Diagnosis Of Systemic Lupus Erythematosus Research Articles

Systemic lupus erythematosus combined with Wilson's disease: a case report and literature review.

Systemic lupus erythematosus (SLE) and Wilson's disease (WD) are both systemic diseases that can affect multiple organs in the body. The coexistence of SLE and WD is rarely encountered in clinical practice, making it challenging to diagnose. We present the case of a 9-year-old girl who initially presented with proteinuria, haematuria, pancytopenia, hypocomplementemia, and positivity for multiple autoantibodies. She was diagnosed with SLE, and her blood biochemistry showed elevated liver enzymes at the time of diagnosis. Despite effective control of her symptoms, her liver enzymes remained elevated during regular follow-up. Laboratory tests revealed decreased serum copper and ceruloplasmin levels, along with elevated urinary copper. Liver biopsy revealed chronic active hepatitis, moderate inflammation, moderate-severe fibrosis, and a trend towards local cirrhosis. Genetic sequencing revealed compound heterozygous mutations in the ATP7B gene, confirming the diagnosis of SLE with WD. The girl received treatment with a high-zinc/low-copper diet, but her liver function did not improve. Upon recommendation following multidisciplinary consultation, she underwent liver transplantation. Unfortunately, she passed away on the fourth day after the surgery. SLE and WD are diseases that involve multiple systems and organs in the body, and SLE complicated with WD is rarely encountered in the clinic; therefore, it is easy to misdiagnose. Because penicillamine can induce lupus, it is not recommended. Liver transplantation is indicated for patients with liver disease who do not respond to medical treatment with WD. However, further research is needed to determine the optimal timing of liver transplantation for patients with SLE complicated with WD.

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.

Maternal and fetal outcomes in pregnancy complicated by pre-existing lupus nephritis: Insights from a developing country Pakistan.

Pregnancy in women with systemic lupus erythematosus (SLE) has remained a great challenge for clinicians in terms of maternal and fetal outcomes. The outcomes in women with pre-existing lupus nephritis (LN) are variable. The impact of different classes of LN on maternal and fetal outcomes during pregnancy is not well defined, as data is very scarce, especially from the developing countries. A retrospective analysis was conducted on 52 women with 89 pregnancies. All had biopsy-proven LN. Those women who conceived at least 6months after the diagnosis were included. The analysis was conducted between July 1998 and June 2018 at Sindh Institute of Urology and Transplantation (SIUT), evaluating the outcomes for both the mother and the fetus with a minimum follow-up of 12months after child birth. The mean maternal age at SLE diagnosis was 21.45 ± 6years and at first pregnancy was 26.49 ± 5.63years. The mean disease duration was 14.02 ± 19.8months. At conception, 47 (52.8%) women were hypertensive, 9 (10%) had active disease while 38 (42.7%) and 42 (47.2%) were in complete and partial remission, respectively. A total of 17 (19.1%) were on mycophenolate mofetil (MMF), which was switched to azathioprine (AZA). Out of 89 pregnancies, 56 (62.9%) were successful, while 33 (37.07%) had fetal complications like spontaneous abortion, stillbirth, perinatal death, and intrauterine growth retardation (IUGR). There were more vaginal deliveries (33 [58.92%]) than caesarean sections (23 [41.07%]). Renal flare was observed in 33 (37.1%) women while 15 (16.9%) developed pre-eclampsia. Proliferative LN was found in 56 (62.9%) cases, but no significant differences were found in maternal and fetal outcomes in relation to LN classes (p = .58). However, disease outcomes at 12months were significantly poor in those with active disease at the time of conception (p < .05). There was only one maternal death. A total of 10 (11.2%) women showed deterioration in renal function and 5 (5.6%) were dialysis-dependent at 12months. The maternal and fetal outcomes in pre-existing LN depend on the disease activity at the time of conception. No correlation was found between International Society of Nephrology/Renal Pathology Society (ISN/RPS) classes of LN and adverse disease and pregnancy outcomes.

