ObjectivesThe aim of this study was to compare the clinicopathological characteristics and outcomes of lupus nephritis (LN) between late-onset and early-onset systemic lupus erythematosus (SLE) patients.MethodsWe reviewed the clinical, serological and histological characteristics of all patients with LN admitted to our nephrology unit between 2007 and 2024. Our patients were divided into two groups according to their age at diagnosis: Early-onset SLE (younger than 50years) and late-onset SLE (50years or older).ResultsA total of 231 patients were recruited, of whom 43 had late-onset SLE and 188 had early-onset SLE. The mean age at diagnosis of SLE was 58.36 ± 7.61years in the late-onset SLE group and 29.23 ± 9.03years in the early-onset SLE group. There was no difference in the time from SLE diagnosis to LN. Compared with early-onset group, late-onset group had a higher prevalence of discoid rash but a lower frequency of leukopenia. Late-onset patients had higher serum creatinine levels and lower prevalence of anti-SSA and anti-RNP antibodies. The frequency of class VI LN was statistically significantly higher in the late-onset group. The use of oral corticosteroids, hydroxychloroquine and immunosuppressive drugs was significantly lower in late-onset SLE patients than in early-onset SLE patients. The latter were significantly less likely to progress to chronic kidney disease.ConclusionOur results indicate that SLE patients have different clinical, serological and histological manifestations depending on the age at onset of the disease. Late-onset SLE patients are more likely to have rheumatoid arthritis at onset. They have more severe chronic renal lesions and a worse renal outcome.

ObjectiveSystemic lupus erythematosus (SLE) is complexed with multi systemic involvements associated with genetic and environmental factors. Herein, we delve into the molecular mechanism behind COVID-19 vaccine-induced SLE.MethodsTo elucidating the molecular mechanism, we employed the whole genome sequencing (WGS) and immune cell RNA sequencing of CD4+ T, CD8+ T, and B cells.ResultsOur findings suggest that in genetically predisposed individuals, COVID-19 vaccination might provoke SLE by triggering host-harbored virus signaling and cytokine pathway-associated immune response.ConclusionsThis study deepens the underlying mechanism and highlights the importance of evaluating the genetic susceptibility to autoimmune disease before COVID-19 vaccination.

IntroductionLupus nephritis (LN), a severe organ impairment associated with systemic lupus erythematosus (SLE), significantly affects patient prognosis. Despite therapeutics advances, up to 15% of patients progress to end-stage kidney disease (ESKD) within 15 years, with kidney transplantation emerging as the preferred renal replacement therapy. Conversely, belimumab has shown promise in enhancing remission rates and improving renal outcomes in LN. however, there is little information in the literature regarding the use of belimumab in kidney transplant patients and its potential interaction with other immunosuppressive therapies.MethodsThis report presents three clinical cases of renal transplant recipients with LN who received belimumab following transplantation to manage their systemic disease The aim of this case report was to evaluate the efficacy and safety of belimumab administration in renal transplant patients.ResultsBelimumab successfully discontinued or reduced the dose of glucocorticoids, resolved the clinical manifestations of SLE and maintained stable renal function throughout follow-up.ConclusionThese cases highlight belimumab's potential as an effective and safe therapeutic option in managing extrarenal SLE symptoms and reducing glucocorticoid dependence in kidney transplant recipients. Although limited data exists, these findings align with previous evidence supporting belimumab's safety and efficacy in LN.

