Sjögren's syndrome and neuromyelitis optica spectrum disorders (NMOSD)--a case report and review of literature.

BACKGROUND: Neuromyelitis optica (NMO) is a rare relapsing auto-immune disease of the central nervous system which is sometimes found in association with other autoimmune disorders including Sjogren's syndrome. We present the case of a middle aged female with Sjogren's syndrome (SS) and Neuromyelitis optica spectrum disorders (NMOSD) who had a rapidly declining neurological illness that responded to immunosuppressive therapy. CASE PRESENTATION: A 51-year-old female with Sjogren's syndrome and recent history of varicella zoster infection presented with right upper and lower extremity weakness of one week duration. She was noted to have contrast enhancement at C2-C4 cord levels on cervico-thoracic MRI. Comprehensive work up was negative except for presence of a mild lymphocytic pleocytosis and oligoclonal bands in the CSF. She was diagnosed with transverse myelitis secondary to varicella zoster infection and was treated with high dose steroids in addition to acyclovir with improvement in her symptoms. Two months later she developed left upper and lower extremity weakness, bilateral dysesthesias and urinary incontinence. Repeat MRI of the cervico-thoracic spine revealed worsening enhancement at lower cervical cord levels (C5-7) with extension to T1. CSF analysis was unchanged; however immunological work up was abnormal for elevated NMO-IgG/AQP4 antibody. She was diagnosed with NMOSD and was treated with immunosuppressive therapy. Initially with IV methylprednisone and Cyclophosphamide therapy followed by Mycophenolate mofetil (MMF) maintenance therapy with good response. Repeat MRI 6 months later showed near complete resolution of previous abnormal cord signal changes. CONCLUSION: One needs to recognize the relationship between autoimmune diseases especially SS and NMOSD. The presence of NMO antibody has been associated with a relapsing disease course and a careful follow-up, besides use of remission maintenance agents such as MMF and Azathioprine are important to consider.

Chemotherapy induced right ventricular cardiomyopathy; a systematic review and meta-analysis.

Background: Left ventricular dysfunction and cardiomyopathy are well documented adverse effects associated with chemotherapy agents. Limited information exists regarding the impact of chemotherapeutic agents on the integrity and function of the right ventricle (RV). Objectives: The current metanalysis compared pre- chemotherapy versus post- chemotherapy RV parameters measured on 2D echocardiography in patients receiving anthracycline and/or trastuzumab across all breast cancer patients. Methods: A systematic search across PubMed, EMBASE and Cochrane databases were performed from inception of the databases until November 2021 for relevant studies. We used the inverse variance method with a random effect model and DerSimonian and Laird method of Tau2 generation to calculate mean difference [MD] with 95% confidence interval [CI]. The analysis was carried out using RevMan Version 5.3 (Copenhagen: The Nordic Cochrane Centre, The Cochrane Collaboration, 2014). Results: Fifteen studies, constituting total of 644 patients, met the inclusion criteria, with most studies having a follow up period of less than 12 months from initiation of chemotherapy. Anthracycline and/or Trastuzumab chemotherapy resulted in a statistically significant reduction in right ventricular ejection fraction (RVEF) at follow-up [MD: 2.70, 95% CI: 0.27 to 5.13, P-value- 0.03, I2- 71%, χ2 P-value < 0.05]. Treatment with Anthracycline and/or Trastuzumab chemotherapy resulted in a significant reduction in RV fractional area change (RVFAC) at follow-up [MD: 3.74, 95% CI: 1.33 to 6.15, P-value < 0.01, I2- 68%, χ2 P-value < 0.05]. RV free wall longitudinal strain (RVFWLS) was lower at baseline, while LVEF was significantly reduced at follow-up [MD: -1.00, 95% CI: -1.86 to -0.15, P-value < 0.05, I2- 0%, χ2 P-value-0.40], [MD: 4.04, 95% CI: 2.08 to 6.01, P-value < 0.01, I2- 91%, χ2 P-value < 0.05], respectively. However, treatment with Anthracycline and/or Trastuzumab chemotherapy had no statistically significant effect on Tricuspid annular plane systolic excursion (TAPSE) at follow-up [MD: 0.53, 95% CI: -0.11 to 1.17, P-value-0.11, I2- 98%, χ2 P-value < 0.05]. Conclusions: Chemotherapy with anthracyclines and trastuzumab negatively affects right ventricular function leading to decline in RVEF, RVFAC, RVFWLS and LVEF.