Enrolment of the first paediatric cohort into the Australian lupus registry and biobank: A single-centre experience.

We aim to report on the feasibility of establishment of the first paediatric cohort as part of the longitudinal database of the Australian Lupus Registry and Biobank (ALRB) and to describe the enrolment data with a focus on clinical characteristics, serological data, treatment strategies and patient/parent-reported outcome measures. All patients under the age of 18years with a diagnosis of systemic lupus erythematosus (SLE) attending the paediatric rheumatology service of a single, tertiary hospital were identified. Patients were enrolled in the ALRB if they met ≥4/11 of the American College of Rheumatology (ACR) 1997 SLE classification criteria or the Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria. Enrolment data including demographics, clinical characteristics, serological profiles, disease activity and damage assessments were recorded. Peds-QL Rheumatology and General Modules were used to assess patient and parent-reported outcomes. Twenty-seven patients were eligible for inclusion, with 26 patients (96%) consenting for enrolment. Twenty-five patients (92%) consented for biobanking. Twenty patients (77%) were female. The median age at enrolment was 16years (interquartile range (IQR) 13.7, 17.4). The median disease duration from diagnosis was 3.2years (IQR 1.4, 5.3). Sixteen patients (62%) had synovitis, 16 (62%) had cutaneous involvement, 4 (15%) had serositis, 17 (65%) had haematological involvement and 7 (27%) had renal involvement at enrolment. Nineteen patients (73%) were prescribed at least two disease-modifying anti-rheumatic medications (DMARDs). Hydroxychloroquine (n = 22, 85%) and mycophenolate mofetil (n = 9, 35%) were the most commonly prescribed DMARDs. The median SLEDAI-2K score was 2 (IQR 2, 4). Six patients (23%) had active disease (SLEDAI-2K ≥6) at enrolment. Three patients (11.5%) had reported damage using the SLICC/ACR Damage Index. Twenty-three children (88%) and eighteen parents (69%) completed the Paediatric Quality of Life Inventory. Quality of life scores reported across domains of physical, emotional, social and school functioning at enrolment were comparable to previously studied paediatric cohorts with SLE and other chronic diseases. We have established our centre as the first paediatric participating site of the ALRB, providing contemporary data on the clinical characteristics, serological profile and health-related quality of life outcomes of Australian children with SLE. Paediatric involvement with this national registry will provide a unique perspective for future clinical and scientific research. Collection of Australian-specific paediatric longitudinal data will also enable a broader understanding of SLE within a multicultural Australian population.

The impact of Libman-sacks endocarditis on inpatient outcomes with systemic lupus erythematosus: A retrospective study.

The existing literature offers limited insights into the influence of Libman-Sacks Endocarditis (LSE) on inpatient outcomes in individuals with Systemic Lupus Erythematosus (SLE). This study aimed to explore the characteristics and prognosis of SLE patients with LSE and the impact of LSE in patients with SLE on inpatient outcomes including inpatient mortality, length of stay, acute heart failure, atrial fibrillation, and cerebrovascular accidents (CVA). This study included adult patients who were hospitalized with SLE between the years 2019 and 2020, using the National Inpatient Sample (NIS) database. The total number of patients with a diagnosis of SLE in the years 2019 and 2020 in the NIS database was 150,411. Of those, 349 had a diagnosis of LSE. The study population was divided into two groups: one group with SLE and LSE, and another group with SLE but without LSE. Caucasians made up 54.9% of the patients with a diagnosis of SLE in our patient population, while African Americans made up 26.9% and the Hispanics accounted for 12.2%. Of patients with LSE, Caucasians and African Americans represented 42.9% each. Patients with a diagnosis of LSE had a higher inpatient mortality than those with SLE without LSE (aOR: 9.74 CI 1.12-84.79, p 0.04). Patients with SLE with LSE were more likely to have acute heart failure than those without LSE, although this was not statistically significant (aOR 1.18 CI 0.13-11.07, p 0.88). Similarly, patients with SLE with LSE were more likely to have atrial fibrillation than those without LSE (aOR 4.45 CI: 0.77-25.57, p 0.10). CVAs were significantly higher in SLE patients with LSE than those without LSE (aOR 141.43 CI 16.59-1205.52, p < .01). Patients who develop LSE were found to have significantly higher risks of inpatient mortality and cerebrovascular accidents. Early and precise detection of LSE in such patients may ensure timely intervention and prevention of the associated adverse outcomes. Further studies may attempt to develop screening methods for detection of LSE to effectively reduce morbidity and mortality associated with SLE.

