Presentation Of Systemic Lupus Erythematosus Research Articles

A report on neglected case of systemic lupus erythematosus presenting by Degos’ skin disease and diffuse non-scarring alopecia with dramatic response to treatment

A report on neglected case of systemic lupus erythematosus presenting by Degos’ skin disease and diffuse non-scarring alopecia with dramatic response to treatment

Systemic Lupus Erythematosus (SLE) and Thrombotic Thrombocytopenic Purpura (TTP) Presenting as Recurrent Strokes: A Case Report

Systemic Lupus Erythematosus (SLE) and Thrombotic Thrombocytopenic Purpura (TTP) Presenting as Recurrent Strokes: A Case Report

A Rare Presentation of Systemic Lupus Erythematosus in a Patient With Fever of Unknown Origin

A Rare Presentation of Systemic Lupus Erythematosus in a Patient With Fever of Unknown Origin

A Neglected case of Systemic lupus erythematosus presenting by Degos’ skin disease and diffuse non-scarring alopecia with dramatic response to treatment, clinically and dermoscopy.

A Neglected case of Systemic lupus erythematosus presenting by Degos’ skin disease and diffuse non-scarring alopecia with dramatic response to treatment, clinically and dermoscopy.

Idiopathic intracranial hypertension as the initial presenting manifestation of systemic lupus erythematosus in a child.

Idiopathic intracranial hypertension (IIH) is a diagnosis of exclusion characterized by features of raised intracranial pressure (ICP) in the absence of brain parenchymal lesion, vascular malformations, hydrocephalus, or central nervous system (CNS) infection. Commonly used other terms for this entity include benign intracranial hypertension (BIH) or pseudotumor cerebri. Few case reports of systemic lupus erythematosus (SLE) presenting as IIH are available in the literature. We report a 12-year-old girl presented with chronic holocranial headache and occasional episodes of projectile vomiting for the last 6 months and then developed blurring of vision for the last month. She fulfilled the criteria for IIH. Subsequent evaluation revealed a diagnosis of SLE. The occurrence of IIH in SLE is not coincidental and is reported in 1%-5.4% of patients with SLE. Though corticosteroids have not been widely used in IIH, underlying SLE warranted administering corticosteroids with subsequent complete resolution of IIH. Pediatricians, neurologists, intensivists, and ophthalmologists should consider SLE as a differential diagnosis in children presenting with IIH.

Uncommon Presentation of Systemic Lupus Erythematosus: Intracranial Mass Lesions as the First Manifestation

Uncommon Presentation of Systemic Lupus Erythematosus: Intracranial Mass Lesions as the First Manifestation

Mononeuritis multiplex as clinical presentation of systemic lupus erythematosus

Mononeuritis multiplex as clinical presentation of systemic lupus erythematosus

A Case of Systemic Lupus Erythematosus Presenting as Acute Pulmonary Embolism in a 34 Years Old Women

A Case of Systemic Lupus Erythematosus Presenting as Acute Pulmonary Embolism in a 34 Years Old Women

Severe pulmonary hypertension as the initial manifestation of systemic lupus erythematosus: A case report a 36 years old man

Severe pulmonary hypertension as the first sign of systemic lupus erythematosus (SLE) is uncommon, but has a considerable influence on prognosis compared to other published cases of SLE. However, with the development of new therapeutic alternatives, patient mortality has decreased significantly in recent years. This case, involving a 36-year-old man whose initial presentation of SLE was severe PAH, illustrates the importance of early detection in these individuals to achieve better outcomes.

Systemic lupus erythematosus presenting with atypical hemolytic uremic syndrome: a case report and review of the literature.

