Presentation Of Systemic Lupus Erythematosus Research Articles

Systemic Lupus Erythematosus Presenting as Isolated Thrombocytopenia: A Case Report and Review of the Literature

Systemic Lupus Erythematosus Presenting as Isolated Thrombocytopenia: A Case Report and Review of the Literature

Generalized lymphadenopathy as an initial presentation of systemic lupus erythematosus: case report and review of the literature

BackgroundSystemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disorder with a wide array of clinical manifestations. Lymphadenopathy is occasionally observed in patients with SLE, and although it is sometimes a feature of active disease, it is rarely the initial presenting feature at the time of diagnosis.Case presentationWe report the case of a 35-year-old woman with clinical features of generalized lymphadenopathy, fatigue, fever, and malaise of approximately 3 months duration with no other constitutional disorders or systemic involvement suggestive of SLE.Despite extensive laboratory, radiological, and histological evaluations for malignancies and infectious causes of generalized lymphadenopathy, all results returned normal. About a month later, she developed inflammatory polyarthritis, with a markedly elevated erythrocyte sedimentation rate (ESR) of 120 mm/h, and a subsequent serologic workup confirmed the diagnosis of SLE.Autoimmune serology testing revealed positive results for various antibodies, including antinuclear antibody (ANA) at a titre of 1:5120, homogenous and speckled pattern, anti-Sm, anti-dsDNA, U1-snRNP, Ku, SSA-AntiRo, SSB-AntiLa, anti-SM/RNP, anti-Histone, ribosomal-p protein, and anti-nucleosome antibodies. The diagnosis of SLE was established using the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ ACR) criteria.She was on oral prednisolone, hydroxychloroquine, and calcium/vitamin D3 tablets. The patient improved remarkably, all swollen lymph nodes regressed spontaneously and she had no further need for a hematology review.ConclusionWhile similar cases have been reported in Europe and some parts of Asia, in Africa, a careful search of the literature revealed that such cases have been reported only from Egypt in North Africa and not from other parts of the continent; hence, this case report highlights the importance of recognizing atypical presentations of SLE to improve awareness and diagnosis in different regions.

Eighty-six cases of clinical characteristics and outcomes of systemic lupus erythematosus-associated macrophage activation syndrome: A meta-analysis study.

To improve our understanding of systemic lupus erythematosus (SLE)-macrophage activation syndrome (MAS). A systematic review was performed, to retrieve all those papers on patients with SLE-MAS, in individual or aggregated form. The data in each of these medical records were extracted and analyzed to identify the characteristics of SLE-MAS. A total of 86 SLE-MAS patients were included (25 males and 61 females. The mean (±standard error of the mean) age was 31.21 ± 1.694 years. MAS occurred as the initial presentation of SLE in 47 people (54.65%) and during the course of SLE in 39 (45.35%). A coinfection was reported in 23 (26.74%) patients. The mean Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score was 16.54 ± 0.9462. Overall, 10 patients (11.63%) died. The SLEDAI-2K score was higher in patients with MAS as an initial manifestation of SLE than in those where MAS occurred during the course of SLE. The proportion of patients receiving steroid pulse therapy was lower in patients with coinfections. The deceased group demonstrated lower platelet and ferritin levels. Multiple regression analysis revealed that age and thrombocytopenia were independent factors associated with poor prognosis. In receiver operating characteristic analysis, a platelet count cutoff value of ≤47 × 109/L was a predictor of poor outcome. SLE-MAS patients demonstrated high lupus activity, and lupus activity was especially higher in patients with MAS as an initial manifestation. Lupus activity was the predominant trigger of lupus MAS. Thrombocytopenia was an independent factor for poor prognosis.

Partial long-term clinical improvement after a BCG challenge in systemic lupus erythematosus-prone mice

Systemic Lupus Erythematosus (SLE) is an autoimmune disorder that causes a breakdown of immune tolerance. Current treatments mainly involve general immunosuppression, increasing the risk of infections. On the other hand, Bacillus Calmette-Guérin (BCG) has been investigated as a potential therapy for autoimmune diseases in recent years, prompting an ongoing investigation. This study aimed to evaluate the effect of BCG vaccination on early and late clinical presentation of SLE in a murine disease model. MRL/MPJ-Faslpr mice were immunized with BCG or treated with PBS as a control. The progress of the disease was evaluated at 27 days post-immunization (dpi) (early) and 56 dpi (late). Clinical parameters and proteinuria were monitored. Blood samples were collected for measurement of antinuclear antibodies (ANAs), anti-double-stranded DNA (anti-dsDNA), and cytokine determination was performed using ELISA. Samples collected from mice were analyzed by flow cytometry and histopathology. We observed a clinical improvement in BCG-treated mice, reduced proteinuria in the latter stages of the disease, and decreased TNF-α. However, BCG did not elicit significant changes in ANAs, anti-dsDNA, histopathological scores, or immune cell infiltration. BCG was only partially beneficial in an SLE mouse model, and further research is needed to determine whether the immunity induced by this vaccine can counteract lupus’s autoimmune response.