Autoimmune responses and therapeutic interventions for systemic lupus erythematosus: a comprehensive review.

Systemic Lupus Erythematosus (SLE) or Lupus is a multifactorial autoimmune disease of multiorgan malfunctioning of extremely heterogeneous and unclear etiology that affects multiple organs and physiological systems. Some racial groups and women of childbearing age are more susceptible to SLE pathogenesis. Impressive progress has been made towards a better understanding of different immune components contributing to SLE pathogenesis. Recent investigations have uncovered the detailed mechanisms of inflammatory responses and organ damage. Various environmental factors, pathogens, and toxicants, including ultraviolet light, drugs, viral pathogens, gut microbiome metabolites, and sex hormones trigger the onset of SLE pathogenesis in genetically susceptible individuals and result in the disruption of immune homeostasis of cytokines, macrophages, T cells, and B cells. Diagnosis and clinical investigations of SLE remain challenging due to its clinical heterogeneity and hitherto only a few approved antimalarials, glucocorticoids, immunosuppressants, and some nonsteroidal anti-inflammatory drugs (NSAIDs) are available for treatment. However, the adverse effects of renal and neuropsychiatric lupus and late diagnosis make therapy challenging. Additionally, SLE is also linked to an increased risk of cardiovascular diseases due to inflammatory responses and the risk of infection from immunosuppressive treatment. Due to the diversity of symptoms and treatment-resistant diseases, SLE management remains a challenging issue. Nevertheless, the use of next-generation therapeutics with stem cell and gene therapy may bring better outcomes to SLE treatment in the future. This review highlights the autoimmune responses as well as potential therapeutic interventions for SLE particularly focusing on the recent therapeutic advancements and challenges.

SLE diagnosis research based on SERS combined with a multi-modal fusion method

As artificial intelligence technology gains widespread adoption in biomedicine, the exploration of integrating biofluidic Raman spectroscopy for enhanced disease diagnosis opens up new prospects for the practical application of Raman spectroscopy in clinical settings. However, for systemic lupus erythematosus (SLE), origin Raman spectral data (ORS) have relatively weak signals, making it challenging to obtain ideal classification results. Although the surface enhancement technique can enhance the scattering signal of Raman spectroscopic data, the sensitivity of the SERS substrate to airborne impurities and the inhomogeneous distribution of hotspots degrade part of the signal. To fully utilize both kinds of data, this paper proposes a two-branch residual-attention network (DBRAN) fusion technique, which allows the ORS to complement the degraded portion and thus improve the model's classification accuracy. The features are extracted using the residual module, which retains the original features while extracting the deep features. At the same time, the study incorporates the attention module in both the upper and lower branches to handle the weight allocation of the two modal features more efficiently. The experimental results demonstrate that both the low-level fusion method and the intermediate-level fusion method can significantly improve the diagnostic accuracy of SLE disease classification compared with a single modality, in which the intermediate-level fusion of DBRAN achieves 100% classification accuracy, sensitivity, and specificity. The accuracy is improved by 10% and 7% compared with the ORS unimodal and the SERS unimodal modalities, respectively. The experiment, by fusing the multimodal spectral, realized rapid diagnosis of SLE disease by fusing multimodal spectral data, which provides a reference idea in the field of Raman spectroscopy and can be further promoted to clinical practical applications in the future.

