Severe Lupus Nephritis Research Articles

Pregnancy-Triggered Severe Lupus Nephritis with Pleural Effusion: A Case Report

Background: Lupus nephritis (LN) is a severe manifestation of systemic lupus erythematosus (SLE), characterized by kidney inflammation. Pregnancy can trigger or exacerbate LN due to hormonal shifts and altered immune responses. This case highlights the challenges in diagnosing and managing pregnancy-associated LN. Case presentation: A 27-year-old woman presented with anasarca, malar rash, shortness of breath, and foamy urine during her first pregnancy. She had a history of SLE with previous symptoms limited to skin and joint involvement. Investigations revealed nephrotic-range proteinuria, hematuria, elevated creatinine, and positive anti-nuclear antibodies (ANA). Renal biopsy confirmed Class IV lupus nephritis. She was diagnosed with pregnancy-triggered severe LN with nephrotic syndrome, pleural effusion, and a hypercoagulable state. Treatment included high-dose corticosteroids and mycophenolate mofetil, with close monitoring of both maternal and fetal health. Conclusion: This case underscores the importance of recognizing and promptly managing LN in pregnancy. Early diagnosis, multidisciplinary care, and individualized treatment are crucial to optimize maternal and fetal outcomes.

A Case Report of Severe Systemic Lupus Erythematosus with Anti-centromere Antibody.

Systemic lupus erythematosus (SLE) is a systemic disease that affects several organs. The diagnosis was performed using the international classification criteria updated in 2019. The significance of antinuclear antibodies (ANAs) with a centromere pattern has not been clearly documented in these criteria. We herein report a patient with ANAs with a centromere pattern and anti-centromere antibodies, without anti-Sm antibodies or anti-dsDNA antibodies, who developed severe lupus nephritis, neuropsychiatric SLE, and lupus pleuritis. The patient was successfully treated with prednisolone, hydroxychloroquine, mycophenolate mofetil, cyclophosphamide, and belimumab, despite several relapses. Anti-centromere antibodies are not regarded as characteristics of SLE but can be detected in SLE patients with severe organ involvement.

Lupus nephritis randomised controlled trials: evidence gaps and under-represented groups

ObjectiveWe performed a scoping review of randomised clinical trials (RCTs) assessing pharmacological therapies for the initial management of lupus nephritis (LN), focusing on study design, included populations and outcome definitions,...

Efficacy and Safety of Belimumab in Severe Lupus Nephritis: A Retrospective Single-Center Study

Efficacy and Safety of Belimumab in Severe Lupus Nephritis: A Retrospective Single-Center Study

Qualified kidney injury biomarkers demonstrate value during early clinical drug development.

Drug-induced kidney injury (DIKI) is of significant concern, both during drug development and in clinical practice. We report a patient-centric approach for clinical implementation of the FDA-qualified kidney safety biomarker panel, highlighting Phase 1 and 2 trials for candidate therapeutics in Pfizer's portfolio (PFE-1 and PFE-2, respectively) that induced kidney tubular injury in rat toxicity studies. Clusterin (CLU), cystatin-C (CysC), kidney injury molecule-1 (KIM-1), N-acetyl-beta-d-glucosaminidase (NAG), neutrophil gelatinase-associated lipocalin (NGAL), and osteopontin (OPN) were measured in urine samples from (i) Phase 1 healthy volunteers (HVs; n = 12) dosed with PFE-1, (ii) Phase 2 rheumatoid arthritis (RA) patients (n = 266) dosed with PFE-2, (iii) lupus patients on standard-of-care therapies (n = 121), and (iv) healthy volunteers (n = 60). The FDA-defined composite measure (CM), calculated as the geometric mean response across the 6 biomarkers, was increased ∼30% in HVs administered 100 mg PFE-1 relative to placebo, providing evidence of DIKI. In contrast, the CM for RA patients dosed with PFE-2 was comparable to placebo controls, helping to de-risk the concern for DIKI at clinically relevant doses. Comparing individual biomarker concentrations across disease states revealed that CLU, KIM-1, NAG, NGAL, and OPN are elevated in the urine of RA and lupus patients (those without severe active proliferative lupus nephritis) relative to HVs. Overall, these case studies demonstrate the value of using the FDA-qualified kidney biomarker panel to guide risk assessment, dose selection, and clinical decision making for novel therapeutics, both in HVs and patient populations.