Systemic lupus erythematosus (SLE) can present with a diverse array of hematologic manifestations, among which atypical hemolytic uremic syndrome (aHUS) is a rare entity. SLE-triggered aHUS has significant morbidity and mortality without timely intervention, yet its frequency remains uncertain and optimal strategies for complement-directed therapies are largely expert-driven. We performed a comprehensive literature review and present a case of a 23-year-old female newly diagnosed with SLE/class IV lupus nephritis who developed aHUS that rapidly responded to the C5 antagonist, eculizumab. Review of the current literature identified forty-nine published cases of SLE with concurrent aHUS and revealed a predilection for aHUS in younger SLE patients, concurrent presentation with lupus nephritis, anti-dsDNA positivity, and complement system abnormalities. Over seventy percent of cases used eculizumab as complement-directed therapy with a trend towards faster time to improvement in laboratory parameters, though reported outcomes were highly variable. Early recognition of aHUS in SLE is pivotal in guiding appropriate therapeutic interventions, and prompt initiation of eculizumab may reduce the potential morbidity associated with plasmapheresis and additional immunosuppression. While eculizumab showcases promising results, its optimal timing and duration remain elusive. An understanding of a patients' complement genetics could aid management strategies, and ongoing research into complement-targeted therapies offers promising avenues for both SLE and aHUS treatment.

Combined immunosuppression with cyclosporin A, mycophenolate mofetil (MMF) and dexamethasone for activity control of recurrent secondary hemophagocytic lymphohistiocytosis (sHLH) with underlying systemic lupus erythematosus (SLE)

A42-year-old female patient was admitted to hospital due to acute neurological symptoms (dysarthria, disorientation). After exclusion of cerebral ischemia and hemorrhage an autoimmune encephalitis was diagnosed. Shortly before as an outpatient the suspicion of the presence of systemic lupus erythematosus (SLE) was voiced. The patient showed a constellation of high levels of inflammatory laboratory parameters and within a few days developed a severe pancytopenia. In the presence of all diagnostic criteria a secondary hemophagocytic lymphohistiocytosis (sHLH) was diagnosed and confirmed by a kidney biopsy during the course of the underlying SLE. The immunosuppressive treatment with etoposide and high-dose dexamethasone according to the HLH-94 protocol only showed temporary success. After 3 weeks of treatment with a protocol-conform dose reduction, under running treatment a new exacerbation of symptoms was confirmed. A renewed dose escalation of the drugs used did not lead to control of the symptoms. The inflammatory activity could only be sustainably controlled by the use of cyclosporin A in combination with mycophenolate mofetil (MMF) and dexamethasone. After stabilization of the condition of the patient, an outpatient follow-up care was possible.

A mysterious hematochezia in an adolescent boy: atypical presentation of childhood systemic lupus erythematosus

Thrombosis is a well-known entity in presence of antiphospholipid antibody (APLA) associated with systemic lupus erythematosus (SLE) as a hematological complication. Bleeding manifestations instead of thrombosis is hardly found in literature in presence of APLA seromarkers in SLE. Since these can range from minor bleeding like epistaxis to major life-threatening intracranial bleeding, early diagnosis and prompt treatment is essential to manage such condition. We report a 12 years old boy with no significant past history presented with hematochezia and epistaxis along with pallor requiring blood transfusion. Hematological investigations were normal except for elevated PT, aPTT and INR. Common causes of coagulopathy were ruled out. Upon suspecting systemic diseases, the investigations were carried out which revealed ANA 4+ along with high titre of dsDNA, low C3 and C4 complement and positive anti-β2 glycoprotein, anticardiolipin antibody and lupus anticoagulant. Diagnosis of SLE was made according to ACR-EULAR criteria with no renal involvement. Immunological basis was considered for coagulopathy in this child. He was started on oral prednisolone, hydroxychloroquine and methotrexate. He is now under close monitoring of the coagulation profile for titration of steroid dose. We want to create awareness about the uncommon hematological manifestation of SLE presenting as bleeding diathesis instead of thrombosis through this case report and that can be life threatening too if not treated promptly. A high index of suspicion and careful follow-up may help in preventing adverse outcome of the disease.

