Corrigendum to 'TNF-alpha Inhibition for the Treatment of Cardiac Sarcoidosis' Seminars in Arthritis and Rheumatism. 2020 Jun;50(3):546-552.
Seminars in arthritis and rheumatism
Strongyloides Hyperinfection After Immunosuppression in an Immigrant From El Salvador A Case for Early Diagnosis and Treatment
JCR-JOURNAL OF CLINICAL RHEUMATOLOGY
2021; 27 (4): E128-+
Ultrasound evaluation of the hands and wrists in patients with systemic sclerosis: Osteophytosis is a major contributor to tender joints.
Seminars in arthritis and rheumatism
2021; 51 (4): 735-740
Arachidonic acid-regulated calcium signaling in T cells from patients with rheumatoid arthritis promotes synovial inflammation.
2021; 12 (1): 907
OBJECTIVE: To evaluate the prevalence and clinical associations of ultrasound (US) findings of inflammatory arthritis and joint and soft tissue pathology in patients with systemic sclerosis (SSc).METHODS: The hands and wrists of 43 SSc patients and 35 age-balanced controls were evaluated by clinical exam and musculoskeletal US. Synovial and tenosynovial pathology were assessed using semi-quantitative Gray Scale (GS) and Power Doppler (PD) scoring. US evaluation for osteophytes, erosions, ulnar artery occlusion, and median nerve cross-sectional areas was performed. Tender joints (TJ), swollen joints (SJ), modified Rodnan skin score (mRSS), digital ulcers, contractures, and calcinosis were evaluated. Concordance between US and physical exam findings at each joint region were assessed, and associations between their severity were analyzed.RESULTS: TJs and SJs were present in 44.2% and 62.8% of SSc patients, respectively. Inflammatory arthritis, defined as having both GS>0 and PD>0, was observed in 18.6% of SSc patients and no controls. There was a high concordance by joint region between GS synovial hypertrophy and osteophytes (kappa=0.88) as well as TJs (kappa=0.72). SSc patients had more osteophytes compared to controls (48.8% vs 22.9%, p=0.018) as well as higher osteophyte severity (p=0.033).CONCLUSIONS: Despite a high percentage of tender and swollen joints, less than 20% of SSc patients met criteria for inflammatory arthritis on US. The high concordance of osteophytes with GS synovial hypertrophy and tender joints suggest that osteophytosis may be a significant contributor to joint pain in SSc patients.
View details for DOI 10.1016/j.semarthrit.2021.04.020
View details for PubMedID 34144383
The Lancet Rheumatology
2020; 2 (1): E10
Annals of the rheumatic diseases
Effects of COVID-19 on Informal Caregivers and the Development and Validation of a Scale in English and Spanish to Measure the Impact of COVID-19 on Caregivers.
Journal of applied gerontology : the official journal of the Southern Gerontological Society
Rheumatoid arthritis (RA) and psoriatic arthritis (PsA) are two distinct autoimmune diseases that manifest with chronic synovial inflammation. Here, we show that CD4+ T cells from patients with RA and PsA have increased expression of the pore-forming calcium channel component ORAI3, thereby increasing the activity of the arachidonic acid-regulated calcium-selective (ARC) channel and making T cells sensitive to arachidonic acid. A similar increase does not occur in T cells from patients with systemic lupus erythematosus. Increased ORAI3 transcription in RA and PsA T cells is caused by reduced IKAROS expression, a transcriptional repressor of the ORAI3 promoter. Stimulation of the ARC channel with arachidonic acid induces not only a calcium influx, but also the phosphorylation of components of the T cell receptor signaling cascade. In a human synovium chimeric mouse model, silencing ORAI3 expression in adoptively transferred T cells from patients with RA attenuates tissue inflammation, while adoptive transfer of T cells from healthy individuals with reduced expression of IKAROS induces synovitis. We propose that increased ARC activity due to reduced IKAROS expression makes T cells more responsive and contributes to chronic inflammation in RA and PsA.
View details for DOI 10.1038/s41467-021-21242-z
View details for PubMedID 33568645
Development and Evaluation of the 8-item Caregiver Self-Efficacy Scale (CSES-8).
