cox proportional hazards models adjusting for other cancer characteristics, treatment, and comorbidities. Two additional cohorts of women without cancer with and without SLE were also studied. RESULTS: We identified 494 BC SLE cases and 145,517 BC non-SLE cases, of whom we matched 9,708. Women with SLE were less likely to receive radiation, breast conserving surgery, or endocrine therapy. The 8-year OS estimate for women with early BC (stages 0-II) with and without SLE was 52% (95% confidence interval [95% CI] 45%-59%) and 74% (95% CI 73%-75%), respectively. In the Cox multivariable model, BC and SLE had increased risk of death (hazard ratio [HR] 1.65, 95% CI 1.38-1.98). Women with BC and SLE also had increased risk of death compared with women with SLE but without cancer (HR 1.42, 95% CI 1.05-1.92) after adjusting for SLE severity. Women with SLE and BC received less glucocorticoids, antimalarials, and immunosuppressants after cancer diagnosis than those without cancer. CONCLUSION: Systemic lupus is a risk factor for increased mortality in women with early BC.
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Neoplasias de la Mama , Lupus Eritematoso Sistémico , Humanos , Femenino , Anciano , Estados Unidos/epidemiología , Neoplasias de la Mama/terapia , Medicare , Incidencia , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/terapia , Lupus Eritematoso Sistémico/epidemiología , Factores de Riesgo , Modelos de Riesgos ProporcionalesRESUMEN
There are differences in recommendations for the management of immune-related adverse events (irAEs) associated with immune checkpoint inhibitors (ICIs). To assess the real-world management of irAEs, three surveys regarding ICI-induced hepatitis (IIH), renal irAEs, and myositis were developed and sent to experts in each area. Fifty-six surveys were completed (17 IIH, 20 renal irAEs, and 19 myositis). All experts agreed on performing imaging in every suspected case of severe IIH. Sixty-five percent agreed on performing a liver biopsy in patients not responding to corticosteroids. The most common indication for corticosteroid use (59%) was for severe IIH not improving after discontinuation of ICIs. Additionally, 60% of the experts agreed on performing a biopsy for stage 2/3 acute kidney injury (AKI), and 70% recommended imaging for any stage of AKI. Thirty-five percent favored corticosteroids in AKI patients with creatinine levels 2-3-fold above baseline. For myositis, 58% would recommend a muscle biopsy in a patient with weakness and creatine kinase levels of 5000 U/L; 47% would also opt for an endomyocardial biopsy when the troponin levels are increased. Fifty-eight percent recommended oral corticosteroids for myositis, and 37% recommended additional therapy, mainly immunoglobulins. These results show substantial differences in expert practice patterns for the management of severe liver, kidney, and muscular irAEs.
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The emergence of checkpoint inhibitors has created a paradigm shift for the treatment of various malignancies. However, although these therapies are associated with improved survival rates, they also carry the risk of immune-related adverse events (irAEs). Moderate to severe irAEs are typically treated with glucocorticoids, sometimes with the addition of immunosuppressants as steroid-sparing therapy. However, it is unclear how glucocorticoids and immunosuppressants may impact cancer survival and the efficacy of immune checkpoint therapy on cancer. In this narrative review, we discuss the effects of glucocorticoids and immunosuppressants including methotrexate, hydroxychloroquine, azathioprine, mycophenolate mofetil, tumor-necrosis factor (TNF)-inhibitors, interleukin-6 inhibitors, interleukin-1 inhibitors, abatacept, rituximab, and Janus kinase inhibitors (JAKi) on cancer-specific outcomes in the setting of immune checkpoint inhibitor use.