363 The Rapidity of Systemic Lupus Erythematosus Diagnosis in Membranous Nephropathy with Full House Staining

363 The Rapidity of Systemic Lupus Erythematosus Diagnosis in Membranous Nephropathy with Full House Staining

Clinical and laboratory characteristics of early-onset and delayed-onset lupus nephritis patients: A single-center retrospective study.

Lupus nephritis (LN) manifests systemic lupus erythematosus (SLE) and is characterized by various clinical and laboratory features. This study aimed to comprehensively evaluate the characteristics of LN patients according to the time of LN diagnosis: early-onset (LN diagnosed within one year from SLE diagnosis) vs. delayed-onset (LN diagnosed more than one year after SLE diagnosis). We conducted a retrospective analysis of medical records from all SLE patients treated at the University Hospital in Kraków, Poland, from 2012 to 2022. We collected data on demographic, clinical, and laboratory characteristics, including histological findings, treatment modalities, and disease outcomes. Statistical analyses were performed to identify factors impacting LN development and prognosis. Among 331 LN patients, early-onset was diagnosed in 207 (62.54%) and delayed-onset was documented in 122 cases (36.86%). In 2 (0.6%) LN cases, the time of first kidney manifestation in the SLE course was unknown. Delayed-onset LN had a higher female-to-male ratio and younger age at SLE diagnosis. This group was associated with more severe clinical manifestations. In turn, studied subgroups did not differ in internist comorbidities, kidney histopathology, and family history regarding autoimmune diseases. Delayed-onset LN exhibited a higher frequency of anti-dsDNA, anti-Smith, anti-Ro, anti-RNP, and anti-cardiolipin IgG autoantibodies. During a 14-year follow-up period, 16 patients died. Mortality rate and causes of death were comparable in both analyzed subgroups. More severe clinical manifestations in delayed-onset LN prompt strict monitoring of non-LN SLE patients to diagnose and treat kidney involvement early. Also, recognizing the higher frequency of autoantibodies such as anti-dsDNA or anti-Smith in delayed-onset LN underscores the potential value of autoantibody profiling as a diagnostic and prognostic tool.

Methyl-Prednisolone Pulses and Prolonged Remission in Systemic Lupus Erythematosus: A Propensity Score Analysis of the Longitudinal Lupus-Cruces-Bordeaux Inception Cohort.

To analyze the effect of methyl-prednisolone pulses (MP), given during the first year after the diagnosis of systemic lupus erythematosus (SLE), in achieving prolonged remission according to the degree of lupus activity at presentation. Observational study of routine clinical care data of the Lupus Cruces-Bordeaux cohort. The endpoint was prolonged remission, i.e. during 5 consecutive yearly visits. The effect on remission of MP during the first year was analyzed in the whole cohort and according to the baseline SLEDAI-2K score: <6, 6-12 and >12, reflecting mild, moderate and severe activity, respectively. For adjustment, logistic regression with propensity score (PE) and other therapeutic covariates was performed. Two hundred thirty-three patients were included. Prolonged remission was achieved by 132 (57%) patients. MP were associated with prolonged remission (PE-adjusted OR 2.50, 95%CI 1.04-623, p=0.042). A strong clinical effect was seen among patients with moderate (adjusted OR 5.28, 95%CI 1.27-21.97, p=0.022) and with moderate-severe SLE activity (adjusted OR 4.07, 95%CI 1.11-14.82, p=0.033). The administration of MP resulted in reduced average doses of prednisone during the first year among patient with moderate (mean 6.6 vs 10.2 mg/d, p=0.017) and severe activity (mean 14 vs. 28 mg/d, p= 0.015). The odd of prolonged remission was increased by longer-term use of HCQ and decreased by higher initial doses of prednisone. This study supports the use of MP to induce prolonged remission in patients with SLE, particularly in those with moderate and severe activity. The extended use of HCQ also contributes to achieve prolonged remission. This article is protected by copyright. All rights reserved.