To understand how the COVID-19 pandemic has affected caregivers, we assessed its perceived impact on caregiving through a new measure: the Caregiver COVID-19 Limitations Scale (CCLS-9), in Spanish and English. We also compared levels of caregiver self-efficacy and burden pre-COVID-19 and early in the pandemic. We administered surveys via internet to a convenience sample of caregivers in January 2020 (pre-pandemic, n = 221) and in April-June 2020 (English, n = 177 and Spanish samples, n = 144) to assess caregiver self-efficacy, depression, pain, and stress. We used the early pandemic surveys to explore the validity of the CCLS-9. The pre-COVID-19 survey and the April English surveys were compared to determine how the COVID-19 pandemic affected caregivers. The CCLS-9 had strong construct and divergent validity in both languages. Compared to pre-COVID-19, caregiver stress (p = .002) and pain (p = .009) were significantly greater early in COVID-19, providing evidence of its validity. COVID-19 added to caregiver stress and pain.
View details for DOI 10.1177/0733464820971511
View details for PubMedID 33143545
Development and validation of a rheumatologist satisfaction with practice scale: The rheumatologist satisfaction scale.
2019; 98 (48): e18114
This paper describes the development and evaluation of a short caregiving self-efficacy measure. The self-administered 8-item Caregiver Self-efficacy Scale (CSES-8) was developed to reflect components of typical caregiver-support interventions and to be practical for inclusion in future self-efficacy and caregiving research.We administered the CSES-8 in two samples: participants in an intervention for caregivers of persons with cognitive disabilities, and a voluntary on-line survey for caregivers of adults. We evaluated the completion rate, item-scale correlations, reliability, descriptive statistics, and preliminary construct validity of the CSES-8 in both samples, and sensitivity to change in the intervention sample.The intervention caregivers' sample (N=158) was 85% female (mean age=65 years). The on-line survey sample (N=138) was 90% female (mean age=78). In both samples, the CSES-8 had excellent internal-consistency reliability (0.89 and 0.88) and good distribution with sufficient variability to detect change. Test-retest reliability was good in the on-line sample (0.73). As evidence of construct validity, most hypotheses were confirmed in both samples. The CSES-8 was sensitive to change at 6 months for caregivers in the intervention program (p < .001).The CSES-8 is short, comprehensive with respect to common components of interventions to improve caregivers' quality of life, and sensitive to change. It can serve a useful role exploring mechanisms by which caregiver intervention studies work, and it can be helpful in examining whether self-efficacy mediates the effect of these intervention on various outcomes such as psychological well-being.
View details for DOI 10.1093/geront/gnaa174
View details for PubMedID 33146727
TNF-alpha inhibition for the treatment of cardiac sarcoidosis.
Seminars in arthritis and rheumatism
There is a paucity of succinct measures of physician satisfaction. As part of a Performance Improvement Project, we developed and piloted a simple questionnaire to determine rheumatologists satisfaction.Thirty 5 rheumatologists in the academic or private setting were sent opened-ended questions to determine the factors that made them satisfied or dissatisfied with respect to their rheumatology practice. From the responses we formed 14 questions 1 to 10 scale centering on satisfaction and dissatisfaction that was piloted in 30 rheumatologists and subsequently validated in 173 rheumatologists within the US and Latin America.Our combined sample included 173 rheumatologists (55 English and 118 Spanish-speaking respondents). The mean satisfaction for the combined sample was 6.92 (standard deviation=1.1, range 4.08-9.62). The strongest contributors to physician satisfaction were "Seeing interesting and challenging cases" (8.6?±?1.5) and "The ability to make a difference in patient's life" as well as "Establishing long term relationship with patients" (8.39?±?1.5). The strongest contributors to physician dissatisfaction were "Getting inappropriate referrals not in the scope of practice" (4.3?±?2.13) and "Time spent on documentation" (4.5?±?2.59). The scale had good reliability, relatively normal distribution, and little or no redundancy among items.A simple and practical questionnaire to measure physician satisfaction, in particular rheumatologists satisfaction, was developed, piloted and successfully validated on a predominately academic sample of rheumatologists within the US and Latin America. This scale will serve as a means to identifying potential barriers to the implementation of performance improvement projects in the practice of Rheumatology.