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BACKGROUND: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by systemic inflammation, fibrosis, vascular injury, reduced quality of life, and limited treatment options. Autologous hematopoietic stem cell transplantation (HSCT) has emerged as a potential intervention for severe SSc refractory to conventional treatment. OBJECTIVES: To assess the benefits and harms of autologous hematopoietic stem cell transplantation for the treatment of systemic sclerosis (specifically, non-selective myeloablative HSCT versus cyclophosphamide; selective myeloablative HSCT versus cyclophosphamide; non-selective non-myeloablative HSCT versus cyclophosphamide). SEARCH METHODS: We searched for randomized controlled trials (RCTs) in CENTRAL, MEDLINE, Embase, and trial registries from database insertion to 4 February 2022. SELECTION CRITERIA: We included RCTs that compared HSCT to immunomodulators in the treatment of SSc. DATA COLLECTION AND ANALYSIS: Two review authors independently selected studies for inclusion, extracted study data, and performed risk of bias and GRADE assessments to assess the certainty of evidence using standard Cochrane methods. MAIN RESULTS: We included three RCTs evaluating: non-myeloablative non-selective HSCT (10 participants), non-myeloablative selective HSCT (79 participants), and myeloablative selective HSCT (36 participants). The comparator in all studies was cyclophosphamide (123 participants). The study examining non-myeloablative non-selective HSCT had a high risk of bias given the differences in baseline characteristics between the two arms. The other studies had a high risk of detection bias for participant-reported outcomes. The studies had follow-up periods of one to 4.5 years. Most participants had severe disease, mean age 40 years, and the duration of disease was less than three years. Efficacy No study demonstrated an overall mortality benefit of HSCT when compared to cyclophosphamide. However, non-myeloablative selective HSCT showed overall survival benefits using Kaplan-Meier curves at 10 years and myeloablative selective HSCT at six years. We graded our certainty of evidence as moderate for non-myeloablative selective HSCT and myeloablative selective HSCT. Certainty of evidence was low for non-myeloablative non-selective HSCT. Event-free survival was improved compared to cyclophosphamide with non-myeloablative selective HSCT at 48 months (hazard ratio (HR) 0.34, 95% confidence interval (CI) 0.16 to 0.74; moderate-certainty evidence). There was no improvement with myeloablative selective HSCT at 54 months (HR 0.54 95% CI 0.23 to 1.27; moderate-certainty evidence). The non-myeloablative non-selective HSCT trial did not report event-free survival. There was improvement in functional ability measured by the Health Assessment Questionnaire Disability Index (HAQ-DI, scale from 0 to 3 with 3 being very severe functional impairment) with non-myeloablative selective HSCT after two years with a mean difference (MD) of -0.39 (95% CI -0.72 to -0.06; absolute treatment benefit (ATB) -13%, 95% CI -24% to -2%; relative percent change (RPC) -27%, 95% CI -50% to -4%; low-certainty evidence). Myeloablative selective HSCT demonstrated a risk ratio (RR) for improvement of 3.4 at 54 months (95% CI 1.5 to 7.6; ATB -37%, 95% CI -18% to -57%; RPC -243%, 95% CI -54% to -662%; number needed to treat for an additional beneficial outcome (NNTB) 3, 95% CI 2 to 9; low-certainty evidence). The non-myeloablative non-selective HSCT trial did not report HAQ-DI results. All transplant modalities showed improvement of modified Rodnan skin score (mRSS) (scale from 0 to 51 with the higher number being more severe skin thickness) favoring HSCT over cyclophosphamide. At two years, non-myeloablative selective HSCT showed an MD in mRSS of -11.1 (95% CI -14.9 to -7.3; ATB -22%, 95% CI -29% to -14%; RPC -43%, 95% CI -58% to -28%; moderate-certainty evidence). At 54 months, myeloablative selective HSCT at showed a greater improvement in skin scores than the cyclophosphamide group (RR 1.51, 95% CI 1.06 to 2.13; ATB -27%, 95% CI -6% to -47%; RPC -51%, 95% CI -6% to -113%; moderate-certainty evidence). The NNTB was 4 (95% CI 3 to 18). At one year, for non-myeloablative non-selective HSCT the MD was -16.00 (95% CI -26.5 to -5.5; ATB -31%, 95% CI -52% to -11%; RPC -84%, 95% CI -139% to -29%; low-certainty evidence). No studies reported data on pulmonary arterial hypertension. Adverse events In the non-myeloablative selective HSCT study, there were 51/79 serious adverse events with HSCT and 30/77 with cyclophosphamide (RR 1.7, 95% CI 1.2 to 2.3), with an absolute risk increase of 26% (95% CI 10% to 41%), and a relative percent increase of 66% (95% CI 20% to 129%). The number needed to treat for an additional harmful outcome was 4 (95% CI 3 to 11) (moderate-certainty evidence). In the myeloablative selective HSCT study, there were similar rates of serious adverse events between groups (25/34 with HSCT and 19/37 with cyclophosphamide; RR 1.43, 95% CI 0.99 to 2.08; moderate-certainty evidence). The non-myeloablative non-selective HSCT trial did not clearly report serious adverse events. AUTHORS' CONCLUSIONS: Non-myeloablative selective and myeloablative selective HSCT had moderate-certainty evidence for improvement in event-free survival, and skin thicknesscompared to cyclophosphamide. There is also low-certainty evidence that these modalities of HSCT improve physical function. However, non-myeloablative selective HSCT and myeloablative selective HSCT resulted in more serious adverse events than cyclophosphamide; highlighting the need for careful risk-benefit considerations for people considering these HSCTs. Evidence for the efficacy and adverse effects of non-myeloablative non-selective HSCT is limited at this time. Due to evidence provided from one study with high risk of bias, we have low-certainty evidence that non-myeloablative non-selective HSCT improves outcomes in skin scores, forced vital capacity, and safety. Two modalities of HSCT appeared to be a promising treatment option for SSc though there is a high risk of early treatment-related mortality and other adverse events. Additional research is needed to determine the effectiveness and adverse effects of non-myeloablative non-selective HSCT in the treatment of SSc. Also, more studies will be needed to determine how HSCT compares to other treatment options such as mycophenolate mofetil, as cyclophosphamide is no longer the first-line treatment for SSc. Finally, there is a need for a greater understanding of the role of HSCT for people with SSc with significant comorbidities or complications from SSc that were excluded from the trial criteria.
