Presentation Of Systemic Lupus Erythematosus Research Articles

Bicytopenia Secondary to Autoimmune Myelofibrosis as the First Presentation of an Undiagnosed Systemic Lupus Erythematosus: A Rare Case Report

Bicytopenia Secondary to Autoimmune Myelofibrosis as the First Presentation of an Undiagnosed Systemic Lupus Erythematosus: A Rare Case Report

Variable presentations of systemic lupus erythematosus based on presence or absence of antidouble stranded DNA antibodies: A case series

Systemic lupus erythematosus (SLE) presents with diverse clinical features causing diagnostic challenges. Apart from the clinical features, autoantibodies are important for diagnosis along with certain laboratory parameters. Diagnosis is made with the European League against Rheumatism/American College of Rheumatology 2019 Criteria. The case series presented here signifies the correlation between anti ds DNA positivity and its association with poor prognosis and renal disease, whereas antidouble stranded DNA (anti-dsDNA) negativity may lead to lack of renal involvement and may be associated with polyserositis. The importance lies in the fact that these patients with anti-dsDNA negativity should be followed up for assessing conversion to positivity of anti-dsDNA, thus altering the prognosis and leading to renal involvement. Moreover, anti-SSA positive SLE patients must be followed up for possible development of sicca symptoms.

Fever

Fever is one of the most common presentations of Systemic Lupus Erythematosus (SLE) (95%) but it is also associated with other manifestations. A 32-year-old female patient who presented with chief complaints of fever and burning micturition and during the course of the stay developed different manifestations like facial rash, leukopenia, proteinuria, and SLE was diagnosed on the antinuclear antibody (ANA) profiling of the patient and the clinical criteria. Here we present a case where the patient presented mainly with urinary tract infection which in turn acted as a trigger for immune reaction leading to the full-blown SLE.

Thrombotic thrombocytopenic purpura as initial presentation of systemic lupus erythematosus.

Thrombotic thrombocytopenic purpura as initial presentation of systemic lupus erythematosus.

Systemic lupus erythematosus presenting as Guillain‒Barre syndrome

A 37-year-old male patient presented with acute symmetrical ascending areflexic flaccid quadriparesis progressive in nature for the past 6 days before admission preceded by tingling sensation in both hands and feet with the right-sided lower motor type of facial nerve palsy for 2 days. Clinically, this was consistent with Guillain–Barre (GB) syndrome and was given intravenous immunoglobulin (IVIG). Cerebrospinal fluid study and nerve conduction study also supported the clinical diagnosis of GB syndrome. He developed Type 2 respiratory failure on the 4th day of admission for which he was intubated. After successful extubation, his weakness even worsened markedly despite IVIG therapy and was subjected to plasmapheresis following which the weakness improved remarkably. On further investigation for the associated fever, arthralgia, pallor, nephritis, antinuclear antibody (3+ coarse speckled pattern), anti-smith antibody, and anti-dsDNA antibody positivity were detected with low serum complement levels, and renal biopsy revealing class III lupus nephritis. He was put on oral corticosteroids and cyclophosphamide which resulted in further clinical improvement. The initial presentation of GB syndrome of systemic lupus erythematosus is uncommon and makes this case interesting.

Systemic lupus erythematosus presenting as unprovoked brachio-cephalic vein thrombosis: A case report

Systemic lupus erythematosus presenting as unprovoked brachio-cephalic vein thrombosis: A case report

Acute Confusional State in a Neuropsychiatric Systemic Lupus Erythematosus Patient: A Case Report

Systemic Lupus Erythematosus (SLE) affects various systems in human including central and peripheral nervous systems. The Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) is a severe complication of SLE which causes a formidable challenge in term of diagnosis and its management. Acute confusional state (delirium) is a rare entity of numerous symptoms of NPSLE which is being overlooked even though it is associated with increased mortality. The present case report describe about the presentation of SLE consisting of hyperpigmentation maculopapular rashes over face, neck regions, lower back and extremities in a 36-yearold female with abrupt onset and fluctuating course of psychotic symptoms, acute confusional state and oddities in behaviours. Treatment with low dose of antipsychotic (aripiprazole) resolve the symptom with due consideration taken to rule out steroid induced manifestation of above symptoms.