Ocular manifestations of juvenile lupus erythematosus: An 2023 update

Aims/Purpose: Juvenile Systemic Lupus Erythematosus (jSLE) is a chronic multisystem inflammatory disease that manifests before the age of 16 years, following a remitting ‐ relapsing course. The range of clinical presentations of juvenile Systemic Lupus Erythematosus (jSLE) in children is multifaceted. Ocular manifestations are uncommon for children with jSLE but can have a significant effect on their vision if not diagnosed early. Any part of the visual pathway can be affected by jSLE. The aim of this report is to systematically review the ocular manifestations of jSLE.Methods: A systematic review according to Preferred Reporting Items for Systematic Reviews and Meta‐Analyses (PRISMA) 2020 Guidelines was conducted searching MEDLINE and Scopus, including reference lists with appropriate terms for jSLE ocular manifestations. Epidemiological studies, preferably cross‐sectional studies, that describe the prevalence of ocular manifestations in children with a diagnosis or upon diagnosis of jSLE were assessed.Results: Our search yielded 131 results, screened by two independent reviewers. 33 studies were finally included in the review after preliminary assessment. Keratoconjunctivitis sicca, dry eye syndrome, retinopathy and optic neuritis are among the conditions that have been reported. Ocular manifestations in jSLE may be the result of three different mechanisms: the disease itself, infections related to immunosuppression and side effects of drug therapy. Retinal involvement is one of the most important manifestations, implying high possibility of visual loss. It is usually caused by immune complexes and autoantibodies deposition to the retinal vessels or hypertension resulting from kidney disease.Conclusions: Ocular manifestations can occur early in the course of jSLE, sometimes even before other systemic symptoms and can serve as markers of disease activity. Diagnosing ocular manifestations in children with jSLE is important for early intervention, monitoring disease activity, preventing vision loss and providing comprehensive care. By addressing ocular manifestations alongside systemic lupus management, healthcare providers can optimize outcomes and improve the overall well‐being of children with jSLE.

Macrophage Activation Syndrome as the Primary Presentation of Pediatric Systemic Lupus Erythematosus: A Case Report and Review of the Literature

Macrophage Activation Syndrome as the Primary Presentation of Pediatric Systemic Lupus Erythematosus: A Case Report and Review of the Literature

Initial Presentation of SLE among the Different South African Ethnic Groups

Background: Systemic lupus erythematosus (SLE) is a chronic, immune-mediated, inflammatory multi-organ disease. It is characterized by a variety of clinical features including abnormalities of the joints, skin, kidneys, lungs, heart, blood and brain. In addition to gender, genetic, environmental, hormonal and chemical factors appear to play a role in disease activity. Previous studies in South Africa have shown an increasing trend of new diagnoses of SLE patients, with increasing target organ damage but there is an obvious distinction between different organs in terms of incidence. The primary aim of this study was to identify the major initial presentation of SLE patients among the three main South African ethnic groups: Black, Mixed ethnicity, and White. Material and Methods: This was a retrospective descriptive and analytic study in which patients with SLE were compared among the three ethnic groups in South Africa. Data was obtained from patient’s records contained in the Enterprise Content Management System (ECM) and medical records. Results: A total of 198 patients were seen in the Rheumatology Division with SLE between March 1993 and November 1999. The study showed that the most affected group was the mixed ethnicity population (81.4%), followed by the black African group (11.6%), then the white group (7%). Arthritis was the most frequent disorders among the three-different ethnic groups. However, there appear to be some differences in the autoantibody profile.
 Conclusion:This study focused on the initial presenting symptoms. A long-term cohort on the development of the disease may yield further results on possible ethnic differences, and continued research into susceptibility loci and phenotypic associations in enriched multi-ethnic cohorts and looking for the causes of death among the three ethnic groups and related to the first presentation is suggested.

A rare case of Systemic Lupus Erythematosus presenting as acute myopericarditis with bilateral pleural effusions.