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Trasplante de Células Madre Hematopoyéticas , Esclerodermia Sistémica , Adulto , Ciclofosfamida/uso terapéutico , Humanos , Ensayos Clínicos Controlados Aleatorios como Asunto , Esclerodermia Sistémica/terapiaRESUMEN
OBJECTIVES: We conducted a systematic review with metanalysis to investigate the utility of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and procalcitonin (PCT) in diagnosing infections in hospitalized patients with SLE. METHODS: We searched Medline, Embase, Web of Science, ClinicalTrials.gov, and Cochrane Central Register of Controlled Trials (CENTRAL) with a search strategy developed by a medical librarian. We included retrospective, cross-sectional, case-control, and prospective studies in our analysis. We used the Quality Assessment of Diagnostic Studies (QUADAS-2) to assess for bias and applicability. We obtained mean differences, sensitivities, and specificities in our analysis. RESULTS: We included 26 studies in our analysis. Most studies had an unclear or high risk of bias and our results were widely heterogenous. For the diagnosis of infections, the CRP had a pooled sensitivity of 0.75 (95%CI 0.57-0.94) and specificity of 0.72 (0.59-0.85), PCT had a pooled sensitivity of 0.68 (95% CI 0.0.59-0.77) and specificity of 0.75 (0.59-0.90), and for ESR pooled estimates were not calculated but sensitivity ranged from 50 to 69.8 and specificity from 38.5 to 55.6. Modifying cut-offs improved sensitivities and specificities. The ESR, CRP, and PCT mean differences were all greater in infection groups versus non-infection (10.1, 95% CI 3.2-17.0; 46.8, 95% CI 36.5-57.0; 0.53, 95% CI 0.26-0.80; respectively). DISCUSSION: Poor sensitivities and specificities were observed for the evaluated biomarkers with substantial heterogeneity in the cut-offs used to determine infection. Although mean biomarker values were increased in the infection group compared with the non-infection, our findings do not support the widespread use of ESR, CRP, or PCT in diagnosing infection in hospitalized patients with SLE due to increased heterogeneity and risk of bias. Further investigation is needed.
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Lupus Eritematoso Sistémico , Polipéptido alfa Relacionado con Calcitonina , Biomarcadores , Sedimentación Sanguínea , Proteína C-Reactiva/análisis , Estudios Transversales , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Estudios Prospectivos , Estudios RetrospectivosRESUMEN
OBJECTIVES: Patients with systemic lupus erythematosus (SLE) have a high risk of cardiovascular disease that could potentially increase postoperative major adverse cardiac events (MACE). We determined the rate of MACE in patients with SLE undergoing noncardiac surgery using national claims-based data. METHODS: This was a retrospective cohort study using Optum Clinformatics Data Mart from 2007 to 2020. We identified a cohort of patients with SLE who had undergone noncardiac surgeries using Current Procedural Terminology codes. We also identified two control cohorts without SLE, one with diabetes mellitus (DM) and one without DM. After matching cases and controls by age and sex, the odds of MACE were estimated using multivariable logistic regression models also including race and the Revised Cardiac Risk Index (RCRI) scores. We also examined use of preoperative cardiac testing. RESULTS: We identified 4750 patients with SLE, 496,381 DM controls, and 1,484,986 non-DM controls. After matching, the odds ratio (OR) for MACE in patients with SLE versus non-DM controls was 1.51 (95% confidence interval 1.09-2.08), which decreased after adjustment for RCRI score (OR: 0.97, 95% confidence interval 0.7-1.36). No significant differences were observed in the incidence of MACE between patients with SLE and DM controls (0.82 vs 1.04, P = 0.16). High-risk patients with SLE (RCRI score of ≥3) were less likely to receive preoperative cardiac testing than non-DM controls (42.7% vs 35.1%, P < 0.05). CONCLUSION: Patients with SLE have an increased risk of postoperative MACE, which is driven by increased RCRI scores. Concerningly, high-risk patients received less cardiac testing 2 months before surgery than non-DM controls.