VEXAS syndrome with progression of MDS to MDS/MPN overlap syndrome.

VEXAS (vacuoles, E1 enzyme, X linked, autoinflammatory, somatic) syndrome is a novel inflammatory syndrome that was first described in December 2020. Patients with VEXAS syndrome have a somatic mutation in the UBA1 gene, inflammatory conditions and usually haematological conditions. Haematological conditions reported in patients with VEXAS syndrome include myelodysplastic syndrome (MDS), clonal cytopenia of undetermined significance, plasma cell neoplasm including multiple myeloma/monoclonal gammopathy of undetermined significance, haemophagocytic lymphohistiocytosis and monoclonal B-cell lymphocytosis. Here we describe a patient with VEXAS syndrome who had a progression of MDS to MDS/myeloproliferative neoplasm overlap syndrome. The ocular findings so far reported in these patients include episcleritis, uveitis, blepharitis and orbital cellulitis. Here we report retinal detachment as a clinical feature of VEXAS syndrome. This finding has a significant implication in patient management as it warrants higher vigilance for this sight-threatening complication.

A Systematic Review of COVID-19 and Pericarditis.

Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), was first identified in Wuhan, China in December 2019. Since then, the disease has spread globally, leading to the ongoing pandemic. It can cause severe respiratory illness; however, many cases of pericarditis have also been reported. This systematic review aims to recognize the clinical features of pericarditis and myopericarditis in COVID-19 patients. Google Scholar, Medline/PubMed, CINAHL, Cochrane Central, and Web of Science databases were searched for studies reporting "Coronavirus" or "COVID" and "Peri-myocarditis," "heart," or "retrospective." Case reports and retrospective studies published from May 2020 to February 2021 were reviewed. In total, 33 studies on pericarditis, myopericarditis, and pericardial infusion were included in this review. COVID-19 pericarditis affected adult patients at any age. The incidence is more common in males, with a male-to-female ratio of 2:1. Chest pain (60%), fever (51%), and shortness of breath (51%) were the most reported symptoms, followed by cough (39%), fatigue (15%), myalgia (12%), and diarrhea (12%). Laboratory tests revealed leukocytosis with neutrophil predominance, elevated D-dimer, erythrocyte rate, and C-reactive protein. Cardiac markers including troponin-1, troponin-T, and brain natriuretic peptide were elevated in most cases. Radiographic imaging of the chest were mostly normal, and only 31% of chest X-rays showed cardiomegaly and or bilateral infiltration. Electrocardiography (ECG) demonstrated normal sinus rhythm with around 59% ST elevation and rarely PR depression or T wave inversion, while the predominant echocardiographic feature was pericardial effusion. Management with colchicine was favored in most cases, followed by non-steroidal anti-inflammatory drugs (NSAIDs), and interventional therapy was only needed when patient developed cardiac tamponade. The majority of the reviewed studies reported either recovery or no continued clinical deterioration. The prevalence of COVID-19-related cardiac diseases is high, and pericarditis is a known extrapulmonary manifestation. However, pericardial effusion and cardiac tamponade are less prevalent and may require urgent intervention to prevent mortality. Pericarditis should be considered in patients with chest pain, ST elevation on ECG, a normal coronary angiogram, and COVID-19. We emphasize the importance of clinical examination, ECG, and echocardiogram for decision-making, and NSAIDs, colchicine, and corticosteroids are considered to be safe in the treatment of pericarditis/myopericarditis associated with COVID-19.

