Presentation Of Systemic Lupus Erythematosus Research Articles

Lymphocytes Change Their Phenotype and Function in Systemic Lupus Erythematosus and Lupus Nephritis.

Systemic lupus erythematosus (SLE) is a complex autoimmune disease, characterized by considerable changes in peripheral lymphocyte structure and function, that plays a critical role in commencing and reviving the inflammatory and immune signaling pathways. In healthy individuals, B lymphocytes have a major role in guiding and directing defense mechanisms against pathogens. Certain changes in B lymphocyte phenotype, including alterations in surface and endosomal receptors, occur in the presence of SLE and lead to dysregulation of peripheral B lymphocyte subpopulations. Functional changes are characterized by loss of self-tolerance, intra- and extrafollicular activation, and increased cytokine and autoantibody production. T lymphocytes seem to have a supporting, rather than a leading, role in the disease pathogenesis. Substantial aberrations in peripheral T lymphocyte subsets are evident, and include a reduction of cytotoxic, regulatory, and advanced differentiated subtypes, together with an increase of activated and autoreactive forms and abnormalities in follicular T cells. Up-regulated subpopulations, such as central and effector memory T cells, produce pre-inflammatory cytokines, activate B lymphocytes, and stimulate cell signaling pathways. This review explores the pivotal roles of B and T lymphocytes in the pathogenesis of SLE and Lupus Nephritis, emphasizing the multifaceted mechanisms and interactions and their phenotypic and functional dysregulations.

Primary Presentation of Systemic Lupus Erythematosus with Pulmonary Embolism: A Case Report

Pulmonary embolism (PE) as the first clinical manifestation of systemic lupus erythematosus (SLE) is an unusual and rare presentation. This case report discusses a 30-year-old male who presented with chest pain and hypoxemia at a hospital in Dubai. Initial workup revealed elevated D-dimer levels and a positive CT pulmonary angiogram confirming PE. The patient’s medical history was unremarkable, except for a recent transient leg discomfort. Upon further investigation, hypoalbuminemia and proteinuria suggested nephrotic syndrome. A thorough autoimmune evaluation revealed elevated anti-dsDNA antibodies, leading to a diagnosis of SLE nephritis. This case emphasizes the necessity of considering SLE as a differential diagnosis for young patients with unexplained thrombotic events, even in the absence of traditional SLE symptoms. Treatment and early recognition are important in managing thrombotic complications in SLE.

A Rare Case of Pulmonary Arterial Hypertension as the Initial Presentation of Systemic Lupus Erythematosus in a Pediatric Patient

Pulmonary hypertension (PH) diagnosis can be challenging, particularly in young patients with systemic lupus erythematosus (SLE). We report a 10-year-old female presenting with suspected sinus venosus atrial septal defect (ASD) with Eisenmengerisation, later diagnosed with primary pulmonary arterial hypertension (PAH) secondary to SLE. She received vasodilators and immunosuppressants, showing partial treatment response.

A Case Report of Systemic Lupus Erythematosus Presenting as Isolated Mononeuropathy Multiplex

Mononeuropathy multiplex is a pattern of progressive sensory and motor deficits in the distribution of two or more peripheral nerves. The differential for mononeuropathy multiplex includes vasculitis, autoimmune disorders, infectious diseases, sarcoidosis, amyloidosis, cryoglobulinemia, and paraneoplastic disease. We present a case of a 42-year-old woman with hypothyroidism who presented with 1 week of ascending numbness and weakness, persistent fevers, and 3 months of constant burning pain in both feet. Her neurologic exam was notable for asymmetric weakness, loss of sensation, and diminished reflexes of her lower extremities. Other than mild fever and tachycardia, there were no systemic exam findings. Her laboratory workup was significant for elevated inflammatory markers, positive ANA, anti-ribonucleoprotein, smooth muscle antibody, and lupus anticoagulant, high anti-dsDNA titers, and low complement levels. An MRI of her lumbar spine was negative for lumbosacral nerve root pathology. Cerebrospinal fluid studies demonstrated normal cell counts, glucose, and protein and no evidence of infection. An electromyography (EMG) and nerve conduction studies (NCS) demonstrated asymmetric involvement of multiple individual peripheral nerves. Given the patient’s symptoms, asymmetric exam findings, laboratory results, and EMG/NCS findings, she was diagnosed with mononeuropathy multiplex secondary to systemic lupus erythematosus (SLE) and started on steroid and immunosuppressant therapy with symptomatic improvement. Overall, this case illustrates the unique presentation of mononeuropathy multiplex as the initial manifestation of SLE. The identification of this specific type of neuropathy may play a crucial role in the prompt diagnosis of SLE and timely initiation of treatment, preventing further complications of the disease.

Systemic lupus erythematosus: an unusual presentation of stroke - a case report from Azal hospital, Sana'a, Yemen

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder characterized by various clinical manifestations and potential multi-organ involvement. It predominantly affects women, with peak incidence during middle age. Case report: This case report describes a 36-year-old female patient who presented with right-sided body weakness, fever, and headache but maintained normal speech. The patient, with a known history of SLE, was diagnosed with an ischemic stroke. Following appropriate fluid resuscitation and medical management, the patient improved. Notably, the patient had a previous ischemic stroke four years ago while on warfarin but without antiplatelet therapy. Conclusion: Our findings suggest that stroke may be an initial presentation of SLE and that warfarin alone may not adequately protect against stroke in this patient population.