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Systemic lupus erythematosus (SLE) is a complex autoimmune disease with systemic clinical manifestations including, but not limited to, rash, inflammatory arthritis, serositis, glomerulonephritis, and cerebritis. Treatment options for SLE are expanding and the increase in our understanding of the immune pathogenesis is leading to the development of new therapeutics. Autoantibody formation and immune complex formation are important mediators in lupus pathogenesis, but an important role of the type I interferon (IFN) pathway has been identified in SLE patients and mouse models of lupus. These studies have led to the development of therapeutics targeting type I IFN and related pathways for the treatment of certain manifestations of SLE. In the current narrative review, we will discuss the role of type I IFN in SLE pathogenesis and the potential translation of these data into strategies using type I IFN as a biomarker and therapeutic target for patients with SLE.
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La displasia ósea esclerosante es una afectación en el desarrollo intrínseco del esqueleto, por alteración en la formación y modelado del hueso, que lleva a una excesiva acumulación ósea con un aumento de la densidad (esclero-sis). Existen varios tipos y todos ellos son de origen genético. Presentamos el caso de una paciente de 37 años que llega a la consulta sin diagnóstico previo, por dolor en miembros inferiores de larga evolución con reagudizaciones, asociado a deformidad e impotencia funcional, que cedía parcialmente con analgésicos comunes. (AU)
Bone sclerosing dysplasia is an affectation of the intrinsic development of the skeleton by an alteration in bone formation and modeling. It causes excessive bone accumulation with an increase in density (sclerosis). There are several types of bone sclerosing dysplasia. They are of genetic origin. We report here a 37 year-old patient without a previous diagnosis of sclerosing bone dysplasia who was seen in the clinic for pain in the lower limbs associated with bone deformity with only partial response to analgesics. (AU)
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Humanos , Femenino , Adulto , Enfermedades del Desarrollo Óseo/diagnóstico por imagen , Melorreostosis/diagnóstico por imagen , Imagen por Resonancia Magnética , Radiografía , Tomografía Computarizada Espiral , Manejo del Dolor , Cadera/patología , Pierna/patologíaRESUMEN
OBJECTIVES: To identify patient-centered core domains for prospective longitudinal observational studies (LOS) in rheumatoid arthritis. METHODS: Our working group held a virtual meeting in November 2020 to review data from a literature review and patient qualitative interviews, and to discuss strategies to move forward on domain identification and selection using the OMERACT 2.1 domain selection process. RESULTS: Important candidate domains and subdomains were identified including in the areas of life impact. Consensus was reached on moving forward with a Delphi process. CONCLUSIONS: The meeting provided future directions to identify and select a core set of domains for use in LOS.
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Artritis Reumatoide , Evaluación de Resultado en la Atención de Salud , Artritis Reumatoide/tratamiento farmacológico , Consenso , Humanos , Atención Dirigida al Paciente , Estudios ProspectivosRESUMEN
OBJECTIVE: To determine rates of cervical cancer screening and associated abnormal results in women with systemic lupus erythematosus (SLE). METHODS: We identified women with an initial diagnosis of SLE in the MarketScan Commercial Claims and Encounters Database from 2001 to 2014. Cervical cancer screening rates and associated diagnostic claims within 3 years of the initial claim were determined. Multivariable logistic regression was performed to evaluate the association of screening with lupus treatment. A matched logistic regression analysis was conducted to compare screening rates to those in age-matched women without connective tissue disease. RESULTS: We included 4,316 women with SLE. Screening rates were higher in women with SLE than in general controls (73.4% versus 58.5%; P < 0.001). Factors associated with decreased screening included recent time (odds ratio [OR] 0.70 [95% confidence interval (95% CI)] 0.55-0.89) (2012-2014 compared to 2001-2005), age ≥61 years (OR 0.27 [95% CI 0.18-0.39]), comorbidity score ≥2 (OR 0.71 [95% CI 0.6-0.83]), corticosteroid use (OR 0.77 [95% CI 0.61-0.97]), and use of immunosuppressants (OR 0.80 [95% CI 0.69-0.94]). Abnormal pathology result claims were more common in women with SLE than in general controls (12.3% versus 9.8%; P < 0.001). CONCLUSION: Though with higher rates than the general cohort, over 25% of the patients with SLE were not screened, and screening rates seem to be decreasing over time. Patients with SLE are at higher risk of abnormal cervical screening test results than controls, supporting the need for regular screening.