A Prospective Observational Study of Disease Severity and Mortality in Hispanic American Patients With Systemic Sclerosis.

To characterize disease manifestations in Hispanic American patients with systemic sclerosis (SSc) in comparison with non-Hispanic White and Black patients. Longitudinal clinical characteristics were collected prospectively in the Genetics versus Environment in Scleroderma Outcome Study cohort. All patients fulfilled the classification criteria for SSc and had a disease duration less than five years at enrollment. A cohort of 427 patients, consisting of 124 Hispanic, 220 non-Hispanic White, and 83 non-Hispanic Black participants were examined. At enrollment, Hispanic patients were significantly younger but had longer disease duration, higher frequency of U1-RNP positivity as well as concurrent systemic lupus erythematosus (SLE) diagnosis, and lower income and educational levels in comparison to non-Hispanic White patients. Compared with non-Hispanic Black patients, Hispanic patients had more frequently limited cutaneous involvement and anticentromere antibodies. In the longitudinal analysis, Hispanic patients had significantly lower forced vital capacity percents predicted (point estimate, -9.3%; P < 0.001) than non-Hispanic White but not Black patients. Hispanic patients had similar longitudinal modified Rodnan Skin Scores like non-Hispanic White patients but lower measurements than non-Hispanic Black patients (point estimate, -3.2; P = 0.029). Hispanic patients had significantly higher serially obtained perceived functional disability scores than White patients (point estimate, 0.29; P < 0.001). Hispanic patients also had higher mortality rates than White Americans even after adjustment for age, gender, and socioeconomic statuses. Hispanic patients have higher likelihood of having U1-RNP positivity and SLE overlap, more severe restrictive lung disease, as well as higher rate of mortality than non-Hispanic White patients.

Chronic interstitial lung disease associated with systemic lupus erythematosus: A multicentric study of 89 cases.

Chronic interstitial lung disease (ILD) occurs rarely with systemic lupus erythematosus (SLE) as compared with other connective tissue diseases. This multicentric retrospective study of patients with SLE-ILD from the OrphaLung and French SLE networks during 2005-2020 aimed to describe the characteristics of patients with SLE-ILD and analyse factors associated with prognosis. We analysed data for 89 patients with SLE-ILD (82 women, 92.1%) (median age at SLE diagnosis: 35 years [interquartile range 27-47]). All patients met the 2019 EULAR/ACR criteria for the diagnosis of SLE. Forty two (47.2%) patients were positive for anti-ribonuclear protein antibodies and 45 (50.6%) for anti SSA/Ro antibodies. A total of 58 (65.2%) patients had another connective tissue disease: Sjögren's syndrome (n = 33, 37.1%), systemic sclerosis (n = 14, 15.7%), inflammatory myopathy (n = 6, 6.7%), or rheumatoid arthritis (n = 6, 6.7%). ILD was diagnosed along with SLE in 25 (28.1%) patients and at a median of 6 (0-14) years after the SLE diagnosis. The most frequent CT pattern was suggestive of non-specific interstitial pneumonia (n = 41, 46.0%) with or without superimposed organizing pneumonia. After a median follow-up of 86.5 [39.5-161.2] months, 18 (20.2%) patients had died and 6 (6.7%) underwent lung transplantation. The median 5-year and 10-year transplantation-free survival were 96% (92-100) and 87% (78-97). In total, 44 (49.4%) patients showed ILD progression. Cutaneous manifestations and Raynaud's phenomenon were associated with better survival. Only forced vital capacity was significantly associated with survival and ILD progression. ILD is a rare manifestation of SLE with good overall prognosis but with possible risk of ILD progression. Patients with SLE-ILD frequently have another connective tissue disease.