View details for DOI 10.1097/MD.0000000000018114
View details for PubMedID 31770236
Strongyloides Hyperinfection After Immunosuppression in an Immigrant From El Salvador: A Case for Early Diagnosis and Treatment.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases
Recurrent carpal tunnel syndrome associated with extension of flexor digitorum muscle bellies into the carpal tunnel: A case series.
Archives of plastic surgery
2018; 45 (5): 474?78
Tumor necrosis factor alpha (TNF-?) inhibitors are increasingly being used for treating refractory cardiac sarcoidosis. There is a theoretical risk, however, that these therapies can worsen heart failure, and reports on efficacy and safety are lacking.We conducted a retrospective review of all cardiac sarcoidosis patients seen at Stanford University from 2009 to 2018. Data were collected on patient demographics, diagnostic testing, and treatment outcomes.We identified 77 cardiac sarcoidosis patients, of which 20 (26%) received TNF-? inhibitor treatment. The majority were treated for progressive heart failure or tachyarrhythmia, along with worsening imaging findings. All TNF-? inhibitor treated patients demonstrated meaningful benefit, as assessed by changes in advanced imaging, echocardiographic measures of cardiac function, and prednisone use.A large cohort (n = 77) of cardiac sarcoidosis patients has been treated at Stanford University. Roughly one-fourth of these patients (n = 20) received TNF-? inhibitors. Of these patients, none had worsening heart failure and all saw clinical benefit. These results help support the use of TNF-? inhibitors for the treatment of cardiac sarcoidosis based on real-world evidence and highlight the need for future prospective studies.
View details for DOI 10.1016/j.semarthrit.2019.11.004
View details for PubMedID 31806154
Risk of hemorrhagic transformation after ischemic stroke in patients with antiphospholipid antibody syndrome.
While the success or failure of carpal tunnel release ultimately depends on the interplay of a wide array of factors, a broad understanding of the normal anatomy of the carpal tunnel accompanied by awareness of the possible variations of the individual structures that make up its contents is crucial to optimizing surgical outcomes. While anatomic variants such as extension of the flexor digitorum muscle bellies have been described as a cause of primary carpal tunnel syndrome (CTS), there have been no reports depicting its association with recurrent CTS following initially successful carpal tunnel release, a finding with potentially significant prognostic implications that can aid in operative planning. In such cases where muscle extension is identified preoperatively, careful debulking of the muscle belly may be beneficial in improving long-term surgical outcomes.
View details for PubMedID 30282420
View details for PubMedCentralID PMC6177626
Bilateral Giant Cell Arteritis Presenting as Bilateral Sudden Vision Loss.
2017; 81 (4): 235?36
Several rheumatologic conditions including systemic lupus erythematosus, antiphospholipid antibody (APS) syndrome, rheumatoid arthritis, and scleroderma are known risk factors for stroke. The risk of hemorrhagic transformation after an acute ischemic stroke (AIS) in these patients is not known.We queried the Nationwide Inpatient Sample (NIS) data between 2010 and 2012 with ICD 9 diagnostic codes for AIS. The primary outcome was the development of hemorrhagic transformation. Multivariate predictors for hemorrhagic transformation were identified with a logistic regression model. Using SAS 9.2, Survey procedures were used to accommodate for hierarchical two stage cluster design of NIS.APS (OR 2.57, 95% CI 1.14-5.81, p = 0.0228) independently predicted risk of hemorrhagic transformation in multivariate regression analysis. Similarly, in multivariate regression models for the outcome variables of total charges of the hospitalization and length of stay (LOS), patients with APS had the highest charges ($56,286, p = 0.0228) and LOS (3.87 days, p = 0.0164) compared to other co-variates. Univariate analysis showed increased mortality in the APS compared to the non-APS group (11.68% vs. 7.16%, p = 0.0024).APS is an independent risk factor for hemorrhagic transformation in both thrombolytic and non-thrombolytic treated patients. APS is also associated with longer length and cost of hospital stay. Further research is warranted to identify the unique risk factors in these patients to identify strategies to reduce the risk of hemorrhagic transformation in this subgroup of the population.
