Systemic lupus erythematosis

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple systems of the body. Recent research on the gut microbiota dysbiosis associated with SLE patients has gained traction and warranted further exploration. It has not been determined whether the change in the gut microbiota is a cause of SLE or a symptom of SLE. However, based on the physiological and pathophysiological role of the bacteria in the gut microbiome, as levels of the bacteria rise or fall, symptomatology in SLE patients could be affected...

Lupus is a relatively rare disease; however, many of the patients diagnosed with lupus experience an acute confusional state. Despite the prevalence, lupus cerebritis remains a diagnosis of exclusion due to the number of differential diagnoses of the cause of acute confusional state and unreliable clinical markers. This case report highlights the significant duration it takes to work up a broad differential before initiating treatment for lupus. Our case involves a 65-year-old woman with a documented history of lupus, who presented with acute encephalopathy...

Septic arthritis is uncommon in pediatric patients, who are less likely to have major risk factors such as underlying joint disease or prosthetic joints. It only rarely affects the elbow and is usually caused by Gram-positive cocci, with Staphylococcus aureus being the most common bacterial organism. We present the case of a 15-year-old previously healthy female who experienced new-onset monoarticular nontraumatic elbow pain and was found to have a synovial effusion growing from Moraxella catarrhalis . The atypical clinical presentation, coupled with the growth of an unusual organism, raised concern for an underlying immunocompromising or inflammatory joint disorder...

BACKGROUND: Enteritis is one of the rare systemic manifestations in childhood-onset systemic lupus erythematosus and its diagnosis is very challenging. This is a rare case of an adolescent girl with recurrent non-specific gastro-intestinal symptoms which were later diagnosed to be owing to lupus enteritis, the only presenting manifestation of an active flare. CASE REPORT: A 15-year-old girl was admitted with recurrent episodes of abdominal pain, vomiting and loose stools...

Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune blistering disorder of cutaneous lupus erythematosus (CLE) that typically manifests as an acute vesiculobullous eruption in a patient with systemic lupus erythematosus (SLE). Also, it can rarely present as the initial clinical manifestation of SLE. There is no established US Food and Drug Administration (FDA) therapy for BSLE. We report a case of a 71-year-old Hispanic woman with SLE and lupus nephritis classes III and V who presented to the hospital with a worsening rash with painful, ruptured blisters involving the upper arms, chest, and back...

Kikuchi-Fujimoto Disease (KFD), or histiocytic necrotizing lymphadenitis (HNL), is a rare self-limiting disorder presenting with fever and swollen lymph nodes. It is characterized by the focal proliferation of reticular cells, the presence of nuclear debris, and histiocytes. In advanced cases, it can present with hepato-splenomegaly and generalized lymphadenopathy. Historically, it has been associated with viral infections, as it frequently was found to be associated with upper respiratory symptoms. Alternative explanations include the immune response of T-cells leading to alteration in CD8-positive T-cell-mediated cell apoptosis...

BACKGROUND: Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare benign disease that presents as cervical lymphadenopathy and fever. CASE PRESENTATION: A 25-year-old South Asian female dentist, recently married, presented to our emergency department due to two weeks of fever, sore throat, swollen neck, and cough. The patient initially presented to a private clinic and was prescribed antibiotics on two visits. On physical examination, her neck was swollen with palpable and tender right posterior and submandibular lymph nodes...

Background Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by various clinical manifestations. Despite efforts to improve outcomes, mortality rates remain high, and certain disparities, including gender, may influence prognosis and mortality rates in SLE. This study aims to examine the gender disparities in outcomes of SLE hospitalizations in the US. Methods We conducted a retrospective analysis of the Nationwide Inpatient Sample (NIS) database between 2016 and 2020. The NIS database is the largest publicly available all-payer database for inpatient care in the United States, representing approximately 20% of all hospitalizations nationwide...

Because most patients with lupus nephritis have a positive antinuclear antibody (ANA), ANA-negative lupus nephritis is a rare complication of systemic lupus erythematosus (SLE). In the 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) classification criteria for SLE, a negative ANA precludes further work-up of SLE. The following case discusses a patient with multiple negative ANA titers but was diagnosed with SLE based on the findings of the kidney biopsy showing lupus nephritis...

Serum amyloid A (SAA) subtypes 1-3 are well-described acute phase reactants that are elevated in acute inflammatory conditions such as infection, tissue injury, and trauma, while SAA4 is constitutively expressed. SAA subtypes also have been implicated as playing roles in chronic metabolic diseases including obesity, diabetes, and cardiovascular disease, and possibly in autoimmune diseases such as systemic lupus erythematosis, rheumatoid arthritis, and inflammatory bowel disease. Distinctions between the expression kinetics of SAA in acute inflammatory responses and chronic disease states suggest the potential for differentiating SAA functions...

