The impact of Libman-sacks endocarditis on inpatient outcomes with systemic lupus erythematosus: A retrospective study.

INTRODUCTION: The existing literature offers limited insights into the influence of Libman-Sacks Endocarditis (LSE) on inpatient outcomes in individuals with Systemic Lupus Erythematosus (SLE). This study aimed to explore the characteristics and prognosis of SLE patients with LSE and the impact of LSE in patients with SLE on inpatient outcomes including inpatient mortality, length of stay, acute heart failure, atrial fibrillation, and cerebrovascular accidents (CVA). METHODS: This study included adult patients who were hospitalized with SLE between the years 2019 and 2020, using the National Inpatient Sample (NIS) database. The total number of patients with a diagnosis of SLE in the years 2019 and 2020 in the NIS database was 150,411. Of those, 349 had a diagnosis of LSE. The study population was divided into two groups: one group with SLE and LSE, and another group with SLE but without LSE. RESULTS: Caucasians made up 54.9% of the patients with a diagnosis of SLE in our patient population, while African Americans made up 26.9% and the Hispanics accounted for 12.2%. Of patients with LSE, Caucasians and African Americans represented 42.9% each. Patients with a diagnosis of LSE had a higher inpatient mortality than those with SLE without LSE (aOR: 9.74 CI 1.12-84.79, p 0.04). Patients with SLE with LSE were more likely to have acute heart failure than those without LSE, although this was not statistically significant (aOR 1.18 CI 0.13-11.07, p 0.88). Similarly, patients with SLE with LSE were more likely to have atrial fibrillation than those without LSE (aOR 4.45 CI: 0.77-25.57, p 0.10). CVAs were significantly higher in SLE patients with LSE than those without LSE (aOR 141.43 CI 16.59-1205.52, p < .01). DISCUSSION: Patients who develop LSE were found to have significantly higher risks of inpatient mortality and cerebrovascular accidents. Early and precise detection of LSE in such patients may ensure timely intervention and prevention of the associated adverse outcomes. Further studies may attempt to develop screening methods for detection of LSE to effectively reduce morbidity and mortality associated with SLE.

A String of Pearls: Linear Immunoglobulin A (IgA) Bullous Dermatosis in the Setting of Imipramine Use and Newly Diagnosed Ulcerative Colitis.

Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is an autoimmune disease affecting children or adults that leads to subepithelial vesiculobullous lesions on the skin and/or mucosa. Due to the histologic and clinical appearance of the disease with tense and pruritic blisters, direct immunofluorescence is required for diagnosis, which features the characteristic linear deposition of IgA autoantibodies along the basement membrane zone. LABD can be idiopathic, drug-induced, or associated with a systemic disease such as inflammatory bowel disease. Many drugs have been implicated, such as antibiotics, anti-hypertensives, anti-epileptics, analgesics, and immunosuppressive medications. Treatment of LABD centers on discontinuation of the offending drug, if applicable, as well as pharmacotherapy with dapsone as the first-line treatment. Adjunctive therapy with sulphonamides, systemic corticosteroids, cyclosporine, colchicine, intravenous immunoglobulins, tetracyclines, erythromycin, and dicloxacillin has also shown benefits. We report the case of a young adult patient who developed LABD with a background of recent initiation of treatment with imipramine and newly diagnosed ulcerative colitis.

The Burden of Cardiac Arrhythmias in Gout: A National Representative Database Study.