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Detección Precoz del Cáncer/estadística & datos numéricos , Lupus Eritematoso Sistémico , Neoplasias del Cuello Uterino/diagnóstico , Frotis Vaginal/estadística & datos numéricos , Adulto , Anciano , Estudios de Cohortes , Femenino , Humanos , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
Herein we describe the case of an elderly diabetic gentleman presenting with a two-week history of dyspnea and nonproductive cough, found to have a large left anterolateral chest wall mass. Further characterization through computed tomography (CT) of the chest revealed a soft tissue mass in the left anterior lower hemithorax found to be hepatocellular carcinoma (HCC). The liver, spleen, and pancreas were unremarkable. Diagnostic labs were unremarkable. The patient had no history of hepatitis, alcohol abuse, or illicit substance use. Pathological examination and immunohistochemical staining of the chest mass biopsy were consistent with metastatic hepatocellular carcinoma (HCC). The patient opted to pursue no further medical intervention and expired two weeks later. To the authors' knowledge, this is one of very few descriptions of isolated hepatocellular carcinoma found in the absence of a primary liver lesion and classical risk factors for hepatocarcinogenesis. This case highlights that HCC may present independently of liver lesions seen on imaging in a patient without clear signs or symptoms of liver. HCC should be considered in cases of isolated tumors with unclear primaries as ectopic carcinogenesis and occult primary malignancy are possibilities.
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CONTEXT: Although off-label medications are frequently prescribed in palliative care, there are no published studies examining their use in the U.S. OBJECTIVES: We examined the frequency of off-label medication use in cancer patients admitted to an acute palliative care unit (APCU). METHODS: This prospective observational study enrolled consecutive patients with advanced cancer admitted to the APCU of a tertiary care cancer center. We collected data on all prescription events, including indications for use, from admission to discharge. Off-label use was checked against the U.S. Food and Drug Administration-approved indications. RESULTS: Among the 201 patients, median survival was 10 days (95% CI 7-13), and 85 (42%) patients died in the APCU. We documented 6276 prescription events, and 2199 (35%) were off-label. Among off-label prescriptions, central nervous system agents (n = 1606, 73%), hormones and synthetic substitutes (n = 302, 14%), and autonomic drugs (n = 183, 8%) were most commonly prescribed. Haloperidol (n = 720, 33%), chlorpromazine (n = 292, 13%), dexamethasone (n = 280, 13%), glycopyrrolate (n = 175, 8%), hydromorphone (n = 161, 7%), and morphine (n = 156, 7%) were most frequently prescribed off-label. The most common indications for off-label prescribing were delirium (n = 783, 36%) and dyspnea (n = 449, 20%). Seventy percent of all off-label prescription events had strong evidence supporting use, and 19% of prescription events had moderate or weak evidence for use. CONCLUSION: One-third of prescription events in the APCU were off-label, with majority of off-label use having a strong level of supporting evidence. Our findings highlight the need for more research in key areas such as delirium and dyspnea management.
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Hospitalización , Uso Fuera de lo Indicado , Cuidados Paliativos , Adulto , Anciano , Anciano de 80 o más Años , Instituciones Oncológicas , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neoplasias/terapia , Estudios Prospectivos , Análisis de Supervivencia , Centros de Atención Terciaria , Adulto JovenRESUMEN
Patients with rheumatoid arthritis (RA) often have difficulties adhering to their medical treatment plans. We determined the characteristics of patients with RA who used reminders and the association between reminders and adherence. A total of 201 patients with RA were asked the frequency of reminders use such as pill containers, calendars, or diaries. Patients completed self-reported adherence questionnaires, and their disease activity and functional ability were measured. Sixty-eight patients (34 %) reported using a reminder. Factors associated with reminder use were older age (yes-mean age 54 vs no-mean age 49, p = 0.004), race (Whites-54 % vs Blacks-30 % vs Hispanics-26 %, p = 0.003), and sex (males-50 % vs females 28 %, p = 0.005). Working patients were less likely to use reminders (employed-21 % vs unemployed-43 %, p = 0.006). Use of calendars was associated with adherence while away from home (ρ = 0.16, p = 0.03), when busy (ρ = 0.16, p = 0.03), and use of any reminder was associated with adherence when running out of pills (ρ = 0.15, p = 0.04). The use of calendar reminders was associated with fewer tender joints (ρ = -0.17, p = 0.02). Few patients with RA used reminders, and whites, males and patients of increasing age were most likely to use reminders. Our findings show that reminders can assist patients with RA in taking medications, particularly when they are most prone to forgetting, such as when they are away from home or busy. Providers should encourage using reminders as a low-cost aid to enhance adherence.