Sustained depression of B cell counts in lupus nephritis after treatment with rituximab and/or belimumab is associated with fewer disease flares.

To study the risk of lupus nephritis flare (LNF) or severe lupus flare (SLF) as a function of B cell count kinetics in lupus nephritis (LN) patients after they achieve at least a partial renal response (PRR) with induction treatment that includes rituximab (RTX) and/or belimumab (BLM). We performed a retrospective analysis of a cohort of 19 patients with severe LN that received a B cell agent (BCA), RTX and/or BLM, as part of an initial treatment regimen for an LN flare and had subsequent CD19+ B cell measurements in peripheral blood. We then characterized the follow-up periods, after B cell depressions occurred and PRR were achieved, by the corresponding trajectories of B cell counts (BCC). Time periods with sustained low BCC were type 1 (T1) episodes, while those with repletion of BCC>100 cells/μL were called type 2 (T2) episodes. Time periods with rapid BCC repletion, defined as >50 cells/μL in ≤6months, were called T2b episodes. Corresponding C3, C4, and anti-dsDNA levels were recorded for each episode. The time from PRR until an event, either a LNF or SLF, or to censoring, either at the end of the study period or the end of available patient follow-up, was assessed for each episode type. Kaplan-Meier survival analysis was used to compare time to flare between T1 and T2 episodes. There were 26 episodes of B cell depression. Seventeen (65%) were T1 and 9 (35%) were T2. Compared to T1 episodes, T2 episodes were 9.0 times more likely to result in flare over the follow-up period (hazard ratio (HR) = 9.0, 95% CI for HR = 2.2-36.7); this risk was even larger for T2b vs T1 episodes. Median BCC was 14 cells/μL in T1 and 160 cells/μL in T2 episodes. Both C3 and C4 levels significantly increased over the duration of the episode in T1 episodes only. Sustained low BCC was associated with prolonged serologic and clinical response, whereas repletion, and particularly rapid repletion, of B cells after treatment with BCA was associated with subsequent disease flare.

Complement C4d as a biomarker for systemic lupus erythematosus and lupus nephritis.

Background: Increasing studies in the last decade have led to the widespread understanding that C4d, a split product of complement component 4 (C4), is a potential biomarker for systemic lupus erythematosus (SLE) and lupus nephritis (LN).Purpose: The aim of this review is to summarize the highlights of studies investigating the use of C4d as a biomarker for diagnosing and monitoring SLE and LN patients.Data collection: we searched PubMed/Medline and Wanfang databases using the terms "C4d and systemic lupus erythematosus", "C4d and lupus nephritis", and "Complement C4d".Results: The deposition of C4d on circulating blood cells has been shown in several clinical studies to be a potential diagnostic marker that can be used to monitor patients with SLE. In addition, C4d deposits on circulating blood cells may be a helpful diagnostic marker for LN, one of the most severe complications of SLE. Meanwhile, studies utilizing renal biopsy specimens have indicated that C4d deposition in the renal peritubular capillaries of LN patients may predict more severe LN or a worse patient prognosis. Generally, a high plasma C4d level and a high plasma C4d/C4 ratio may also be promising indicators that can be used to monitor patients with SLE and LN.Conclusions: C4d detection may be a novel strategy for further clinical prediction and therapy.

Lupus Nephritis Biomarkers: A Critical Review.