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

A Case of Confluence: Pericardial Effusion Unveiling Hashimoto’s Hypothyroidism and Atypical Systemic Lupus Erythematosus in a Young Female with Syncope

This case report presents a remarkable convergence of autoimmune diseases in a young female patient who initially presented with syncope and was subsequently diagnosed with pericardial effusion-induced cardiac tamponade. Through meticulous investigation, she was found to have Hashimoto’s thyroiditis alongside an atypical presentation of systemic lupus erythematosus (SLE), marked by a unique form of antinuclear antibody positivity. Treatment involved a combination of steroids, immunosuppressants, and thyroid hormone replacement. This intricate association between intersecting autoimmune disorders, with atypical presentations and cardiovascular manifestations, underscores the complexity of autoimmune pathology and the importance of a high index of suspicion in reaching accurate diagnoses.

A Case of Immune-Mediated Coombs-Negative Hemolytic Anemia in a Patient with Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is an auto-immune connective tissue disorder that can involve any organ in the body. The American College of Rheumatology criteria (ACR) include autoimmune hemolytic anemia with reticulocytosis as one of the criteria in the diagnosis of SLE. Anti-erythrocyte antibodies in SLE are mainly warm-type IgG. We present a case of systemic lupus erythematosus presenting with immune-mediated Coombs-negative hemolytic anemia. This case highlights the possibility of autoimmune hemolysis occurring with a negative Coombs test, where the autoantibody levels are too low to be detected by a conventional direct antiglobulin test. (DAT)

Immune thrombocytopaenia as initial presentation of systemic lupus erythematosus: A case report and literature review

Immune thrombocytopaenia as initial presentation of systemic lupus erythematosus: A case report and literature review

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.

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

Refractory focal epilepsy as a rare clinical presentation of systemic lupus erythematosus

Systemic Lupus Erythematosus (SLE) is a chronic autoimmune multisystem disease with prominent neuropsychiatric manifestations. Neuropsychiatric features are seen early in the course of disease with seizures forming one of the most frequent manifestations of Neuro Psychiatric Systemic Lupus Erythematosus (NPSLE). The mechanism of seizure is contemplated to be immune mediated. Both generalised and focal seizures are seen in SLE. They can sometimes be the only symptom marking the onset of disease in the absence of other commonly encountered clinical features of the disease. We report cases of two young females who were diagnosed and treated as drug resistant focal onset seizures with impaired awareness which later turned out to be NPSLE. Both patients showed dramatic response when initiated on immunotherapy. These cases highlight the importance of considering an immune mediated process when a young female presents with pharmacoresistant localisation related epilepsy.

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.

Atypical Systemic Lupus Erythematosus Presenting as Polymyalgia Rheumatica in an Elderly Native American Male

Atypical Systemic Lupus Erythematosus Presenting as Polymyalgia Rheumatica in an Elderly Native American Male

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 fundus changes and association with systemic conditions in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease that affects multiple organs and systems. Ocular involvement is estimated to manifest in one-third of individuals with SLE, of which lupus retinopathy and choroidopathy represent the severe subtype accompanied by vision impairment. Advancements in multimodal ophthalmic imaging have allowed ophthalmologists to reveal subclinical microvascular and structural changes in fundus of patients with SLE without ocular manifestations. Both ocular manifestations and subclinical fundus damage have been shown to correlate with SLE disease activity and, in some patients, even precede other systemic injuries as the first presentation of SLE. Moreover, ocular fundus might serve as a window into the state of systemic vasculitis in patients with SLE. Given the similarities of the anatomy, physiological and pathological processes shared among ocular fundus, and other vital organ damage in SLE, such as kidney and brain, it is assumed that ocular fundus involvement has implications in the diagnosis and evaluation of other systemic impairments. Therefore, evaluating the fundus characteristics of patients with SLE not only contributes to the early diagnosis and intervention of potential vision damage, but also holds considerate significance for the evaluation of SLE vasculitis state and prediction of other systemic injuries.

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.