The association between gout and arrhythmias has not been thoroughly examined. This study discusses the underappreciated burden, patterns, and outcomes of several arrhythmias, which may have prognostic value in patients with gout. This is a retrospective cohort study that used the US National Inpatient Sample for 2015-2019. Complex samples multivariable logistic and linear regression models were used to assess the incidence and trends in gout-related arrhythmia and consequential inpatient mortality, hospital length of stay (LOS), hospitalization charges, and predictors of mortality. Hospitalizations that included a diagnosis of gout accounted for 60,360 admissions. Arrhythmias affected roughly one-fourth of those. When compared to individuals without arrhythmia, those who experienced arrhythmias were older. Arrhythmias were found to be equally common in both men and women. The most common subtype was AF (88%), followed by atrial flutter (6.2%), conduction disorders (4.7%), and ventricular tachycardia (3.2%). In individuals with gout, there was a rising trend in arrhythmia-related hospital admissions and mortality. The gout-arrhythmia group had more traditional cardiac comorbidities. After adjusting for baseline variables, the arrhythmia group had significantly greater mortality (693 vs 77 per 100,000 hospitalizations), mean LOS (4.3 vs 3.7 days), and hospital costs ($33,057 vs $28,384). In gout, incident arrhythmia dramatically raised the risk of death (adjusted odds ratio, 2.06; 95% CI, 1.95-2.16; P < 0.001). Gout patients who are hospitalized with concurrent arrhythmia have a likelihood of longer stays in the hospital and higher mortality. Early identification and treatment of arrhythmia may benefit outcomes in gout patients.

Immunoglobulin A Vasculitis Associated With COVID-19 Infection Successfully Treated With Corticosteroid Regimen Without Relapse.

Immunoglobulin A (IgA) vasculitis is an autoimmune disease associated with bacterial and viral infections that typically presents with palpable purpura, arthralgia, abdominal pain, and renal involvement. Coronavirus disease 2019 (COVID-19) infection has been found to trigger numerous autoimmune and rheumatologic conditions, including IgA vasculitis. We report a patient who had a COVID-19 infection and then two weeks later developed severe abdominal pain, nausea, emesis, diarrhea, hematochezia, palpable purpura, and arthralgia. Skin biopsy revealed deposition of IgA and C3 complement granular deposition with fibrinogen deposition in superficial dermal vessel walls consistent with IgA vasculitis. The patient was treated with intravenous methylprednisolone followed by oral prednisone with significant improvement and no relapse after tapering and discontinuing steroids in six weeks. This case of biopsy-proven IgA vasculitis precipitated by active COVID-19 infection demonstrates the ability of COVID-19 infection to induce IgA vasculitis and its response to corticosteroid treatment.

Acute, Painless Monocular Vision Loss: Non-arteritic Ischemic Optic Neuropathy Associated With Untreated Obstructive Sleep Apnea, Uncontrolled Type 2 Diabetes Mellitus, Essential Hypertension, and Hyperlipidemia: A Case Report.

Non-arteritic ischemic optic neuropathy (NAION) is a common cause of acute, painless monocular vision loss in adults older than 50. NAION is a diagnosis of exclusion established once arteritic disease and other etiologies of acute vision loss have been ruled out. Clinicians need to distinguish NAION from arteritic ischemic optic neuropathy (AION) since failing to appropriately treat patients presenting with AION results in an inferior prognosis. NAION is often associated with risk factors like obstructive sleep apnea, atherosclerosis, diabetes mellitus, hypertension, hyperlipidemia, smoking, and phosphodiesterase-5 inhibitors. Clinicians need to address these risk factors to help prevent the development of NAION in their patients. Here, we present the case of a 63-year-old Caucasian male who presented with acute, painless monocular vision loss.

Predictors of remission in rheumatoid arthritis patients treated with biologics: a systematic review and meta-analysis.