Acute myopericarditis is a rare presentation in clinical practice with multiple aetiologies. Eventhough cardiac manifestations are known to be present in up to 50% of Systemic Lupus Erythematosus (SLE) patients, acute myopericarditis is an uncommon presentation, occurring in up to 1% of patients. Here we report a patient who presented with fever and pleuritic type chest pain and was managed as acute myopericarditis with bilateral exudative pleural effusions and later diagnosed to have SLE. The patients were initially treated with nonsteroidal anti-inflammatory drugs and later with steroids and hydroxychloroquine. Early diagnosis of myopericarditis and identification of the aetiology is essential to halt the progression of disease. Pericarditis due to Tuberculosis need to be excluded before starting steroids in the Sri Lankan setting.

Autoimmune Haemolytic Anaemia as Initial and Only Presentation of Systemic Lupus Erythematosus: A Case Report

Autoimmune haemolytic anaemia (AIHA) is an acquired disorder resulting in the presence of antibodies against red blood cell antigens which leads to intravascular haemolysis. It can occur as the initial and sometimes the only presenting feature of Systemic Lupus Erythematosus (SLE). In this report, we describe the diagnostic evaluation and ongoing management of a 13-year-old female with AIHA as the first clinical feature of SLE. She presented with paleness of the body and yellowish discoloration of the eyes. Initial examination showed severe pallor with moderate jaundice. Investigations revealed severe anaemia with slightly elevated reticulocyte count and positive direct coombs test(DCT). Peripheral blood film showed microcytic, hypochromic anaemia. Bone marrow aspiration showed a hypocellular marrow for the age of the child. Immunological screening results were positive for antinuclear antibody (ANA) and anti-double stranded DNA (Anti-ds DNA). Patient is currently on Prednisolone, hydroxychloroquine and mycophenolate mofetil(MMF). The diagnosis of SLE with AIHA requires a good history, physical examination, high index of suspicion and extensive investigations. Management entails multidisciplinary approach, supportive care, initiation of treatment with steroids and immunosuppressive drugs if necessary to minimize morbidity and mortality.

Epstein-Barr virus infection as potential indicator of the occurrence and clinical presentation of systemic lupus erythematosus.

The relationship between Systemic lupus erythematosus (SLE) and Epstein-Barr virus (EBV) infection has been suggested for decades, but the underlying mechanism of the EBV influence on SLE development remains to be elucidated. The goals of this research, which included 103 SLE patients and 99 controls, were to investigate the association of the parameters of EBV infection and SLE, to explore whether pooled demographic, clinical and EBV markers achieve a more significant effect on SLE development than each of them individually, and to evaluate EBV nuclear antigen 1 (EBNA1) and latent membrane protein 1 (LMP1) gene polymorphisms in isolates from SLE patients. Comprehensive results related to serological, molecular and sequence markers of EBV infection in SLE patients demonstrated even 24 times higher possibility of having SLE if there is the presence of anti-EBV-EA(D) (early antigen) IgG antibodies (OR=24.086 95%CI OR=2.86-216.07, p=0.004). There was the same distribution of glucocorticoids (p=0.130), antimalarials (p=0.213), and immunosuppressives (p=0.712) in anti-EBV-EA(D) IgG positive and negative SLE patients. Further, higher anti-EBV-EA(D) IgG antibodies titers were identified as independent factors associated with lymphopenia, hematological SLE manifestation (OR=1.041, 95%CI OR=1.01-1.08, p=0.025, while a higher titer of anti-CA (viral capsid antigen) IgG antibodies (OR=1.015, 95%CI OR=1.01-1.03, p=0.019) and positive RF (rheumatoid factors) (OR=4.871, 95%CI OR=1.52-15.61, p=0.008) were identified as independent factors associated with alopecia within SLE. Finally, novel data on EBV EBNA1 and LMP1 gene polymorphisms in lupus are reported. The results support further investigation targeting EBV as a prognostic marker and therapeutic goal for lupus.