A Case Report of a Male with Systemic Lupus Erythematous with First Presentation of Annular Macular Itchy Rashes on his Extremities

Background: Systemic lupus erythematosus (SLE) is an idiopathic autoimmune disease which impacts multiple organs and has various clinical presentations. Among all the organs, the skin is the most frequently affected.&#x0D; Case Presentation: This case report presents a 34-year-old male who presented with severe, itchy, and painful rashes over his right arm and both legs, along with a history of pigmented skin lesions. Initially, the patient was given corticosteroid creams by a dermatologist, but the condition progressed, requiring further investigation. The patient's medical history revealed hypertension and, on review, signs of rashes, fatigue, and arthralgias were noted.&#x0D; Physical examination indicated mild swelling and tenderness in both knees. Additionally, an examination revealed his skin was warm, he had fast capillary refill (&lt;2 seconds), and he had an erythematic rash on the right arm and both legs. A nodular erythema rash was seen on the left leg, along with pigmented skin lesions on the right leg, including a bigger lesion measuring approximately 2.5 cm. Laboratory results indicated increased creatinine, low white blood cell count, anemia, and abnormal protein levels, raising doubts about SLE. Further tests confirmed a raised level of autoantibodies, low complement levels, and positive lupus-related antibodies.&#x0D; Later, the patient was referred to a rheumatologist and an SLE diagnosis was confirmed based on laboratory results and hematological manifestations, mucocutaneous involvement, and lupus nephritis. Treatment initially started with hydroxychloroquine and prednisolone, then tests were repeated and the patient underwent a renal biopsy that revealed focal segmental glomerulosclerosis. He was again prescribed methylprednisolone and prednisolone for three days. The patient's condition resolved and he made a successful recovery.&#x0D; Conclusion: This case demonstrates the multiple clinical manifestations of SLE, the necessity of in-depth investigations, and the requirement for multidisciplinary care to address the intricacies of organ involvement in the disease.

BCD020 rituximab bioanalog compared to standard treatment in juvenile systemic lupus erythematosus: The data of 12 months case-control study.

Systemic lupus erythematosus (SLE) is the most frequent and serious systemic connective tissue disease. Nowadays there is no clear guidance on its treatment in childhood. There are a lot of negative effects of standard-of-care treatment (SOCT), including steroid toxicity. Rituximab (RTX) is the biological B-lymphocyte-depleting agent suggested as a basic therapy in pediatric SLE. To compare the benefits of RTX above SOCT. The data from case histories of 79 children from the Saint-Petersburg State Pediatric Medical University from 2012 to 2022 years, were analyzed. The diagnosis of SLE was established with SLICC criteria. We compared the outcomes of treatment of SLE in children treated with and without RTX. Laboratory data, doses of glucocorticosteroids, disease activity measured with SELENA-SLEDAI, and organ damage were assessed at the time of initiation of therapy and one year later. Patients, treated with RTX initially had a higher degree of disease activity with prevalence of central nervous system and kidney involvement, compared to patients with SOCT. One year later the disease characteristics became similar between groups with a more marked reduction of disease activity (SELENA-SLEDAI activity index) in the children who received RTX [-19 points (17; 23) since baseline] compared to children with SOCT [-10 (5; 15.5) points since baseline, P = 0.001], the number of patients with active lupus nephritis, and daily proteinuria. During RTX therapy, infectious diseases had three patients; one patient developed a bi-cytopenia. RTX can be considered as the option in the treatment of severe forms of SLE, due to its ability to arrest disease activity compared to SOCT.

Comparison of the SLE Risk Probability Index (SLERPI) scale against the European League Against Rheumatism/American College of Rheumatology (ACR/EULAR) and Systemic Lupus International Collaborating Clinics (SLICC) criteria.