Lupus nephritis (LN), a major complication in individuals diagnosed with systemic lupus erythematosus, substantially increases morbidity and mortality. Despite marked improvements in the survival of patients with severe LN over the past 50 years, complete clinical remission after immunosuppressive therapy is achieved in only half of the patients. Therefore, timely detection of LN is vital for initiating prompt therapeutic interventions and improving patient outcomes. Biomarkers have emerged as valuable tools for LN detection and monitoring; however, the complex role of these biomarkers in LN pathogenesis remains unclear. Renal biopsy remains the gold standard for the identification of the histological phenotypes of LN and guides disease management. However, the molecular pathophysiology of specific renal lesions remains poorly understood. In this review, we provide a critical, up-to-date overview of the latest developments in the field of LN biomarkers.

Efficacy of double filtration plasmapheresis combined with immunosuppressive agents in the treatment of severe lupus nephritis.

To evaluate the efficacy of double filtration plasmapheresis combined with immunosuppressive agents in the treatment of severe lupus nephritis. A retrospective analysis was conducted on the medical records of 102 cases of severe lupus nephritis treated between January 2021 and December 2022 in the General Practice Department at the Affiliated Hospital of North Sichuan Medical College. Patients who received immunosuppressive agents were included in the control group and those who received additional double filtration plasmapheresis were included in the observation group. Changes in liver and kidney function indicators, immune function indicators, disease activity, peripheral blood immunoglobulins, total albumin levels, gamma globulin levels, erythrocyte sedimentation rates (ESR), and inflammatory marker levels, and overall clinical efficacy were compared between the two groups. After therapy, kidney function indicators in the observation group were lower than in the control group, while serum albumin (Alb), total albumin level, complement component 3 (C3) and C4 levels were higher (all P<0.05). Anti-double-stranded DNA antibody (ds-DNA) and white blood cell (WBC) counts in the observation group were also lower than those in the control group. Additionally, the systemic lupus erythematosus disease activity index (SLEDAI) scores, the levels of tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), C-reactive protein (CRP), and ESR were lower in the observation group than those in the control group (all P<0.05). The total clinical effective rate was higher in the observation group than in the control group (P<0.05). The combination of immunosuppressive agents with double filtration plasmapheresis in patients with severe lupus nephritis can significantly improve liver and kidney function, enhance immune function, and reduce inflammation, demonstrating good therapeutic effects and safety.

Purification and analysis of kidney-infiltrating leukocytes in a mouse model of lupus nephritis.

Purification and analysis of kidney-infiltrating leukocytes in a mouse model of lupus nephritis.

Symposium 6

The ultimate goal of treatment of systemic lupus erythematosus (SLE) is to prolong survival without compromising the quality of life. The survival rates of SLE have plateaued since the 2000s’ and morbidities related to disease activity and treatment are still problematic. A big unmet need for SLE therapy is to develop strategies that can enhance efficacy, reduce disease flares and drug related complications. Cocktail immunosuppressive therapies for the synergistic effect of individual drugs may enhance efficacy and enable dosage reduction. However, risk of infective complications, adoption of a step-up or step-down approach and the choice of agents are still controversial, partly because of the issue of cost-effectiveness. Belimumab is the first biological agent licensed for pediatric and adult patients with renal and non-renal SLE, based on several pivotal randomized controlled trials (BLISS-52, BLISS-76, BLISS-NEA, BLISS-SC, BLISS-LN). The continuous use of belimumab enables reduction of disease flares, glucocorticoid doses and organ damage. Anifrolumab, a monoclonal antibody against type I interferon receptors, has shown efficacy for non-renal SLE in the TULIP studies. It is recently licensed for moderate to severe SLE patients who are receiving standard therapies. Although rituximab did not show benefit in renal and non-renal SLE, it is commonly used off-label to treat refractory lupus manifestations. The newer generation anti-CD20 biologic, obinutuzumab, shows promising preliminary results in a phase 2 lupus nephritis (LN) trial. Combination of anti-CD20 and anti-BAFF is being studied in LN but the results are conflicting hitherto. Ianalumab, a monoclonal antibody against BAFF receptor that exhibits dual mechanisms of action (BAFF inhibition and B cell depletion), is being studied in LN. The updated 2023 EULAR recommendations for SLE have included the role of biological agents in the treatment algorithm. Belimumab and anifrolumab may be considered in non-major organ SLE, including skin disease, that is refractory to hydroxychloroquine with or without other immunosuppressive agents. Initial combination therapy of glucocorticoids, mycophenolate mofetil or low dose cyclophosphamide, with belimumab is an option for the treatment of severe LN, and belimumab may be used as long-term maintenance therapy of SLE/LN. In this talk, the evidence and the role of the older and newer biologic and targeted agents in SLE/LN will be reviewed.