BackgroundChildhood onset systemic lupus erythematosus (cSLE) exhibits more severe and more aggressive clinical features compared to adult onset SLE. We investigated factors associated with long-term outcomes among the initial parameters at the time of diagnosis in cSLE.Patients and methodsThis study included patients initially diagnosed with cSLE who were less than 18 years old between January 2009 and December 2021. We excluded patients with prior diagnoses, those transferred from another hospitals, individuals with clinical findings related to infection or post-transplantation, and underlying diseases. A retrospective review of electronic medical records was conducted to gather initial laboratory data, and assess clinical manifestations, including SLE disease activity index-2K (SLDAI-2k). We analyzed parameters associated with survival, as well as events such as flare, complications, and new organ involvement.ResultsA total of 109 patients were enrolled in this study. The mean age was 14.4 ± 2.3 years old, and the female to male ratio was 7.4:1. Twenty-eight patients (25.7%) were diagnosed during the pre-pubertal period. The overall survival rate was 92.9 % (median: 5.0 years). The causes of death were intractable macrophage activation syndrome (n = 2), disease related state (n = 2), and infection (n = 2). The factors related to survival were elevated C-reactive protein (CRP, p = .017, HR: 2.396, 95% CI: 1.165 ∼4.926) in multi-variate analysis, although there were association with CRP, SLEDAI-2K, and false positivity for syphilis (p < .05) in univariate analysis. The event free survival was 10.4% and was related to SLEDAI-2K, anti-Smith antibody, and false positivity for syphilis (p < .05) in univariate analysis. In multivariate analysis, factors associated with event were SLEDAI-2K (p = .035, HR: 2.82, 95% CI: 1.078∼7.375) and, anti-Smith antibody (p = .019, HR: 3.262, 95% CI: 1.218∼8.741).ConclusionInitial SLEDAI-2K and bio-markers for immune response were related to survival and events during follow-up. Clinicians should focus on initial disease activity and laboratory parameters when predicting long-term outcomes in cSLE.

IntroductionPrevious studies indicate that the prognosis of systemic lupus erythematosus (SLE) is poor in Africa. We surveyed African physicians for their perceptions of factors that impact negatively on optimal SLE care and interventions to improve care in Africa.MethodsA cross-sectional, online survey of African dermatologists, rheumatologists, nephrologists, and internists was conducted.ResultsResponses from 226 respondents mostly from West Africa and East Africa, majority practicing in university and state-funded hospitals showed that the commonest reasons for late diagnosis of SLE in Africa were lack of awareness of the disease amongst primary care doctors (92.4%), financial constraints (80.3%) and lack of access to health care (62.5%). Consulting traditional healers, the belief of bewitchment, lack of availability of diagnostic tests and concomitant chronic infections, (tuberculosis and HIV) were also perceived to have resulted in diagnostic delays, especially amongst East and West African respondents. The overwhelming majority (>90%) of respondents felt that increased health care and financial resources was the top priority to improving SLE care in Africa. Continuing medical education for generalists, training of specialists and patient education and awareness programs were considered less important interventions to improve SLE care.ConclusionThe study reveals that the primary reasons for late diagnosis of SLE in Africa are lack of awareness among primary healthcare doctors and financial constraints. Thus, the need for greater financial resources, especially for appropriate medications, medical education and improving patient understanding of the disease through support groups, to improve SLE care and outcomes in Africa.

We report two cases of patients with refractory discoid lupus erythematosus (DLE) and dark skin phototypes who experienced progressive perifollicular repigmentation of depigmented lesions under anifrolumab therapy, with sustained improvement over 18months. These observations highlight a potential novel effect of anifrolumab on cicatricial dyspigmentation in DLE. This effect may represent a valuable therapeutic option for DLE-related damage, particularly in patients with darker skin types.