Nearly 50% of patients with systemic lupus erythematosus (SLE) will develop lupus nephritis (LN). Current treatment regimens for LN are suboptimal as the majority of patients fail to achieve complete renal response after several months of treatment and there are high rates of relapse. We report outcomes in four LN patients who were treated with both voclosporin and belimumab. These patients had no serious infections, and we were able to taper glucocorticoids and reduce proteinuria.

As a chronic autoimmune disease, systemic lupus erythematosus (SLE) primarily affects young women and does not discriminate against any particular organs. In December 2019, coronavirus disease 2019 (COVID-19) spread worldwide, with many speculations of cardiac involvement in the pathogenesis of infection. Moreover, in cases where cardiac symptoms were described, they consisted solely of chest pain or a general deterioration in health if the patient presented with pleural effusion or pericardial effusion. Our patient, a 25-year-old Hispanic woman, initially complained of chest pain, cough, and shortness of breath...

Autoimmune myelofibrosis (AIMF) is a rare disorder characterized by cytopenias and autoimmunity, with characteristic bone marrow findings that include lymphocytic infiltration and fibrosis. AIMF is described predominantly in adult populations who have systemic lupus erythematosis (SLE), with scant pediatric cases described mainly in older adolescents with SLE. Here, we described the largest single-center pediatric experience of pediatric autoimmune myelofibrosis (PAIMF) series, demonstrating both similarities and distinctions from the adult experience...

Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multisystem involvement. It is multifactorial and involves epigenetic, genetic, ecological, and environmental factors. Primarily it leads to activation of both innate and adaptive immunity, which consequently leads to autoreactive B cell activation by T cells and leads to immune complexes deposition in tissues leading to an autoimmune cascade that may be limited to the single organ or can cause a widespread systemic involvement...

PURPOSE OF REVIEW: This manuscript provides an update on clinical and pathophysiological features of juvenile-onset systemic lupus erythematosis (jSLE), challenges applying adult-derived classification criteria, and recent advances in treatment and care. RECENT FINDINGS: Significant scientific advances have improved the understanding of genetic factors (both genetic causes and risk alleles) and associated phenotypic features. Panels of urine/blood biomarker candidates aid in diagnosing jSLE, monitoring disease activity and predicting treatment response...

Macrophage activation syndrome (MAS) is a potentially fatal complication of an autoimmune rheumatologic disease characterized by overwhelming inflammation, multiorgan failure, and high mortality if untreated. We report a rare case of a 56-year-old man who presented with fever for three weeks and had a constellation of clinical features and laboratory findings, meeting the diagnostic criteria for systemic lupus erythematosus (SLE) and SLE-associated MAS. He was treated with high dose intravenous corticosteroid and hydroxychloroquine, resulting in resolution of fever and dramatic clinical improvement...

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease involving multiple systems with a range of clinical presentations caused by the production of antibodies, activation of complements, and deposition of immune complexes. The exact cause of SLE is still unknown. The effectiveness of traditional treatment methods for SLE is very little. Nowadays, resistance to conventional therapy, steroids, and immunosuppressants is common among SLE patients. Patients with refractory disease and nephritis generally have severe drug-induced toxicity which contributes to organ dysfunction, despite available therapies...

Background Autoimmune hepatitis (AIH) is an inflammatory disease of the liver that is characterized by a broad disease spectrum, circulating autoantibodies, and elevated serum globulin levels. Systemic lupus erythematosus (SLE) is a chronic disease that is characterized by a high inflammatory state and is associated with multiorgan system involvement. Despite a well-known association between AIH and other autoimmune diseases, the literature is deficient on the associations between AIH-related outcomes and complications in SLE patients...

Acute myocardial infarction in a young patient is a nebulous entity, but in the absence of traditional cardiovascular risk factors, particular attention must be paid to thrombotic disorders and hypercoagulable states. A 28-year-old male presented with worsening substernal chest pain for 36 hours. He was recently diagnosed with systemic lupus erythematosus (SLE) with active class II lupus nephritis. With an initial electrocardiogram revealing ischemic changes, and an elevated troponin I, a concern was raised for myocardial infarction...

Belimumab is a B-cell depletion therapy that has emerged as an effective and safe treatment option for Systemic Lupus Erythematosus (SLE), but ongoing phase IV trials continue to report its common and rare adverse effects. Our case report seeks to add data to the existing literature on the safety profile of belimumab. We report an interesting and complicated case of a 30-year-old female with a 12-year history of SLE and multiple treatment failures who developed acute pancreatitis in the context of the initiation of belimumab...