MAIT cells altered phenotype and cytotoxicity in lupus patients are linked to renal disease severity and outcome.

Systemic lupus erythematosus (SLE) is an autoimmune disease in which circulating immune complexes can cause different types of glomerulonephritis, according to immune deposits and to the type of glomerular cell injury. Proliferative lesions represent the most severe form of lupus nephritis (LN) and often lead to kidney failure and death. Mucosal-associated invariant T (MAIT) cells are a subset of innate-like T cells that recognize microbial-derived ligands from the riboflavin synthesis pathway. Although abundant in peripheral blood, MAIT cells are enriched in mucosal and inflamed tissues. While previous studies have reported concordant results concerning lower MAIT cell frequencies in the blood of SLE patients, no information is known about MAIT cell function and LN severity and outcome. In the current study, we analyzed the baseline phenotype and function of peripheral blood MAIT cells by flow cytometry in 26 patients with LN and in a control group of 16 healthy individuals. We observe that MAIT cell frequencies are markedly reduced in blood of LN patients. MAIT cells from patients have an altered phenotype in terms of migration, proliferation and differentiation markers, notably in most severe forms of LN. Frequencies of PMA/ionomycin stimulated MAIT cells secreting effector molecules, such as proinflammatory IL-17 and cytotoxic protein granzyme B, are higher in LN patients. Patients undergoing a complete renal remission after immunosuppressive therapy had higher MAIT cell frequency, lower expression of proliferation marker Ki-67 and granzyme B (GzB) at inclusion. Remarkably, GzB production defines a predictive model for complete remission. We report here that blood MAIT cells display proinflammatory and cytotoxic function in severe lupus nephritis which may play a pathogenesis role, but without association with systemic lupus activity. Finally, low cytotoxic profile of MAIT cells may represent a promising prognostic factor of lupus nephritis remission one year after induction therapy.

Relapse of lupus nephritis induced by nivolumab in an 84-year-old patient with oral cancer: a case report.

Various immunity-related adverse events have been reported to be associated with the inhibition of programmed cell death receptor 1. We report a rare case of a relapse of lupus nephritis (LN), involving rapidly progressive glomerulonephritis, which was induced by nivolumab treatment in a patient with oral cancer. The patient had a history of systemic lupus erythematosus and underwent treatment with steroids, rituximab, and plasmapheresis. However, her renal function did not improve, and she died of multiple organ failure. To our knowledge, this is the first description of severe LN induced by nivolumab.

State-of-the-art paradigm of corticosteroid therapy for immune-mediated inflammatory kidney diseases

