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The article focuses on a clinical case involving a 20-year-old woman presenting with inflammatory arthritis, alopecia, and weight loss. Topics include differential diagnosis of joint pain, considerations for systemic rheumatic diseases, and the impact of malnutrition exacerbating illness, leading to a diagnosis of kwashiorkor despite initial suspicion of systemic lupus erythematosus (SLE).

Patients with inflammatory arthritis (IA) are at increased risk of severe COVID-19 due to medication-induced immunosuppression that impairs host defenses. The aim of this study was to assess antibody and B cell responses to COVID-19 mRNA vaccination in IA patients receiving immunomodulatory therapies. Adults with IA were enrolled through the Johns Hopkins Arthritis Center and compared with healthy controls (HC). Paired plasma and peripheral blood mononuclear cell (PBMC) samples were collected prior to and 30 days or 6 months following the first two doses of mRNA vaccines (D2; HC=77 and IA=31 patients), or 30 days following a third dose of mRNA vaccines (D3; HC=11 and IA=96 patients). Neutralizing antibody titers, total binding antibody titers, and B cell responses to vaccine and Omicron variants were analyzed. Anti-Spike (S) IgG and S-specific B cells developed appropriately in most IA patients following D3, with reduced responses to Omicron variants, and negligible effects of medication type or drug withholding. Neutralizing antibody responses were lower compared to healthy controls after both D2 and D3, with a small number of individuals demonstrating persistently undetectable neutralizing antibody levels. Most IA patients respond as well to mRNA COVID-19 vaccines as immunocompetent individuals by the third dose, with no evidence of improved responses following medication withholding. These data suggest that IA-associated immune impairment may not hinder immunity to COVID-19 mRNA vaccines in most individuals. [ABSTRACT FROM AUTHOR]

Background: The emergence of new SARS-CoV-2 variants calls for more data on SARS-CoV-2 mRNA vaccine response. Aims: We aimed to assess the response to a third mRNA vaccine dose against SARS-CoV-2 in inflammatory bowel disease (IBD) patients. Methods: This was a single-center, observational prospective study of IBD patients who received a third mRNA vaccine dose against SARS-CoV-2. Antibody titers were taken post-third-dose at one and three months using the Roche Elecsys anti-SARS-CoV-2-S enzyme immunoassay. Titers less than 0.8 units/mL were considered negative according to the manufactures. Titers between 0.8 units/mL and 250 units/mL were considered non-neutralizing. Titers greater than 250 units/mL were considered neutralizing. Results: Eighty-three patients were included, all of whom had detectable antibodies at 3 months post-third dose. A total of 89% showed neutralizing and 11% non-neutralizing titers. Participants with non-neutralizing titers were more likely to be on systemic corticosteroids (p = 0.04). Two participants seroconverted from negative to positive, whereas 86% with non-neutralizing titers boosted to neutralizing levels. Only one participant with neutralizing titers after a third dose had a decrease to a non-neutralizing level within 3 months. Conclusions: Our findings support the ongoing recommendations for additional doses in immunocompromised individuals. However, longitudinal studies with a greater-sized patient population are needed. [ABSTRACT FROM AUTHOR]

Idiopathic inflammatory myopathies (IIMs) represent a diverse group of systemic autoimmune disorders with variable clinical manifestations and disease course. Currently, the challenges of IIMs are multifold, including difficulties in timely diagnosis owing to clinical heterogeneity, limited insights into disease pathogenesis, as well as a restricted number of available therapies. However, advances utilizing myositis-specific autoantibodies have facilitated the definition of subgroups as well as the prediction of clinical phenotypes, disease course, and response to treatment. Herein we provide an overview of the clinical presentations of dermatomyositis, anti-synthetase syndrome, immune-mediated necrotizing myopathy, and inclusion body myositis. We then provide an updated review of available and promising therapies for each of these disease groups. We synthesize current treatment recommendations in the context of case-based construct to facilitate application to patient care. Finally, we provide high-yield, clinical pearls relevant to each of the subgroups that can be incorporated into clinical reasoning. There are many exciting developments on the horizon for IIM. As insights into pathogenesis evolve, the therapeutic armamentarium is expanding with many novel therapies in development, holding promise for more targeted treatment approaches. [ABSTRACT FROM AUTHOR]

The article describes five cases of demartomyositis (DM) with bullous periorbital oedema identified at the Johns Hopkins Myositis Center, who were all positive for anti-p155/140, suggesting a novel clinical manifestation of this subgroup. Topics discussed include an overview of the case of each patient, condition which prompted concern for angioedema in two participants, and finding on bullous periorbital oedema.