Biologics have emerged as an effective treatment of rheumatoid arthritis (RA). However, there is a significant proportion of patients who fail to respond to biologics. Identifying the predictors that affect the response to biologics remains challenging. A comprehensive literature search of PubMed, Embase, and Web of Science databases was conducted through May 1, 2022. We included all studies that used a multivariate model to assess for the predictors of remission in RA patients treated with biologics. We calculated pooled odds ratios (OR) with 95% confidence intervals (CI) for risk factors reported in ≥ 3 studies using a random-effects model. A total of 16,934 patients with RA who were treated with biologics were included in twenty-one studies. Our study showed that old age (OR 0.98 (0.97, 0.99), P < 0.00001), female gender (OR 0.66 (0.56, 0.77), P < 0.00001), smoking history (OR 0.86 (0.75, 0.99), P 0.04), obesity (OR 0.95 (0.91, 0.99), P 0.02), poor functional status (OR 0.62 (0.48, 1.27), P < 0.00001), high disease activity (OR 0.90 (0.85, 0.96), P 0.0005), and elevated erythrocyte sedimentation rate (OR 0.99 (0.98, 1.00), P 0.009) were poor predictors of remission. On the other hand, positive anti-citrullinated protein antibodies (OR 2.52 (1.53, 4.12), P 0.0003) was associated with high remission rate. Old age, female gender, obesity, smoking history, poor functional status, high disease activity, and elevated ESR at the time of diagnosis have been associated with poor response to biologics. Our findings could help establish a risk stratification model for predicting the remission rate in RA patients receiving biologics.

Balanced Crystalloids versus Normal Saline in Adults with Sepsis: A Comprehensive Systematic Review and Meta-Analysis.

The crystalloid fluid of choice in sepsis remains debatable. We aimed to perform a comprehensive meta-analysis to compare the effect of balanced crystalloids (BC) vs. normal saline (NS) in adults with sepsis. A systematic search of PubMed, EMBASE, and Web of Sciences databases through 22 January 2022, was performed for studies that compared BC vs. NS in adults with sepsis. Our outcomes included mortality and acute kidney injury (AKI), need for renal replacement therapy (RRT), and ICU length of stay (LOS). Pooled risk ratio (RR) and mean difference (MD) with the corresponding 95% confidence intervals (CIs) were obtained using a random-effect model. Fifteen studies involving 20,329 patients were included. Overall, BC showed a significant reduction in the overall mortality (RR 0.88, 95% CI 0.81-0.96), 28/30-day mortality (RR 0.87, 95% CI 0.79-0.95), and AKI (RR 0.85, 95% CI 0.77-0.93) but similar 90-day mortality (RR 0.96, 95% CI 0.90-1.03), need for RRT (RR 0.91, 95% CI 0.76-1.08), and ICU LOS (MD -0.25 days, 95% CI -3.44, 2.95), were observed between the two groups. However, subgroup analysis of randomized controlled trials (RCTs) showed no statistically significant differences in overall mortality (RR 0.92, 95% CI 0.82-1.02), AKI (RR 0.71, 95% CI 0.47-1.06), and need for RRT (RR 0.71, 95% CI 0.36-1.41). Our meta-analysis demonstrates that overall BC was associated with reduced mortality and AKI in sepsis compared to NS among patients with sepsis. However, subgroup analysis of RCTs showed no significant differences in both overall mortality and AKI between the groups. There was no significant difference in the need for RRT or ICU LOS between BC and NS. Pending further data, our study supports using BC over NS for fluid resuscitation in adults with sepsis. Further large-scale RCTs are necessary to validate our findings.

Genome-wide DNA methylation pattern in systemic sclerosis microvascular endothelial cells: Identification of epigenetically affected key genes and pathways.

