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Chorea can be an initial manifestation of systemic lupus erythematosus (SLE) or antiphospholipid syndrome (APS). It has been mostly described in younger female adults in association with other manifestations of SLE. When chorea appears as an initial and only manifestation in SLE/APS patients, the establishment of the correct diagnosis is difficult, and it may be initially attributed to a more common aetiology. Here we report an elderly man who presented with a new onset of right-sided chorea without other clinical manifestations of SLE/APS. He started on steroids a year later, however, there was no improvement. His chorea was symptomatically managed along with aspirin, and hydroxychloroquine as he refused to be on additional immunosuppression. Anticoagulation was relatively contraindicated, and also not favoured by this patient; therefore, aspirin was initiated. Even in elderly patients, once the common etiologies of chorea have been worked up, we suggest doing a rheumatological evaluation. Early diagnosis and prompt treatment can prevent persistent neurological abnormality., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Non-bacterial thrombotic endocarditis, characterised by sterile vegetations, is commonly caused by systemic lupus erythematosus and is known to be complicated with embolic cerebrovascular disease. Embolic myocardial infarction with non-bacterial thrombotic endocarditis is extremely rare. We report a case of ventricular fibrillation arrest from presumed coronary embolisation in non-bacterial thrombotic endocarditis. While there are no standardised guidelines on the management of embolic myocardial infarction in endocarditis, it requires a multidisciplinary approach unique for every encountered clinical scenario., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

The safety, tolerance, and selected renal and non-renal outcome measures were evaluated in 73 SLE patients who received sirolimus therapy for more than 3 months in our institution over the past 21 years. In 12 patients who had lupus nephritis, proteinuria (p = 0.0287), hematuria (p = 0.0232), anti-DNA antibody levels (p = 0.0028) and steroid use were reduced (p = 0.0200). In the non-renal cohort of 61 patients, anti-DNA antibody levels (p = 0.0332) and steroid use were reduced (p = 0.0163). Both in the renal and non-renal cohorts, C3 (renal p = 0.0070; non-renal p = 0.0021) and C4 complement levels were increased (renal p = 0.0063; non-renal p = 0.0042) Adverse effects of mouth sores (2/73), headaches (1/73), and gastrointestinal discomfort were noted in a minority of patients (6/73). Sirolimus was only discontinued in two of 73 patients due to headache and recurrent infections, respectively. This study suggests that sirolimus is well tolerated and exerts long-term therapeutic efficacy in controlling renal and non-renal manifestations of SLE., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Strongyloides stercoralis infection is usually acquired from tropics or subtropics, often causes asymptomatic chronic infection, but in immunosuppressed, it can lead to hyperinfection syndrome. We report a case of chronic abdominal pain resulting from Strongyloides infection in a 55-year-old male with a history of partial small bowel resection for small intestinal lymphoma and a recent diagnosis of chronic kidney disease with proteinuria on steroid therapy. He presented with chronic abdominal pain, nausea, loss of appetite, and weight loss. Initial laboratory workup and imaging including retroperitoneal ultrasound and CT of the abdomen/pelvis were within normal limits, and he was discharged on acid suppression therapy. He was readmitted with worsening symptoms and underwent esophagogastroduodenoscopy (EGD) and duodenal biopsy, which revealed Strongyloides infection. We later discovered a travel history to Cambodia. His symptoms resolved with ivermectin therapy. This case highlights the importance of travel history, which can prevent unnecessary investigations and delay in the diagnosis., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Piranavan et al.)