High Titer ANA at Pediatric ITP Presentation Associated with Progression to Systemic Lupus Erythematosus

Introduction: Immune thrombocytopenia (ITP) is a feature of systemic lupus erythematosus (SLE) in 5-20% of cases. ITP may present at the time of SLE presentation or may precede SLE diagnosis by up to nearly a decade. Even in the absence of an SLE diagnosis, ITP patients may have a positive ANA in ~20% of cases. Some studies suggest that a positive ANA in ITP may herald chronic and more refractory disease, while other studies have not demonstrated a link between ANA+ ITP and progression to SLE. The natural history of ANA+ pediatric ITP patients remains unclear. With the concept that hydroxychloroquine may modulate progression to systemic autoimmunity, European hematologists report using hydroxychloroquine in ANA+ pediatric ITP patients with titers as low as 1:160. However, whether ANA+ ITP patients represent a distinct cohort clinically and should receive different therapies is not well established. The goals of this study were to evaluate the significance and degree of a positive ANA titer at presentation in a cohort of pediatric ITP patients. Methods: Data from a retrospective cohort of 711 ITP patients treated at Texas Children's Hospital from 2012-2022 were reviewed. Patients were defined as SLE if diagnosed as such by rheumatology. To focus on the prognostic value of an ANA upfront, the cohort was refined to include only ITP patients with ANA testing within the newly diagnosed period (0-3 months). Individuals who received IVIG prior to ANA testing were coded as having a missing ANA result given the possibility of false positive results. Being diagnosed concurrently with SLE and ITP was defined as diagnoses within 1 month of each other. Fisher's exact tests were used to compare nonparametric data with a P <0.05 defined as statistically significant. P values were corrected for multiple testing. Results: One hundred sixty-one ITP patients were included for analysis. The cohort was an average of 11.9 years-of-age with a slight female predominance (58.4%). Most of the population self-identified as White and non-Hispanic (84.7%, 45.5%). Nearly 80% had primary ITP and 66.7% were ultimately diagnosed with chronic ITP (cITP). The mean follow-up time for the cohort was 2.2 years (with a range from 0 to 12 years). Forty-seven of 161 (29.2%) individuals had a high titer ANA ³1:1280 at ITP presentation. (Figure 1) There was a statistically significant difference in ANA titer and presence of secondary ITP (P<0.001). Sixty-eight percent (n=23) of secondary ITP patients had a high titer (≥1:1280) ANA versus 20% (n=24) of the primary ITP patients. There was no difference between ANA titer and development of cITP vs. non-cITP. Of this cohort, 20 patients had an ANA tested at ITP presentation and were diagnosed with SLE by rheumatology. Most of these patients had an ANA titer ≥1:1280 (84.2%). The remaining 3 patients had titers of 1:320. Of this cohort, 9 patients had SLE and ITP diagnosed concurrently and 2 had SLE preceding ITP diagnosis by an average of 3.8 years. All 11 patients had an ANA ≥1:1280. Of the 9 who were not diagnosed with SLE at ITP presentation, 6 (66.6%) had an ANA ≥1:1280 and 3 had a titer of 1:320. The lead time to being diagnosed with SLE after presenting with ITP was 1.76 years. Discussion and Conclusions: In this cohort, high titer ANAs in newly diagnosed ITP patients were linked to development of secondary ITP and SLE. Lower titer ANAs were less common, even in those who went on to develop SLE. These findings suggest that specifically high titer ANA results may herald SLE development in pediatric ITP patients. To allow the onset of SLE to be identified earlier, individuals with a high titer ANA at ITP presentation may warrant closer follow-up to monitor for progression to systemic autoimmune disease. This subset of ITP patients could benefit from earlier institution of SLE-directed therapy, such as hydroxychloroquine. Further analysis is underway, including expansion of this analysis to multiple sites within the Pediatric ITP Consortium of North America, as well as surveying pediatric ITP treaters on their practice regarding ANA testing and management in ITP patients.

A Rare Case of Digital Ulceration and Gangrene as an Initial Presentation of Systemic Lupus Erythematosus in a Child

A Rare Case of Digital Ulceration and Gangrene as an Initial Presentation of Systemic Lupus Erythematosus in a Child