A case of systemic lupus erythematosus presenting with bilateral malignant proptosis

A case of systemic lupus erythematosus presenting with bilateral malignant proptosis

‘When Cryptococcus strikes and lupus is found’: a unique covert unveiling of systemic lupus erythematosus presenting as subacute meningitis

Cryptococcal neoformans is a rare fungal pathogen that has been associated with immunocompromised individuals. Due to its rare occurrence, clinicians have a low index of suspicion for diagnosis, which can lead to increased morbidity and mortality. We present an 81-year-old fully functional woman with no known predisposing risk factors or previous immunocompromising conditions who was found to have cryptococcal meningitis on cerebrospinal fluid analysis in the setting of newly diagnosed uncontrolled type 2 diabetes and systemic lupus erythematosus (SLE).

An Unusual Case of Late-onset Systemic Lupus Erythematosus Presenting with Digital Gangrene

Digital gangrene is an uncommon initial manifestation of systemic lupus erythematosus (SLE) and rarely in late-onset disease. This case presents a 50-year-old woman who developed digital gangrene and was subsequently diagnosed with SLE. Early treatment with immunosuppression and anticoagulation halted the progression of the digital ischemia. This case highlights that late-onset SLE, often described as having a more benign and insidious course, can also present with catastrophic limb-threatening manifestations.

A Case of Guillain-Barré Syndrome as the First Presentation of Systemic Lupus Erythematosus

Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with different clinical manifestations. Acute [resembling Guillain-Barré syndrome (GBS)] or chronic (chronic inflammatory demyelinating polyradiculoneuropathy) inflammatory polyradiculoneuropathy has been reported in rare SLE cases. Case Presentation: We reported a 39-year-old woman that presented with acute peripheral neuropathy, and she was eventually diagnosed with SLE. She developed distal numbness and paraesthesia followed by progressive upper and lower extremity weakness and difficulty in swallowing and speaking. She had a history of flu-like illness three weeks before to symptoms. Conclusions: Progressive upper and lower extremity weakness along with areflexia and electrodiagnostic findings suggested the diagnosis of Guillain-Barré syndrome. Over a month, significant neurological recovery occurred, and the patient's function continued to recover.

Purtscher like retinopathy as the initial presentation of paediatric systemic lupus erythematosus in a 4 year old child

The Journal is the primary organ of Continuing Paediatric Medical Education in Sri Lanka. The journal also has a website. Free full text access is available for all readers.The Sri Lanka Journal of Child Health is now indexed in SciVerse Scopus (Source Record ID 19900193609), Index Medicus for South-East Asia Region (IMSEAR), CABI (Centre for Agriculture and Bioscience International Global Health Database), DOAJ and is available in Google, as well as Google Scholar.The policies of the journal are modelled on the Committee on Publication Ethics (COPE) Guidelines on Principles of Transparency and Best Practice in Scholarly Publishing. Sri Lanka Journal of Child Health is recognised by the International Committee of Medical Journal Editors (ICMJE) as a publication following the ICMJE Recommendations.

Peritonitis as the first presentation of systemic lupus erythematous: a case report

BackgroundSystemic lupus erythematosus is an inflammatory disease affecting several organs. Serositis is one of the systemic lupus erythematosus presentations, but peritonitis is a relatively rare presentation. Particularly, it is extremely rare to observe peritonitis as the first presentation of systemic lupus erythematosus.Case presentationHere, we present a case of peritonitis without other symptoms of systemic lupus erythematosus, in a patient who was finally diagnosed with systemic lupus erythematosus. Our patient was a 27-year-old Persian/Caucasian male with fatigue, weakness, weight loss, abdominal distension, massive ascites, and normocytic hemolytic anemia. He did not mention any prior medical conditions and did not use any drugs. There were no signs of thyroid dysfunction, cardiac dysfunction, cancers, infectious diseases, hepatitis, kidney diseases, or other diseases. Low-gradient, high-protein ascites fluid, and positive antinuclear antibody and anti-double stranded DNA were in favor of systemic lupus erythematosus. Corticosteroid pulse therapy led to resolution of ascites, and the patient was discharged with prednisolone and hydroxychloroquine.ConclusionPeritonitis is a rare presentation of systemic lupus erythematosus, particularly as the first presentation and in the absence of other signs and symptoms; however, systemic lupus erythematosus should be considered as one the differential diagnoses for peritonitis when other etiologies have been ruled out.

Juvenile systemic lupus erythematosus presenting as retinal vasculitis.