Immune-related adverse events affecting parathyroid function are rarely reported with immune checkpoint inhibitors (ICPIs). Activating calcium-sensing receptor antibodies causing autoimmune hypoparathyroidism with nivolumab was recently reported. KEYNOTE-189 and CHECKMATE-067 trials reported a 21-29% hypocalcemia event rate, but the etiology of hypocalcemia was not reported. A chart review was performed to study patients receiving ICPI from 2015 to 2018 at multiple sites affiliated with Saint Vincent Hospital. The study population was divided into two groups based on the presence or absence of calcium altering conditions or medications. True hypocalcemia incidence was calculated after correcting calcium for albumin from the initiation of ICPI to their last follow-up. Group 1 ( n  = 83) includes patients with no calcium altering conditions or medications. Group 2 ( n  = 98) includes patients on calcium supplements ( n  = 17), vitamin D ( n  = 44), bisphosphonates ( n  = 24), >stage IIIB chronic kidney disease (CKD) ( n  = 5), and bone metastasis ( n  = 38). Hypocalcemia events in Group 1 vs. Group 2 were 8.4% and 19.3%, respectively. Our entire study demonstrated 26.8% vs. 1.1% of Grade I vs. II hypocalcemia events. However, after correcting the calcium for albumin, hypocalcemia incidence was 0.56% ( n  = 1). No further workup was done to investigate the etiology as that patient passed away. Our data suggest that the true hypocalcemia incidence after using albumin-corrected calcium values is very low in patients receiving IPCI, even in the presence of calcium altering factors. The percentage of patients with hypocalcemia is much higher and similar to the KEYNOTE-189 and CHECKMATE-067 trials when serum calcium values without albumin correction are used. Thus, the higher reported incidence of hypocalcemia in these trials is likely due to the reporting of serum calcium without albumin correction., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2020 Swarna Sri Nalluru et al.)

There is a considerable unmet demand for safe and efficacious medications in the realm of autoimmune and inflammatory diseases. The fate of the immune cells is precisely governed by control of various metabolic processes such as mitochondrial oxidative phosphorylation, glycolysis, fatty acid synthesis, beta-oxidation, amino acid metabolism, and several others including the pentose phosphate pathway, which is a unique source of metabolites for cell proliferation and maintenance of a reducing environment. These pathways are tightly regulated by the cytokines, growth factors, availability of the nutrients and host-microbe interaction. Exploring the immunometabolic pathways that govern the fate of cells of the innate and adaptive immune system, during various stages of activation, proliferation, differentiation and effector response, is crucial for new development of new treatment targets. Identifying the pathway connections and key enzymes will help us to target the dysregulated inflammation in autoimmune diseases. The mechanistic target of rapamycin (mTOR) pathway is increasingly recognized as one of the key drivers of proinflammatory responses in autoimmune diseases. In this review, we provide an update on the current understanding of the metabolic signatures noted within different immune cells of many different autoimmune diseases with a focus on selecting pathways and specific metabolites as targets for treatment., Competing Interests: CONFLICTS OF INTEREST The authors declare that they have no conflicts of interest.

Myelodysplastic syndrome (MDS) is a neoplastic disorder resulting in dysplasia and apoptosis of the hematopoietic clonal cells. The presenting features of MDS are usually dependent on the cellular lineage affected in the bone marrow (BM). Generally, MDS presents in older adults with recurrent infections, anemia, and bleeding tendencies. However, until now, there are no cases of splenic infarction in MDS. Splenic infarction is a rare event and is often reported in myeloproliferative or thromboembolic disorders. In this case report, we present splenic infarction; a never reported clinical manifestation in an MDS patient., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare.

Objective: The objective of this study is to investigate the clinical characteristics and treatment of patients with early-onset gout., Methods: We retrospectively reviewed 327 adult patients with a first diagnosis of gout from 2008 to 2016 using the database of a multispecialty group practice in New England. Patients were classified into the following groups: age 30 years or younger at first diagnosis (group 1), age 31-40 years (group 2), and age over 40 years (group 3). The clinical characteristics and treatment of gout were compared among the three groups., Results: We identified 87 patients in group 1 and 140 patients in group 2. Group 3 included 100 patients randomly chosen from the 7216 patients with a first diagnosis at age over 40 years. Patients within group 1 had significantly higher serum uric acid (sUA) levels at the time of diagnosis and a more prominent family history of gout. Younger patients (groups 1 and 2) had a significantly higher body mass index than patients over 40 years of age (group 3). A substantial number of younger patients also had hypertension or hyperlipidemia. The majority of younger patients met the 2012 American College of Rheumatology (ACR) guidelines for initiating urate-lowering therapy (ULT) on the basis of frequency of gout attacks, whereas the majority of patients over 40 years of age met the guidelines for ULT on the basis of chronic kidney disease. Patients over 40 years of age were more likely to achieve an sUA level less than 6.0 mg/dl., Conclusion: Patients with a first diagnosis of gout at age 40 years or younger frequently had cardiovascular risk factors and were less likely to achieve an sUA level less than 6.0 mg/dl compared with patients over 40 years of age who were treated in routine clinical practice. Clinicians should be aware that patients with early-onset gout may be an undertreated population with poor adherence to ULT and increased risk of recurrent gout and cardiovascular diseases., (© 2019 The Authors. ACR Open Rheumatology published by Wiley Periodicals, Inc. on behalf of American College of Rheumatology.)