Objective: Proteinuria is the clinical expression of lupus nephritis and despite recent advances in the therapeutic armamentarium for lupus nephritis, morbidity and mortality rates remain high. Therefore, the identification of factors that predict lupus nephritis is paramount in preventing damage accrual and disease progression. Lipoprotein (a) (Lp[a]) is a primarily genetically inherited plasma lipoprotein with pro-thrombotic and pro-atherosclerotic effects. Elevated Lp(a) has been observed at early stages of renal impairment in the general population and is associated with the development of chronic kidney disease. However, little is known about renal implications of Lp(a) in SLE. Thus, we evaluated Lp(a) and atherosclerotic events, thrombotic events, renal disease, and disease activity in patients with SLE. Methods: SLE patients fulfilling the revised American College of Rheumatology (ACR) or SLICC classification criteria with a measurement of Lp(a) were included in the analysis. A cutoff of 125 nmol/L was chosen based on expert opinion. Chi-square test was used to compare the differences between patient characteristics and Lp(a) levels. Logistic regression or linear regression were used, where appropriate, to assess the association between Lp(a) values and the measured outcomes. Results: Lp(a) levels from 562 patients were analyzed. There was an association between elevated Lp(a) and a history of proteinuria (OR 1.58, p -value = 0.02). This association remained significant following adjustment for age, sex, race, low C3, and elevated anti-dsDNA (OR = 1.55, p -value = 0.04). There was also an association with eGFR < 60 (p = 0.02). Patients with elevated Lp(a) had higher physician global activity (p = 0.01) and erythrocyte sediment rate (p = 0.03). Conclusion: Elevated Lp(a) was associated with proteinuria, independent of known factors associated with lupus proteinuria, as well as reduced eGFR and physician global activity. Our findings highlight the potential role of Lp(a) as a noninvasive biomarker for early renal disease in SLE. [ABSTRACT FROM AUTHOR]

The article presents a study which evaluated post-vaccination reactogenicity and antibody responses of immunosuppressed patients with autoimmune diseases following a fifth dose (D5). Topics discussed include mycophenolate mofetil (MMF) as the most common immunosuppressive therapy, most common post-vaccination reactions, and limitations of the study.

Objective: To evaluate disease flare and postvaccination reactions (reactogenicity) in patients with rheumatic and musculoskeletal diseases (RMDs) following 2‐dose SARS–CoV‐2 messenger RNA (mRNA) vaccination. Methods: RMD patients (n = 1,377) who received 2‐dose SARS–CoV‐2 mRNA vaccination between December 16, 2020 and April 15, 2021 completed questionnaires detailing local and systemic reactions experienced within 7 days of each vaccine dose (dose 1 and dose 2), and 1 month after dose 2, detailing any flares of RMD. Associations between demographic/clinical characteristics and flares requiring treatment were evaluated using modified Poisson regression. Results: Among the patients, 11% reported flares requiring treatment; there were no reports of severe flares. Flares were associated with prior SARS–CoV‐2 infection (incidence rate ratio [IRR] 2.09, P = 0.02), flares in the 6 months preceding vaccination (IRR 2.36, P < 0.001), and the use of combination immunomodulatory therapy (IRR 1.95, P < 0.001). The most frequently reported local and systemic reactions included injection site pain (87% after dose 1, 86% after dose 2) and fatigue (60% after dose 1, 80% after dose 2). Reactogenicity increased after dose 2, particularly for systemic reactions. No allergic reactions or SARS–CoV‐2 diagnoses were reported. Conclusion: Flares of underlying RMD following SARS–CoV‐2 vaccination were uncommon. There were no reports of severe flares. Local and systemic reactions typically did not interfere with daily activity. These early safety data can help address vaccine hesitancy in RMD patients. [ABSTRACT FROM AUTHOR]

The article discusses a study on the safety of third-dose (D3) SARS-CoV-2 vaccination in patients with rheumatic and musculoskeletal disease (RMD). Topics include the most common local and systemic reactions, limitations of the study, and characteristics of RMD patients, stratified by presence/absence of disease flare requiring treatment and multivariable analysis of factors associated with post-D3 flare.

Rituximab has been associated with worse outcomes in patients with RMDs and SARS-CoV-2 infection ([5]), and thus it is of further concern that these patients may not derive protection from vaccination. I Background: i Patients with rheumatic and musculoskeletal diseases (RMDs) are at increased risk for SARS-CoV-2 infection because of both the immunomodulatory effects of their underlying diseases and treatment with immunosuppressive agents. Thus, we sought to analyze a subset of 20 patients with RMDs who did not develop a detectable antibody response 1 month after completion of 2-dose mRNA vaccination against SARS-CoV-2. [Extracted from the article]

Immunotherapy has heralded the advent of a new era in oncology. Immune checkpoint inhibitors (ICIs) enhance anti-tumor immunity, thereby reinvigorating a patient's immune system to fight cancer. While therapy with this class of agents has resulted in improved clinical outcomes for patients with multiple tumor types, a broad spectrum of immune-related adverse events (irAEs) may affect any organ system, with variable clinical presentations. Prompt recognition and management of irAEs are associated with improved irAE outcomes, and represents an important new clinical challenge for practicing oncologists. Herein, we provide a comprehensive case-based review of the most common and clinically-important irAEs, focussing on epidemiology, clinical manifestations, and management. We also examine future strategies that may provide meaningful insights into the prevention and management of irAEs. [ABSTRACT FROM AUTHOR]

In the article, the authors present a case of a 39-year-old male with acute abdominal pain, vomiting and nausea to discuss segmental arterial mediolysis (SAM) as a rare cause of abdominal pain masquerading as vasculitis. His medical history include untreated hypertension and active tobacco and cannabis use. He was treated with anti-hypertensive drug and low-dose antiplatelet therapy.