A 15-year-old boy presented with a 2-week history of decreased vision in his right eye and a painful rash over his toes. On examination he had pallor, a vasculitic rash over the toes of both feet, and a visual acuity of 6/24 in the right eye. Fundoscopy showed vascular sheathing of the central retinal vessels, cotton wool spots, and dot haemorrhages, consistent with retinal vasculitis (figure A). Blood tests showed a haemoglobin concentration of 8 g/dL, white cell count of 1700 per μL (normal range 4000–11 000 per μL), and platelet count of 70 000 per mL (normal range 150 000–400 000 per mL). His renal and hepatic parameters were normal. Tests for antinuclear antibodies and anti-double-stranded DNA antibodies were positive, and direct Coomb's test was positive. Antinuclear antibody blot test showed positivity for anti-Sjögren's-syndrome-related antigen A antibodies and anti-double-stranded DNA antibodies. Complement concentrations were normal and tests for antiphospholipid antibodies were negative.

A rare presentation of systemic lupus erythematosus with lupus hepatitis

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammation and the presence of circulating autoantibodies directed against self-antigens. Prevalence of SLE in children and adolescents is 1-6 per 100,000 population. Liver dysfunction occurs in approximately in 50-60% of the patients with SLE.And the incidence of lupus hepatitis in diagnosed SLE patients is 9.3%. We are reporting a case of 11-year-old female child who presented with fever, skin rashes, oral ulcers, irritability and positive antinuclear antibody (ANA) with deranged liver function test (LFT) in the form of elevated transaminases, bilirubin level and altered coagulation profile. Hepatitis serology was negative, with low C3 levels, diagnosed as SLE with lupus hepatitis with lupus nephritis stage IV with psychosis. Clinical improvement along with improvement in terms of laboratory findings was seen on corticosteroids therapy. It is important to differentiate lupus hepatitis from autoimmune hepatitis (AIH) as AIH presents similar to lupus hepatitis, has poor prognosis. While lupus hepatitis being rare cause for mortality in patients with SLE has good response with early intervention with corticosteroids therapy.

Abstract 10845: Spontaneous Coronary Artery Dissection as the Primary Presentation of Ana-Negative Lupus

Introduction: Spontaneous coronary artery dissection (SCAD) is a rare cause of myocardial infarction that most often occurs in middle-aged women with minimal classic risk factors and has been associated with hormonal imbalance, pregnancy, fibromuscular dysplasia, and connective tissue disease. SCAD has been seldom associated with systemic lupus erythematosus (SLE). SLE patients typically exhibit positive ANA titers. Here, we present the first known case report of a patient with SCAD as the primary presentation of ANA-negative SLE. Case Presentation: A 55-year-old female with migraines presented for elective kidney donation. Surgery was complicated by hemodynamic instability and NSTEMI. Transthoracic echocardiogram (TTE) showed preserved ejection fraction and hypokinesis of the basal to mid lateral and septal walls. Coronary angiogram was suspicious for type 3 SCAD of the left anterior descending (LAD) coronary artery, and given the clinical course, a coronary wire was advanced to perform intracoronary hemodynamic assessment, which was negative for atherosclerotic disease. Upon retraction of the wire, there was no flow in the LAD, consistent with propagated SCAD, leading to PEA arrest. After resuscitation, the LAD was stented. Repeat TTE showed preserved ejection fraction and normal wall motion. She was discharged on aspirin and clopidogrel. At cardiology outpatient follow up, the patient described symptoms consistent with Raynaud’s phenomenon and was referred to Rheumatology. She ultimately also developed hair loss, oral ulcers, joint pain, photosensitivity, malar erythema, and Sicca symptoms. Her anti-double stranded DNA antibodies were strongly positive twice, but remarkably, ANA screening was repeatedly negative. However, taken together, she met classification criteria for SLE diagnosis. Conclusions: SLE can present in highly atypical fashion, clinically and serologically, as with this case. SCAD is rare, but this case highlights the importance of keeping it on the differential for myocardial infarction, particularly in female patients with non-traditional risk factors or rheumatological disease. This case also exemplifies there should be a low clinical threshold for rheumatological workup in patients diagnosed with SCAD.