View details for PubMedID 28475479
Cutaneous Vasculitis in a Patient with Antiphospholipid Antibody Syndrome.
2016; 80 (2): 75-79
Giant cell arteritis (GCA) is the most common form of primary vasculitis and it mainly involves large to medium sized vessels. It is also referred to as temporal arteritis as it primarily affects the temporal artery. Ocular involvement frequently occurs in GCA; if not promptly diagnosed, it can cause devastating ocular complications including complete vision loss and permanent blindness. In the majority of cases, it is unilateral; however, there are rare instances where bilateral ocular involvement is reported. In our report, we present the case of a patient presenting with bilateral sudden vision loss associated with GCA.
View details for PubMedID 29714410
Autosplenectomy Causing Catastrophic Pneumococcal Meningitis in a Patient with Lupus/Antiphospholipid Antibody Syndrome.
2016; 80 (1): 37-38
Antiphospholipid antibody syndrome (APS) is an acquired thrombophilia, caused by autoantibodies to anticardiolipin (aCL), or antibeta 2 glycoprotein I, or the presence of lupus anticoagulant (LA) in plasma. It is characterized by recurrent venous and/or arterial thrombi and/or pregnancy related morbidities. We present the case of a 52-year-old female with long-standing APS, who developed cutaneous vasculitis following a common cold. Most of the cutaneous manifestations of APS have been found to be thrombotic on histopathology without evidence of perivascular inflammation. Vasculitis is usually seen in APS patients with coexistent Systemic Lupus Erythematosus (SLE). However, our patient had evidence of vasculitis on skin biopsy and did not have SLE. Though rare, this is a disease process which must be considered in patients with primary APS which must be closely monitored for other vasculitic complications of APS, particularly diffuse alveolar hemorrhage.
View details for PubMedID 27024977
Adult Onset Henoch-Schönlein Purpura: Case Report and Review of Literature.
2015; 79 (2): 81-85
We present the case ofa26-year-old female who presented to the hospital with pneumococcal meningitis. A review of her records showed atrophic spleen, and a hypercoagulable workup was positive for Systemic Lupus Erythematous (SLE)/Antiphospholipid Antibody Syndrome (APS). An autosplenectomy from thrombotic occlusion of the splenic artery made her susceptible to pneumococcal meningitis. Autoimmune conditions, particularly SLE and APS, are important causes of hypercoagulable states in a young population, and earlier detection of these conditions and appropriate treatment helps to decrease morbidity and mortality among these patients.
View details for PubMedID 26882790
Efficacy and tolerability of pharmacotherapy options for the treatment of medullary thyroid cancer.
Clinical Medicine Insights. Oncology
2012; 6: 355-362
Henoch-Schönlein purpura (HSP) is an IgA mediated small-vessel vasculitis, more common in children than adults. We present the case of a 37-year-old male who presented with complaints of nausea, vomiting, abdominal pain, purpuric rash over lower extremities, and migratory polyarthralgia five days after being treated with antibiotics for bronchitis. In addition to the abdominal pain, he developed diarrhea and colonic biopsy findings were suggestive of inflammatory bowel disease (IBD). Skin biopsy revealed leukocytoclastic vasculitis with direct immunofluorescence studies (DIF) staining of IgA deposition confirming the diagnosis of HSP. The clinical features of cutaneous eruption with abdominal complaints can be seen with either HSP or IBD; however the specific skin biopsy findings on DIF can distinguish between the two disease processes. Though HSP is primarily seen in the pediatric population, it is a disease process that must be considered in adults presenting with vasculitic skin rashes and abdominal complaints.
View details for PubMedID 26244205
Metastatic and unresectable medullary thyroid carcinoma (MTC) is often difficult to treat as it is relatively unresponsive to radiation and conventional chemotherapy. This emphasizes the importance of the development of targeted therapies for advanced MTC. Vandetanib was approved by the US Food and Drug Administration for the treatment of symptomatic or progressive MTC in patients with advanced disease in April 2011. This therapy proved to be a breakthrough in the management of MTC. We review the efficacy and safety of this novel treatment and other treatments that are being evaluated in this disease.
View details for DOI 10.4137/CMO.S8305
View details for PubMedID 23133319