Timely diagnosis and proper recognition of Systemic Lupus Erythematosus (SLE) is essential to establish early management in inpatients and outpatients. There are different classification scales to identify SLE, which include various clinical and serological aspects. In 2021, the SLE Risk Probability Index (SLERPI) was published, which focuses predominantly on the clinical characteristics of patients with suspected SLE and uses a simple algorithm for early recognition of the disease. The aim of this study is to compare the European League Against Rheumatism/American College of Rheumatology (ACR/EULAR) classification criteria, the Systemic Lupus International Collaborating Clinics (SLICC) criteria, and the SLERPI criteria in a cohort of Colombian patients with SLE and to analyze the correlations observed between their absolute scores. A registry of SLE patients from two referral hospitals in Bogotá, Colombia, was used. 2021 SLERPI, 2019 ACR/EULAR, and 2012 SLICC scores were calculated for each patient and the correlations found between the scales were analyzed. The sensitivities of each were compared, and frequency analyses were conducted among different clinical and laboratory variables. Between 2016 and 2019, 146 patients diagnosed with SLE were registered, including inpatients and outpatients. The median age was 36years (interquartile range 26-51), and 82.2% were women. According to the SLERPI criteria, a high prevalence of antinuclear antibodies (92%), immunological disorders (71%), and arthritis (64%) were observed. The most used treatments were corticosteroids (87.6%) and chloroquine (67.8%). A Spearman evaluation analysis was performed, with a moderately strong correlation of 0.76 (p = .000) between the SLERPI and ACR/EULAR scales and very strong correlation of 0.80 (p = .000) between the SLERPI and SLICC. Patients classified with SLE according to the SLERPI scale exhibited a higher incidence of hematological compromise, along with elevated levels of serological markers such as anti-DNA antibodies. Additionally, this group more commonly received treatments involving corticosteroids and azathioprine, and displayed a higher prevalence of hypertension. The SLERPI scale could be useful in the diagnosis of SLE, especially in early stages, given its good correlation with other classification scales and its good sensitivity.

Comparison of Clinical Features, Treatment and Outcomes of Lupus Nephritis Between Patients With Late- and Early-Onset Systemic Lupus Erythematosus: A Controlled Study.

Studies have found that late-onset systemic lupus erythematosus (SLE) patients (age at diagnosis ≥ 50 years) had less severe disease and milder clinical course, but with higher organ damage and mortality rate than early-onset ones (age at diagnosis < 50 years). Unfortunately, direct comparison of renal manifestations and treatment outcomes between late- and early-onset SLE patients has been determined rarely. This study aimed to compare lupus nephritis (LN) manifestations, treatment, and outcomes between late- and early-onset in SLE patients. Medical records of SLE patients in a lupus cohort at a tertiary care university hospital, seen between January 1994 and June 2020, were reviewed. Late- and early-onset patients were matched with year at SLE diagnosis at a ratio of 1:2 (62 and 124 patients, respectively). Those with LN were identified and analyzed. At SLE onset and end of the study, LN was identified in 29 and 33 late-onset patients, respectively, and 58 and 90 early-onset patients, respectively. At the end of the study, there were 39 and 214 LN flares in late- and early-onset patients, respectively: giving an incident rate (IR) (95% confidence interval (CI))/100 person-years of LN and active LN flares of 2.00 (0.75 - 5.33) vs. 6.11 (4.32 - 8.64), P = 0.020, and 5.78 (2.75 - 12.12) vs. 18.28 (13.93 - 24.00), P = 0.001, respectively. Late-onset patients received a higher proportion of moderate- to high-dose corticosteroids, but fewer immunosuppressive drugs. In all LN flares, no difference existed between the two groups in serum creatinine, degree of proteinuria, and proportion of patients with nephrotic range proteinuria or rapidly progressive glomerulonephritis, and outcomes in terms of complete, partial or no-remission were similar between them. Mortality rate was higher in late-onset patients (27.27% vs. 6.67%, P = 0.004). This matched controlled study of year at SLE diagnosis showed that late-onset SLE patients had lower prevalence of LN and LN flares. Although they received fewer immunosuppressive drugs, their renal manifestations and treatment outcomes were no different from those in early-onset patients.