Patients with inflammatory bowel disease (IBD) are recommended to receive vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), regardless of their immunosuppression status. Immunosuppressive medications represent a mainstay of therapy in moderate to severe IBD; however, their impact on the SARS-CoV-2 vaccine response remains unclear. Studies thus far have shown that patients with IBD on various therapies had detectable antibody responses after standard vaccinations.1-5 To date, one study has examined the kinetics of antibody response at 3 months after vaccination in patients with IBD, but data beyond this time point are not yet available.6 The aim of this study was to assess anti-spike antibody response 6 months after completion of standard SARS-CoV-2 vaccination in patients with IBD. Secondarily, we observed antibody kinetics over 6 months in a subset of patients post-vaccination. [ABSTRACT FROM AUTHOR]

Abstract Background Ischemic preconditioning is an innate mechanism of cytoprotection against ischemia, with potential for end-organ protection. The primary goal of this study was to systematically review the literature to determine the effect of ischemic preconditioning on outcomes after open and endovascular abdominal aortic aneurysm (AAA) repair. Methods The methodology followed the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. We included randomized clinical trials that evaluated the effect of remote ischemic preconditioning (RIPC) in reducing morbidity and mortality in patients undergoing open or endovascular AAA repair surgery. The primary outcomes were death, myocardial infarction, and renal impairment. Outcomes were addressed separately for open AAA repair and endovascular AAA repair (EVAR). Data were collected on patient characteristics, methodology, and preconditioning protocol for each trial. Results Nine trials of ischemic preconditioning in aortic aneurysm surgery were included with a total of 599 patients; 336 patients were included in the open AAA repair meta-analysis, and 263 patients were included in the EVAR meta-analysis. For both open and endovascular repairs, ischemic preconditioning did not have a significant effect on death, myocardial infarction, or renal impairment requiring dialysis. Conclusions The randomized clinical trials investigating the effect of ischemic preconditioning on outcomes after open and endovascular AAA repair that have been completed to date have not been adequately powered to evaluate improvements in patient-important outcomes. The evidence is insufficient to support the use of ischemic preconditioning for AAA repair in clinical practice. The variability in treatment effect across studies may be explained by clinical and methodological heterogeneity. [ABSTRACT FROM AUTHOR]

The coronavirus disease of 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has resulted in increased morbidity and mortality in patients with impaired immunity, hematologic malignancies, and immunosuppressive regimens. COVID-19 can cause a cytokine storm with some patients benefiting from blockade of the pro-inflammatory cytokine, interleukin 6 (IL6). As Castleman disease (CD) is an atypical lymphoproliferative disorder that can involve a cytokine storm and often requires immunosuppressive therapies, including IL6 inhibition, we sought to evaluate outcomes following COVID-19 and SARS-CoV-2 vaccination in CD patients. We administered a survey in April 2021 to characterize experiences with COVID-19 and SARS-CoV-2 vaccination among 300 CD patients enrolled in ACCELERATE, a natural history registry of CD patients. Among 128 respondents, the prevalence of SARS-CoV-2 infection (16/95, 17%), severe disease (1/16, 6%), vaccination rates (112/128, 88%), and vaccine adverse effects after dose one (62/112, 55%) were comparable to the general U.S. population. While there were two cases of CD flares occurring shortly after SARS-CoV-2 infection (N=1) and vaccination (N=1), over 100 patients in this study that were infected and/or vaccinated did not experience CD flares. The median anti-spike titer six months after the second dose among CD patients was comparable to individuals with other immune-related diseases and healthy populations. Data from this small cohort suggest that, despite being on immunosuppressive therapies, CD patients do not appear to be at increased risk of poor COVID-19 outcomes and can mount a humoral response to SARS-CoV-2 vaccination. This study was registered on clinicaltrials.gov (#NCT02817997)., Competing Interests: Disclosure of Conflicts of Interest All other authors report no conflicts of interest.

Competing Interests: D.L.S. has the following financial disclosures: consulting and speaking honoraria from Sanofi, Novartis, CSL Behring, Jazz Pharmaceuticals, Veloxis, Mallinckrodt, Thermo Fisher Scientific, Regeneron, and AstraZeneca. C.M.D. has the following financial disclosures: research grants from GlaxoSmithKline and Abbvie and served on a grant review committee for Gilead Sciences. A.H.K. has received consulting fees from Roche. The remaining authors have no funding or conflicts of interest to disclose.

Idiopathic inflammatory myopathies (IIMs) represent a heterogeneous group of systemic autoimmune diseases characterized by immune-mediated muscle injury. As insights into pathogenesis of IIM evolve, novel therapeutic strategies have become available to optimize outcomes. Herein, we summarize novel and emerging strategies in the management of dermatomyositis (DM), immunemediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM)., Competing Interests: Declaration of competing interest The authors of this manuscript have no financial disclosures or competing interest to disclose as described by Best Practice & Research Clinical Rheumatology., (Copyright © 2022 Elsevier Ltd. All rights reserved.)