Mixed autoimmune hemolytic anemia as the initial presentation of systemic lupus erythematosus: A case report and review.

Autoimmune hemolytic anemia (AIHA) is an acquired condition caused by autoantibody mediated destruction of erythrocytes. AIHA is classified as warm or cold depending on whether the autoantibodies involved react optimally at or below body temperature (37°C), respectively. Mixed AIHA, with features of both, is rare and clinically more severe. We report a case of mixed AIHA that was found to be the presentation of systemic lupus erythematosus (SLE). Treatment with rituximab and prednisone resulted in good response. Although more commonly associated with warm AIHA, SLE can present with mixed AIHA.

Atypical Presentation of SLE

Systemic lupus erythematosus (SLE) presents with a wide array of cutaneous manifestations. The classical cutaneous features are malar rash, discoid rash, annular and papulosquamous lesions. There can also be some atypical manifestations. We present the case report of a patient, which satisfies the ACR/EULAR 2019 criteria for lupus and presented with unusual manifestation of a petechial rash.

Bilateral vocal cords palsy in adolescent Systemic Lupus Erythematosus patient

Background: Executive functions such as speech are uncommonly involved in systemic lupus erythematosus (SLE) presentation. We report a case of SLE who presented with a seizure followed by an inability to speak. Case description: A 17-year-old girl was presented with a sudden loss of voice for 2 days after a seizure following fever for 4 months. The patient was aware and could understand words but could not execute verbal sounds; the sound she produced was muffled. The generalized seizure occurred without any trigger, lasted 2 minutes, and stopped spontaneously. Fever occurred primarily at night which subsided without treatment. She also complained of severe hair loss. Patient with leucopenia, lymphopenia, anemia hemolytic, thrombocytopenia, proteinuria, hematuria, brain atrophy from magnetic resonance imaging (MRI) examination, and echocardiography revealed mild carditis and mild circumferential pericardial effusion. Immunological assay showed antinuclear antibodies (ANA) >1:1000 with nuclear homogenous, low C3 complement, and positive Anti-dsDNA. Systemic Lupus International Collaborating Clinics (SLICC) 2015 total score was 14, and the European League Against Rheumatism (EULAR/ACR) 2019 total score was 31, confirmed patient for SLE diagnosis. Further exploration from fiberoptic laryngoscopy confirmed bilateral vocal cord palsy due to SLE. Management included high dose methylprednisolone, cyclophosphamide, and speech therapy which results in gradual improvement from producing mumble sounds, then words followed by sentences with nasal sounds. Laryngeal involvement is uncommonly reported as an SLE manifestation. Management includes steroids and immunosuppressive for pharmacological and speech therapy as an exercise for vocal cord palsy. Conclusion: The patient must be continuously monitored for complete resolution of the palsy.

Impact of systemic lupus erythematosus (SLE) on outcomes following aseptic and septic revision total knee arthroplasty: an analysis of the 2005-2018 nationwide inpatient sample.

Systemic lupus erythematosus (SLE) can adversely affect surgical outcomes, and the impact on revision total knee arthroplasty (TKA) outcomes is unclear. This study aimed to explore the impact of SLE on in-patient outcomes of revision TKA. The Nationwide Inpatient Sample (NIS) database from 2005 to 2018 was searched for patients aged ≥ 18years old who received revision TKA. Patients with and without SLE were propensity score matched (PSM) at a 1:4 ratio. Associations between SLE and in-hospital outcomes were examined using regression analyses. The study included 133,054 patients, with 794 having SLE. After 1:4 PSM, data of 3,970 patients were analyzed (SLE, 794; non-SLE, 3,176). Multivariate-adjusted analyses revealed that SLE patients had a significantly higher risk of postoperative complications (adjusted odds ratio [aOR] = 1.23, 95% confidence interval [CI]: 1.05-1.44, p = 0.011), non-routine discharge (aOR = 1.22, 95% CI: 1.02-1.46, p = 0.028), major blood loss (aOR = 1.19), respiratory failure/mechanical ventilation (aOR = 1.79), acute kidney injury (AKI) (aOR = 1.47), and wound dehiscence (aOR = 2.09). SLE patients also had a longer length of hospital stay (aBeta = 0.31) and greater total hospital costs (aBeta = 6.35) compared to non-SLE patients. Among those with aseptic failure, SLE patients had a significantly higher risk of postoperative complications (aOR = 1.23) and non-routine discharge (aOR = 1.36). SLE is independently associated with worse in-hospital outcomes in patients undergoing revision TKA. This study highlights the importance of heightened vigilance and tailored perioperative management for patients undergoing major surgeries in the background of SLE. Key Points • SLE significantly increases the risk of non-routine discharge, major blood loss, respiratory failure, acute kidney injury, and wound dehiscence, in patients undergoing aseptic and septic revision TKA. • Patients with SLE experience longer hospital stays and higher hospital costs compared to those without SLE. • The study's findings highlight the necessity for healthcare providers to consider the presence of SLE as a critical factor in preoperative planning and postoperative care to improve outcomes in revision TKA patients.

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.