We need to do better: Readability analysis of online patient information on cancer survivorship and fertility preservation.

OBJECTIVE: National Institute of Health recommends that patient education material should be written at no greater than 6th-grade level. Cancer survivorship and fertility preservation discussion with patients is recommended by many national societies and being done more frequently. We sought to analyze online patient information on cancer survivorship and fertility preservation to see if they meet the criteria set by national guidelines. METHODS: Online patient information on cancer survivorship and fertility preservation was collected and analyzed by six of the most common readability tests. Only websites in English and free to access were used. RESULTS: A total of 15 separate websites for cancer survivorship and fertility preservation was used. All websites failed to meet national guidelines. Cancer survivorship information was written at a high school senior and a 12th-grade level. Fertility preservation information was written at a high school-senior and junior college level. CONCLUSION: Online patient information on cancer survivorship and fertility preservation did not meet national guidelines. Testing across six of the most used readability indexes showed that information is challenging to understand for the general patient population. POLICY STATEMENT: This article shares an insight into the complex and growing fields of cancer survivorship and fertility preservation. Educating patients about their condition is critical and improves outcomes and participation in shared decision making. Healthcare policy should focus on implementing a system that will provide culturally and linguistically appropriate information in the community for patients about their disease.

Incidental Asymptomatic Splenic Infarct in a COVID-19 Patient.

A high incidence of thromboembolic events and coagulation parameter abnormalities are seen in cases of coronavirus disease 2019 (COVID-19). Both venous and arterial thrombosis, including arterial thrombosis in unusual sites, have been reported in COVID patients in recent literature. Herein, we report a case of a 67-year-old female patient with non-critical COVID-19 disease with an incidental finding of an asymptomatic splenic infarct. In the absence of a cardio-embolic source, we believe this was an arterial thromboembolic event in the splenic circulation. The duration and modality of anticoagulation of inpatient and ambulatory COVID patients remains a dynamic discussion. Our case adds the evidence of a clinically silent arterial thrombotic event in a non-critical COVID-19 patient which further emphasizes the need to address the strategies for diagnosis and management of thrombo-embolism to prevent potentially fatal complications.

Observational Study of Thrombotic Events in a Random Cohort of Hospitalized COVID-19 Patients at a Community-Based Hospital of New York City During the Beginning of the 2020 Pandemic.

Coronavirus disease 2019 (COVID-19) continues to pose an unprecedented challenge for the entire world and the healthcare system. Different theories have been proposed elucidating the pathophysiological mechanisms attributing to high mortality and morbidity in COVID-19 infection. Out of them, thrombosis and procoagulant state have managed to earn the maximum limelight. We conducted an observational study based on data from randomly selected 349 hospitalized patients with COVID-19 infection in a community-based hospital in New York City during the first wave of the COVID-19 viral surge in March 2020. The main objective of our study was to assess the risk and occurrence of thrombotic events (both venous and arterial) among the hospitalized patients including the intensive care unit (ICU) and non-ICU admissions with confirmed COVID-19 infection. The primary outcome in our study was defined as the thrombotic events that included myocardial infarction (MI), deep venous thrombosis (DVT), cerebrovascular accidents (CVA), and pulmonary embolism (PE). The study correlated the association of thrombotic events with the level of biomarkers of interest: D-dimer >1000 ng/ml, troponin-I >1 ng/ml, or both. The association of D-dimers and troponin-I with thrombotic events was measured using both univariate and multivariate Cox proportional hazard (PH) regression analysis. Out of a total of 349 patients, 78 patients (22.35%) were found to have elevated biomarkers (D-dimer >1000 ng/ml and/or troponin-I >1 ng/ml) and were categorized as a high-risk group. Eighty-nine patients developed thrombotic complications (evidence of more than one thrombotic event was found in several patients). Two-hundred seventy-one (77.65%) patients had no documentation of thrombosis. The incidence of thrombotic events included myocardial infarction (MI; N=45; 12.8%), cerebrovascular accidents (CVA; N=16; 4.5%), deep venous thrombosis (DVT; N=16; 4.5%), and pulmonary embolism (PE; N=9; 2.57%).