Background: Atheroembolic renal disease (AERD) is caused by occlusion of the small renal arteries from embolized cholesterol crystals arising from ulcerated atherosclerotic plaques. This usually manifests as isolated renal disease or involvement from systemic atheroembolic disease. Here we report a case of AERD that responded well to steroid therapy., Case Summary: A 62-year-old woman with a history of hypertension and stage IIIa chronic kidney disease was referred for rapidly worsening renal function over a 4-mo period. She complained of swollen legs, dyspnea on exertion, and two episodes of epistaxis about a month prior to admission. She reported no history of invasive vascular procedures, use of radio contrast agents, or treatment with anticoagulants or thrombolytic agents. Urinalysis showed a few red blood cells and granular casts. Serology was positive for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA). Non-contrast-enhanced computed tomography of the chest, abdomen, and pelvis showed diffuse atherosclerotic changes in the aortic arch. Thus, c-ANCA-associated vasculitis was suspected, and the patient was started on pulse intravenous methylprednisolone. Her renal biopsy showed evidence of AERD. She was discharged with oral prednisone, and her renal function continued to improve during the initial follow-up., Conclusion: In cases of non-vasculitis-associated ANCA, a high degree of clinical suspicion is required to pursue the diagnosis of spontaneous AERD in patients with clinical or radiological evidence of atherosclerotic burden. Although no specific treatment is available, the potential role of statins and steroids requires exploration., Competing Interests: Conflict-of-interest statement: The authors have no conflicts of interest to declare.

BACKGROUND Takotsubo cardiomyopathy (TC) is characterized as acute left ventricular dysfunction precipitated by intense emotional or physiological stress. The mid-ventricular variant of TC usually has akinesis, with or without ballooning of the mid-ventricular segment, and a hyperdynamic base and apex. Recurrence of the typical and atypical (reversed and mid-ventricular type) forms has been reported in only a very small number of cases. We report a forme fruste presentation of mid-ventricular variant of TC. CASE REPORT A 69-year-old woman with a prior history of stress-induced cardiomyopathy presented with complaint of moderate intensity, persistent, sub-sternal chest discomfort. She reported that her symptoms were similar to those she had during a previous hospitalization in 2015, and this time cited the death of her mother as an inciting stressor. No significant obstructive flow-limiting coronary artery disease was found on cardiac catheterization. However, the left ventriculogram was suggestive of mid-ventricular pattern of TC. Her first symptomatic episode of apparent TC did not reveal completion of the mid-ventricular pattern of the TC variant. The subsequent episode, during this hospitalization, manifested as a completed version of her initial apparent forme fruste of mid-ventricular variant of TC. CONCLUSIONS TC may present in a myriad of clinical forms that must be considered in the evaluation of patients with suspected acute coronary syndromes or cardiomyopathy. Treatment is mainly supportive, and recurrence rates range from 7.7% to 11.4%. To the best of our knowledge, this forme fruste presentation has not been previously reported in recurrent variants of TC.

Dual antiplatelet therapy (DAPT) is widely recognized as the mainstay of treatment after percutaneous coronary intervention (PCI). Premature discontinuation may pose a risk of in-stent thrombosis, acute myocardial infarction, and death. With the increased usage of antiplatelet agents, increased attention has been drawn to their potential allergic reactions. A 66-year-old male with a history of coronary artery disease and ischemic stroke was admitted with worsening severity angina for cardiac catheterization. He was on dual antiplatelet agents, clopidogrel, and aspirin prior to admission. He had PCI and a drug-eluting stent deployment to the culprit vessel. Due to low responsiveness to clopidogrel, he was started on ticagrelor, as prasugrel was contraindicated due to the history of ischemic stroke. A few hours after ticagrelor initiation, he developed shortness of breath, swelling of the throat and tongue, and was diagnosed with angioedema. He didn't have any prior reported history of allergy to any medications to the contrast medium or heparin. The offending medication, ticagrelor, was discontinued. He was managed with intravenous steroids and antihistamines. After the resolution of angioedema, he was discharged with double the dose of clopidogrel in addition to aspirin. The patient did not have any ischemic symptoms or coronary events for the following six-month period of follow-up. The case highlights a relatively rare side effect of ticagrelor. Health care providers should be vigilant about the angioedema following ticagrelor administration. In our patient, it was effectively managed by discontinuing the offending medication and the administration of steroids and histamine blockers. The recovery was prompt, without any serious untoward effects. The DAPT was changed to clopidogrel, double the conventional dose, in addition to aspirin., Competing Interests: The authors have declared that no competing interests exist.