CXCL13 Neutralization Attenuates Neuropsychiatric Manifestations in Lupus-Prone Mice

Neuropsychiatric lupus (NPSLE), the nervous system presentation of systemic lupus erythematosus (SLE), remains challenging to treat due to its unclear pathogenesis and lack of available targeted therapies. A potential contributor to disease progression is brain tertiary lymphoid structures (TLS); these ectopic lymphoid follicles that can develop tissue-targeted antibodies have recently been described in the MRL/lpr lupus mouse strain, a classic model for studying NPSLE. The brains of MRL/lpr mice show a significant increase of CXCL13, an important chemokine in lymphoid follicle formation and retention that may also play a role in the disease progression of NPSLE. The aim of the present study was to inhibit CXCL13 and examine the effect of this intervention on lymphoid formation and the development of neurobehavioral manifestations in lupus mice. Female MRL/lpr mice were injected with an anti-CXCL13 antibody, an IgG1 isotype-matched antibody, or PBS either three times a week for 12 weeks intraperitoneally (IP) starting at 6-8 weeks of age, or continuously intracerebroventricularly (ICV) with an osmotic pump over a two-week period starting at 15 weeks of age. Cognitive dysfunction and depression-like behavior were assessed at the end of treatment. When treatment was delivered IP, anti-CXCL13 treated mice showed significant improvement in cognitive function when compared to control treated mice. Depression-like behavior was attenuated as well. Furthermore, mice that received anti-CXCL13 by the ICV route showed similar beneficial effects. However, the extent of lymphocyte infiltration into the brain and the general composition of the aggregates were not substantively changed by anti-CXCL13 irrespective of the mode of administration. Nevertheless, analysis of brain gene expression in anti-CXCL13 treated mice showed significant differences in key immunological and neuro-inflammatory pathways that most likely explained the improvement in the behavioral phenotype. Our results indicate that CXCL13 affects the behavioral manifestations in the MRL/lpr strain and is important to the pathogenesis of murine NPSLE, suggesting it as a potential therapeutic target.

P047 Musculoskeletal symptoms as first clinical presentation of juvenile-onset systemic lupus erythematosus: Experience from a military internal medicine department

Abstract Background Juvenile-onset systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder occurring before the age of sixteen. The clinical manifestations are variable and the management of the disease at this age may be different. The aim of our study was to investigate first clinical presentation of juvenile-onset SLE. Methods We conducted a retrospective study in a military internal medicine department. We accessed medical records of 103 patients with SLE diagnosed according to the revised 1997 American College of Rheumatology (ACR) criteria or the 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria. Only patients with an age of lupus-onset < the age of sixteen were included. Clinical data were collected and analyzed. The statistical analysis was performed using SPSS 23.0 software. Result There were eight juvenile-onsets SLE: two males and six females. The median age at the diagnosis was 13.7 + 1.9 years-old (11–16). First clinical presentations are shown in table 1. Arthritis and hematological involvement tended to be associated (p = 0.07). Hypocomplementemia and positive anti-DNA antibodies were detected in 50% of cases. All patients received Hydroxychloroquine and most of them combined corticosteroids (7cases). Conclusion Our study showed that musculoskeletal manifestations especially arthritis were frequent at the diagnosis of juvenile-onset SLE. According to the aggressive pharmacological treatment, a multidisciplinary approach should be required

Massive haemorrhage: an unusual presentation of systemic lupus erythematosus

SummarySystemic lupus erythematosus (SLE) usually affects young females and its presentation with haemorrhage is unusual. Intracerebral bleed (ICB) has been described in a few case reports, but massive haematuria has not been reported in the literature so far. Here, we present a case of a 56 year-old-lady who presented with expanding lobar bleed and massive haematuria as the first presentation of SLE secondary to vasculitis. Lupus vasculitis usually presents with thrombosis-related complications and although bleeding in this context is rare, it can lead to catastrophic consequences. Hence, vasculitis-related bleeding should be taken under consideration in a challenging situation like ours.

A Case of Budd-Chiari Syndrome – As an Unusual Early Manifestation of Systemic Lupus Erythematosis with Antiphospholipid Syndrome and Deep Vein Thrombosis

Budd-Chiari syndrome occurs due to obstruction of hepatic venous out-flow which in turn produces intense congestion of the liver. Systemic Lupus Erythematosus (SLE), which is an auto-immune disorder has protean manifestations such as easy fatiguability, arthralgia, photosensitivity, malar rash, fever, alopecia but our case had an unique association of ascites and hepatosplenomegaly due to Budd Chiari syndrome stemming from the root cause of Antiphospholipid antibody syndrome (APLA) is very rare. SLE accounts for 40% of the cases of APLA. SLE presenting with Budd-Chiari syndrome as an early manifestation is unusual and is rarely reported in the literature. Here we report a rare case of a young female who presented with abdominal distension, abdominal pain and fever diagnosed to have Budd-Chiari syndrome as a presenting feature of SLE and on further evaluation she was also found to have antiphospholipid syndrome secondary to SLE.