Acute coronary syndrome in Behcet's syndrome: A systematic review.

Behcet syndrome is a rare vasculitis that affects both arteries and veins. Vasculo-Bechet Syndrome (VBS) is seen predominantly in men. Genetic predisposition and immune dysregulation leading to inflammation, endothelial damage, and impaired fibrinolysis contribute to its pathogenesis. Isolated case reports of Behcet syndrome (BS) with associated acute coronary syndrome (ACS) have been reported in the past. In this study, we present the first systematic review of such cases. A systematic search was conducted using Pubmed, Google Scholar, CINAHL, Cochrane CENTRAL, and Web of Science databases from 1980-2018 to identify case reports of myocardial infarction associated with BS. Cases that fulfilled the criteria for BS were selected for analysis. Demographic data, electrocardiography, echocardiography, angiography findings, and management were analyzed when available. We identified 62 case reports. Most subjects were men with a mean age of 37 years. Twenty-one percent were smokers, but other traditional cardiovascular risk factors were less common. Myocardial infarction was confirmed in half of the cases with findings on electrocardiogram (ECG). Echocardiogram revealed wall motion abnormality in 76% of patients, and angiography showed double-vessel disease in more than half of the cases. Mortality was reported in 1.6% of the cases. This systematic review shows that ACS in BS affects young males with low prevalence of coronary artery disease risk factors. Chest pain is the most common presenting feature and ST-segment elevation myocardial infarction (STEMI) was the most common ECG finding. Immunotherapy may be helpful to prevent future ACS in these patients.

Left gonadal vein thrombosis in a patient with COVID-19-associated coagulopathy.

COVID-19 disease is a viral illness that predominantly causes pneumonia and severe acute respiratory distress syndrome. The endothelial injury and hypercoagulability secondary to the inflammatory response predisposes severely ill patients to venous thromboembolism. The exact mechanism of hypercoagulability is still under investigation, but it is known to be associated with poor prognosis. The most common thrombotic complication reported among these patients is pulmonary embolism. To our knowledge, gonadal vein thrombosis is an uncommon phenomenon that has not been reported in the setting of COVID-19-associated coagulopathy. We report an unusual case of ovarian vein thrombosis and pulmonary embolism associated with COVID-19 presenting with abdominal pain. To our knowledge, this is the first reported case of COVID-19 with absent respiratory symptoms and presentation with venous thrombosis in an unusual location.

Risk of hepatitis B reactivation and cytomegalovirus related infections with Mogamulizumab: A retrospective study of international pharmacovigilance database.

BACKGROUND: Mogamulizumab (Moga) is a C-C chemokine receptor-4 antibody approved in the United States for relapsed /refractory mycosis fungoides and Sézary syndrome. Few cases reported an increased risk of hepatitis B reactivation and cytomegalovirus (CMV) related infection post-Moga. However, literature is limited to mainly case reports and series, while no study has used the Food and Drug Administration adverse events reporting system (FARES) database to investigate the relationship. METHODS: Using United States Food and Drug Administration adverse events reporting system database, we collected all cases of hepatitis B reactivation and CMV related infection between January 1, 2011, and December 31, 2019, for Moga and other drugs. The reporting odds ratio (ROR) was calculated, which was considered significant when the lower limit of 95% confidence interval (CI) >1. FINDINGS: Three hundred and thirty-eight total adverse cases were reported for Moga during the study period, with 261 cases reported indication for use, including cutaneous T cell lymphoma (47.04%), and adult T cell leukemia/lymphoma (30.18%). Eight cases were reported for hepatitis B reactivation with Moga use, compared to 2290 cases with other medications. The ROR is 143.67 (p<0.001, 95% CI, 71.17-290.04). CMV related infection was noted in 17 cases using Moga, while 12,849 cases with others. The ROR is 55.89 (p<0.001, 95% CI, 34.31-91.06). In the Moga group, five deaths occurred in hepatitis B reactivation patients and nine deaths with CMV cases. INTERPRETATION: A signal has been identified between Moga exposure and hepatitis B reactivation as well as CMV related infection. A consideration in future studies should be placed on determining the relationship and investigating the need for pre-treatment screening, close monitoring, and utilization of prophylaxis in this population-based on pre-treatment risks. FUNDING: None.