A case of non-lupus full-house nephropathy diagnosed by kidney biopsy but observed IgA nephropathy on second biopsy.

We describe a case of full-house nephropathy without any underlying disease, including systemic lupus erythematosus. A 40-year-old woman was referred to our hospital with mild proteinuria and microscopic hematuria. The patient was diagnosed with immune complex-mediated glomerulonephritis with a predominant mesangioproliferative pattern based on renal histopathological results using full-house immunofluorescence staining. She showed no clinical criteria for the diagnosis of systemic lupus erythematosus, except for kidney disorders, and tested negative for antinuclear antibodies throughout her clinical course. However, in the second kidney biopsy, no C1q or C4 were detected in the immunofluorescence study, suggesting an immunoglobulin A nephropathy-like pattern. The patient responded favorably to corticosteroid treatment. We found a heterozygous CFHR3-CFHR1 deletion. The association between full-house nephropathy and CFHR3-CFHR1 deletion is unknown, but its influence on the histological pattern in our case is suspected. This indicates the diversity in the pathogenesis of non-lupus full-house nephropathy and warrants further investigation.

Life expectancy and death pattern associated with systemic lupus erythematosus diagnosis in Brazil between 2000 and 2019.

to evaluate the main factors associated with mortality and determine the life expectancy of SLE patients between 2000 and 2019 years in Brazil. death data related to SLE available in the Brazilian Unified Health System (SUS) (DATASUS) were evaluated in all Brazilian states. Three groups of death causes potentially associated from SLE were evaluated: cardiovascular and kidney diseases and infections. The main causes of death associated with SLE were infection and kidney disease. Most SLE patients died between 19 and 50 years of age. Deaths associated with kidney disease were proportionally higher than in the general population with progressive decrease during the period. Instead, there have been an increase in the proportion of deaths due to infections both in SLE and in the general population. SLE patients presented higher mortality compared to the general population matched for sex and age and the main causes associated with death were infection and kidney disease. Public health policies that promote early diagnosis, treatment and prevention of damage are necessary to reduce morbidity and mortality in SLE patients.

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.  

Expression profile of long-noncoding RNAs MIR31HG, NKILA, and PACER in systemic lupus erythematosus patients

ObjectivesGrowing evidence suggests that systemic lupus erythematosus (SLE), an organ-damaging systemic autoimmune illness, may be influenced by long-noncoding RNAs (lncRNAs). This study aimed to assess the relative expression of lncRNAs (MIR31HG, NKILA, and PACER) in patients with SLE to evaluate their role in the disease. Design and MethodsThis study involved 70 patients with SLE and 70 apparently healthy control subjects. The expression levels of lnc-MIR31HG, NKILA, and PACER were quantified using real-time PCR. ResultsLnc-MIR31HG, NKILA, and PACER were significantly upregulated in SLE cases compared to controls (P < 0.001). ROC curve analysis revealed a 91.43 % sensitivity of PACER for the diagnosis of SLE at a cutoff point of > 1.46, followed by NKILA with 90 % sensitivity at a cutoff point of > 1.16, and MIR31HG with 85.71 % sensitivity at a cutoff point of > 1.43. MIR31HG had the highest sensitivity for the diagnosis of lupus nephritis (86.67 %) at a cutoff point of > 7.19, then NKILA with 80 % sensitivity at a cutoff point of > 8.12, and finally PACER expression with 73.33 % sensitivity at a cutoff point of > 18.19. Moreover, MIR31HG and NKILA revealed a significant correlation with albumin/creatinine ratio, estimated glomerular filtration rate, and the SLEDAI score. Regression analysis revealed the potential roles of MIR31HG, NKILA, and PACER expression as predictors for SLE. ConclusionAn upregulated lncRNA panel (MIR31HG, NKILA, and PACER) could play a role in the pathogenesis and, hence, the predispositiontoSLE. MIR31HG and NKILA can serve as prognostic markers significantly linked with disease activity.