Background: The etiology of systemic sclerosis is not clear, but there is evidence suggesting a critical role for epigenetic alterations in disease pathogenesis and clinical expression. We sought, in this study, to characterize the genome-wide DNA methylation signature in systemic sclerosis microvascular endothelial cells. Methods: We performed a genome-wide DNA methylation study in microvascular endothelial cells derived from seven diffuse cutaneous systemic sclerosis patients compared to seven age-, sex-, and ethnicity-matched healthy controls. We paired matched samples on Illumina HumanMethylation450 (three diffuse cutaneous systemic sclerosis microvascular endothelial cells and three controls), and reproduced the results in an independent set of matched patient and controls using Illumina Infinium MethylationEPIC (four diffuse cutaneous systemic sclerosis patients and four controls) to identify differentially methylated genes. Results: We identified 71,353 differentially methylated CpG sites in systemic sclerosis microvascular endothelial cells using Infinium MethylationEPIC microarray in the first group (0.081% of representative probes) and 33,170 CpG sites in the second group using HumanMethylation450 microarray (0.073% of representative probes) in diffuse cutaneous systemic sclerosis microvascular endothelial cells. Among the two groups of subjects, we identified differential methylation of 2455 CpG sites, representing 1301 genes. Most of the differentially methylated CpG sites were hypermethylated (1625 CpG), corresponding to 910 genes. Common hypermethylated genes in systemic sclerosis microvascular endothelial cells include NOS1, DNMT3A, DNMT3B, HDAC4, and ANGPT2. We also identified hypomethylation of IL17RA, CTNNA3, ICAM2, and SDK1 in systemic sclerosis microvascular endothelial cells. Furthermore, we demonstrate significant inverse correlation between DNA methylation status and gene expression in the majority of genes evaluated. Gene ontology analysis of hypermethylated genes demonstrated enrichment of genes involved in angiogenesis (p = 0.0006). Pathway analysis of hypomethylated genes includes genes involved in vascular smooth muscle contraction (p = 0.014) and adherens junctions (p = 0.013). Conclusion: Our data suggest the presence of significant genome-wide DNA methylation aberrancies in systemic sclerosis microvascular endothelial cells, and identify novel affected genes and pathways in systemic sclerosis microvascular endothelial cells.

Subacute Cutaneous Lupus as a Paraneoplastic Manifestation of Non-Hodgkin Lymphoma.

Malignancies have been associated with paraneoplastic syndromes, such as dermatomyositis. Subacute cutaneous lupus erythematosus (SCLE) can occur due to a wide array of cancers. Paraneoplastic SCLE obeys McLean's criteria and often regresses after the underlying malignancy has been treated appropriately. Anti-Ro/SSA antibodies are often present in patients with paraneoplastic SCLE; however, there have been many instances where anti-Ro may not be present. We report a case of non-Hodgkin lymphoma causing SCLE, a malignancy not previously known to be associated with paraneoplastic SCLE. We also highlight the importance of perhaps prompt chemotherapy to treat the underlying malignancy, as a failure to do so may lead to worse patient outcomes.

Henoch-Schonlein Purpura Presenting as Upper Gastrointestinal Bleed in an Adult Patient.

Henoch-Schonlein purpura (HSP) is a multi-system autoimmune disease that is relatively common in pediatric patients. HSP usually manifests as palpable purpura, arthralgia, abdominal pain, and acute kidney injury. Here, we present a case of an adult male with hematemesis as the initial presenting symptom of HSP. A previously healthy, 18-year-old Caucasian male presented with a one-day history of hematemesis associated with abdominal pain and non-bloody diarrhea. He also reported bilateral knee and ankle arthralgias with a painless skin rash on both lower extremities. Physical exam was positive for palpable, purpuric, non-blanchable skin rash involving bilateral lower extremities. Notable labs on admission included a white cell count of 10.8 x 109/L and C-reactive protein of 4.8 mg/L. Upper endoscopy showed non-bleeding erosive gastropathy and duodenal erosions. Skin biopsy of the left leg showed immunoglobulin A (IgA) deposition within the walls of the superficial dermal vessels. The patient was started on intravenous methylprednisolone 500 mg daily followed by a steroid taper. Due to incomplete clinical response to steroids, mycophenolate mofetil 1000 mg twice daily was added and maintained for three months. His symptoms improved significantly, and he no longer complained of abdominal pain or diarrhea. Gastrointestinal manifestations are common in HSP patients. However, the diagnosis will be challenging when these symptoms precede other classical manifestations of HSP. History and physical exam are key components in accurately diagnosing HSP; nevertheless, skin biopsy remains the gold standard to confirm the diagnosis.