COVID-19-Associated Coagulopathy: An Exacerbated Immunothrombosis Response.

Since the onset of the global pandemic in early 2020, coronavirus disease 2019 (COVID-19) has posed a multitude of challenges to health care systems worldwide. In order to combat these challenges and devise appropriate therapeutic strategies, it becomes of paramount importance to elucidate the pathophysiology of this illness. Coronavirus disease 2019, caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV2), is characterized by a dysregulated immune system and hypercoagulability. COVID-associated coagulopathy (CAC) was recognized based on profound d-dimer elevations and evidence of microthrombi and macrothrombi, both in venous and arterial systems. The underlying mechanisms associated with CAC have been suggested, but not clearly defined. The model of immunothrombosis illustrates the elaborate crosstalk between the innate immune system and coagulation. The rendering of a procoagulant state in COVID-19 involves the interplay of many innate immune pathways. The SARS-CoV2 virus can directly infect immune and endothelial cells, leading to endothelial injury and dysregulation of the immune system. Activated leukocytes potentiate a procoagulant state via release of intravascular tissue factor, platelet activation, NETosis, and inhibition of anticoagulant mechanisms. Additional pathways of specific relevance in CAC include cytokine release and complement activation. All these mechanisms have recently been reported in COVID-19. Immunothrombosis provides a comprehensive perspective of the several synergistic pathways pertinent to the pathogenesis of CAC.

Brugada Pattern in Diabetic Ketoacidosis: A Case Report and Scoping Study.

Brugada syndrome is a rare cardiac arrhythmia which is associated with right bundle branch block pattern (RBBB) and ST-segment elevation in right precordial leads. SCNA5 mutation is the most common genetic abnormality associated with Brugada syndrome. Brugada pattern not related to genetic mutations has been previously reported in the setting of fever, metabolic conditions, lithium use, marijuana and cocaine abuse, ischemia and pulmonary embolism, myocardial and pericardial diseases. Multiple isolated cases of Brugada pattern associated with diabetic ketoacidosis (DKA) have been previously reported. We here present a case of type 1 Brugada pattern in a 23 year-old-male who presented with DKA. Brugada pattern in DKA is attributed to acidosis and multiple electrolyte abnormalities including hyperkalemia which alter ion channel expression in the heart thus leading to Brugada pattern which subsequently resolved with treatment of DKA. In such patients, Brugada pattern is not reproducible on procainamide induction cardiac electrophysiology study (EPS). Our scoping study demonstrates male predominance 20/22 cases of (DELETE this highlighted area) Brugada pattern in DKA, a finding that is consistent with prevalence of this disease among males.

Early recognition is the key to success: pulseless electrical activity (PEA) as a manifestation of heparin-induced anaphylactoid reaction.

A 64-year-old man underwent mitral valve replacement. He was hospitalised 9 days later because of slurring of speech and left-sided facial weakness. During hospitalisation, unfractionated heparin (UFH) bolus and drip were initiated due to subtherapeutic international normalised ratio. Within 5 min of UFH bolus, the patient began to experience symptoms of dyspnoea, followed by pulseless electrical activity arrest. He was successfully resuscitated. A decline in platelet count >50% was noted immediately after UFH bolus and cardiac arrest. A diagnosis of heparin-induced thrombocytopenia and anaphylactoid reaction was considered (4Ts score of 7). Heparin was discontinued and argatroban was started. A platelet factor 4 (PF4) assay was strongly positive. Platelet counts subsequently improved following discontinuation of heparin.