Recent renewed interest in layered transition metal dichalcogenides stems from the exotic electronic phases predicted and observed in the single- and few-layer limit. Realizing these electronic phases requires preserving the desired transport properties down to a monolayer, which is challenging. Surface oxides are known to impart Fermi level pinning or degrade the mobility on a number of different systems, including transition metal dichalcogenides and black phosphorus. Semimetallic WTe 2 exhibits large magnetoresistance due to electron-hole compensation; thus, Fermi level pinning in thin WTe 2 flakes could break the electron-hole balance and suppress the large magnetoresistance. We show that WTe 2 develops an ∼2 nm thick amorphous surface oxide, which shifts the Fermi level by ∼300 meV at the WTe 2 surface. We also observe a dramatic suppression of the magnetoresistance for thin flakes. However, due to the semimetallic nature of WTe 2 , the effects of Fermi level pinning are well screened and are not the dominant cause for the suppression of magnetoresistance, supported by fitting a two-band model to the transport data, which showed the electron and hole carrier densities are balanced down to ∼13 nm. However, the fitting shows a significant decrease of the mobilities of both electrons and holes. We attribute this to the disorder introduced by the amorphous surface oxide layer. Thus, the decrease of mobility is the dominant factor in the suppression of magnetoresistance for thin WTe 2 flakes. Our study highlights the critical need to investigate often unanticipated and sometimes unavoidable extrinsic surface effects on the transport properties of layered dichalcogenides and other 2D materials.

Lithium-sulfur batteries (Li-S batteries) have attracted intense interest because of their high specific capacity and low cost, although they are still hindered by severe capacity loss upon cycling caused by the soluble lithium polysulfide intermediates. Although many structure innovations at the material and device levels have been explored for the ultimate goal of realizing long cycle life of Li-S batteries, it remains a major challenge to achieve stable cycling while avoiding energy and power density compromises caused by the introduction of significant dead weight/volume and increased electrochemical resistance. Here we introduce an ultrathin composite film consisting of naphthalimide-functionalized poly(amidoamine) dendrimers and graphene oxide nanosheets as a cycling stabilizer. Combining the dendrimer structure that can confine polysulfide intermediates chemically and physically together with the graphene oxide that renders the film robust and thin (<1% of the thickness of the active sulfur layer), the composite film is designed to enable stable cycling of sulfur cathodes without compromising the energy and power densities. Our sulfur electrodes coated with the composite film exhibit very good cycling stability, together with high sulfur content, large areal capacity, and improved power rate.

Introduction: Systemic lupus erythematosus is a multi-system connective tissue disorder. Peripheral neuropathy is a known and underestimated complication in systemic lupus erythematosus. Ganglionopathy manifests when neuronal cell bodies in the dorsal root ganglion are involved. Autoimmune disorders are a known etiology, with systemic lupus erythematosus being a rare cause., Case Presentation: A 32-year-old South Asian woman presented with oral ulceration involving her lips following initiation of treatment for a febrile illness associated with dysuria. She had a history of progressively worsening numbness over a period of 4 months involving both the upper and lower limbs symmetrically while sparing the trunk. Her vibration sense was impaired, and her reflexes were diminished. For the past 4 years, she had had a bilateral, symmetrical, non-deforming arthritis involving the upper and lower limbs. Her anti-nuclear antibody and anti-double-stranded deoxyribonucleic acid status were positive. Although her anti-Ro antibodies were positive, she did not have clinical features suggestive of Sjögren syndrome. Nerve conduction studies revealed sensory neuronopathy. A diagnosis of systemic lupus erythematosus complicated by sensory neuronopathy was made. Treatment with intravenous immunoglobulin resulted in clinical and electrophysiological improvement., Conclusion: Peripheral neuropathy in systemic lupus erythematosus can, by itself, be a disabling feature. Nerve conduction studies should be considered when relevant. Neuropathy in systemic lupus erythematosus should be given greater recognition, and rarer forms of presentation should be entertained in the differential diagnosis when the clinical picture is atypical. Intravenous immunoglobulin may have role in treatment of sensory neuronopathy in systemic lupus erythematosus.