ObjectiveTo investigate the effects of belimumab (BEL) on systemic lupus erythematosus (SLE) mucocutaneous and vasculitis manifestations.MethodsThis post hoc, integrated Belimumab Summary of Lupus Efficacy (Be-SLE) analysis pooled data from five international Phase 3, randomized, placebo (PBO)-controlled BEL trials (BLISS-52 [NCT00424476; conducted in 2007–2009], BLISS-76 [NCT00410384; 2007–2009], BLISS-SC [NCT01484496; 2011–2015], North East Asia [NCT01345253; 2011–2015], EMBRACE [NCT01632241; 2013–2018]). Adults with active SLE and Safety of Estrogens in Lupus Erythematosus National Assessment-SLE Disease Activity Index (SELENA-SLEDAI) score ≥6 (BLISS-52, BLISS-76) or ≥8 (BLISS-SC, North East Asia, EMBRACE), randomized to BEL (10 mg/kg/month intravenously or 200 mg/week subcutaneously) or PBO, plus standard therapy (ST) were included. Mucocutaneous and vasculitis manifestations (listed below) were measured (baseline and every 4 weeks) for 52 weeks using SELENA-SLEDAI and British Isles Lupus Assessment Group (BILAG).ResultsOf 3086 patients (BEL, n = 1869; PBO, n = 1217), 85% (BEL and PBO) by SELENA-SLEDAI and 58% (BEL) and 62% (PBO) by BILAG (moderate or severe activity) had mucocutaneous manifestations, and <10% had vasculitis at baseline. At Week 52, significantly more BEL-treated than PBO-treated patients demonstrated improvements in SELENA-SLEDAI (59% vs 49%; p < .0001) and BILAG (54% vs 43%; p < .0001) mucocutaneous domains. Significant differences between-treatment favored BEL at Week 52 for resolution of all SELENA-SLEDAI items (vasculitis, rash, alopecia, and mucosal ulcers), and nine of 20 BILAG items (mild maculopapular eruption, localized active discoid lesions, mild alopecia, small mucosal ulceration, malar erythema, subcutaneous nodules, swollen fingers, major cutaneous vasculitis including ulcers, and minor cutaneous vasculitis).ConclusionPatients with SLE treated with BEL plus ST experienced significant improvements in most mucocutaneous and vasculitis manifestations compared with patients receiving PBO plus ST. These data provide additional support for the use of BEL in SLE and show that it is associated with skin improvements.

Objective: Intravenous immunoglobulins (IVIG) are an established treatment in several immune-mediated diseases such as dermatomyositis or Kawasaki disease and they have been used in distinct clinical scenarios in systemic lupus erythematosus (SLE). However, in this latter disease no clear evidence besides hematological or renal involvement supports its use. The aim of the present study is to establish evidence for the treatment of non-renal non-hematological lupus with IVIG.Methods: We have carried out a systematic literature review following PRYSMA principles on the use of this treatment in SLE patients with no renal no hematologic manifestations.Results: We found 35 articles which were split into two subtypes, those with individualized data (29) and those with grouped data. Overall, 198 lupus patients treated with IVIGs [178 (90%) women and 20 (10%) men] were included. In 167 (84.3%) a clinical response was obtained [62 (31.3%) complete and 105 (53%) a partial one]. Adverse events were reported in 40 patients (20.2%), most of them mild with only 2 (1%) severe. According to individualized data, all cases with respiratory problems (5), 82.4% of those with cardiac involvement (18) and 53.8% of those with neuropsychiatric manifestations (18) achieved a complete response as more outstanding results.Conclusions: After reviewing the experience of the use of IVIGs in extra-renal and extra-hematologic SLE patients, this treatment is demonstrated to be useful in refractory cases, especially in pulmonary, cardiac or neuropsychiatric complications with a good safety profile.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, autoimmune peripheral neuropathy, rarely reported as being associated with systemic lupus erythematous (SLE). We report the case of 53-year-old women with a long history of SLE, diagnosed with CIDP during a lupus flare beginning with progressive muscle weakness of lower and upper limbs, without affection of the central nervous system. She received treatment with steroids, intravenous immunoglobulins and anifrolumab and showed clinical and immunological improvement. It is important to take into account the presence of peripheral neurological disorders in patients with SLE, considering CIDP as an uncommon manifestation. Controlling severe active disease is an imperative issue to manage this neurological manifestation which has a serious impact on health-related quality of life and physical function.