Since 1950's corticosteroids (CS) have remained the cornerstone of immunosuppressive therapy for immune-mediated kidney diseases. However multiple adverse events, associated with the prolonged CS therapy, became the basis for the development of novel treatment approaches. Current evidence supports the implementation of the steroid-sparing regimens for the treatment of different types of glomerulonephritis. Randomised controlled trial PEXIVAS demonstrated the efficacy and safety of early steroid tapering, starting from the second week of therapy, in patients with ANCA-associated vasculitis with kidney involvement. Several trials showed the efficacy of oral prednisolone 0.3-0.5 mg/kg/daily as a part of multitarget therapy for severe proliferative lupus nephritis. A combination of calcineurin inhibitors and low-dose CS are effective for remission induction in membranous nephropathy, as well as the steroid-free rituximab regimen for the patients with moderate risk of disease progression. Medium dose CS showed promising effect in patients with IgA-nephropathy. Long-term high dose CS remain the standard-of-care for the treatment of minimal change disease and focal segmental glomerulosclerosis, however patients with steroid-dependent and relapsing disease tacrolimus and rituximab can help to achieve steroid-sparing effect. The role of CS pulse-therapy is currently debated, nevertheless it remains a compulsory treatment in several conditions. Thus, overall trend is directed towards the minimization of the maximal doses of CS and/or treatment duration. However, to implement this approach morphological verification of the diagnosis and personalized assessment of the potential risk and benefit are required.

Clinical efficacy of centrifugal-membranous hybrid double filtration plasmapheresis and membranous double filtration plasmapheresis on severe lupus nephritis.

The study delves into the clinical efficacy and safety of centrifugal-membranous hybrid double filtration plasmapheresis (C/M hybrid DFPP) on severe lupus nephritis (LN) by comparing it with membranous DFPP (M DFPP). A retrospective cohort study was conducted in 70 patients who were diagnosed with severe LN and had received DFPP treatment. A total of 181 DFPPs were performed, including 133C/M hybrid DFPPs (51 patients) and 48M DFPPs (19 patients).The ANA, A-dsDNA titer, quantitative urinary protein, and serum creatinine decreased significantly and hemoglobin increased significantly after the DFPP treatment and at third month after treatment. Two patients in the M DFPP group developed bleeding complications, and four patients in the C/M hybrid DFPP group developed perioral numbness. Although there was no significant difference in clinical efficacy between C/M hybrid DFPP and M DFPP on severe LN, the risk of bleeding complications was significantly lower in the C/M hybrid DFPP group.

Long-term outcomes with rituximab as add-on therapy in severe childhood-onset lupus nephritis.

Long-term data pertaining to rituximab as add-on therapy in childhood-onset lupus nephritis (cLN) is scarce. A retrospective cohort study was conducted on all patients with proliferative cLN, diagnosed ≤ 18years and between 2005 and 2021, who received rituximab for LN episodes that were life/organ threatening and/or treatment resistant to standard immunosuppression. Fourteen patients with cLN (female, n = 10) were included, with median follow-up period of 6.9years. LN episodes (class III, n = 1; class IV, n = 11; class IV + V, n = 2) requiring rituximab occurred at 15.6years (IQR 12.8-17.3), urine protein:creatinine ratio was 8.2mg/mg (IQR 3.4-10.1) and eGFR was 28mL/min/1.73 m2 (IQR 24-69) prior to rituximab treatment. Ten and four patients received rituximab at 1500mg/m2 and 750mg/m2, which were given at 46.5days (IQR 19-69) after commencement of standard therapies. Treatment with rituximab resulted in improvements in proteinuria (ps < 0.001), eGFR (ps < 0.01) and serological parameters, including haemoglobin levels, complement 3 levels and anti-dsDNA antibodies, compared with baseline. Rates of complete/partial remission at 6-, 12- and 24-month post-rituximab were 28.6/42.8%, 64.2/21.4% and 69.2/15.3%. All three patients who required acute kidney replacement therapy became dialysis-free after rituximab. Relapse rate following rituximab was 0.11 episodes/patient-year. There was no lethal complication or severe infusion reaction. Hypogammaglobulinaemia was the most frequent complication (45%) but was mostly asymptomatic. Neutropenia and infections were observed in 20% and 25% of treatments. Upon last follow-up, three (21%) and two (14%) patients developed chronic kidney disease (stage 2, n = 2; stage 4; n = 1) and kidney failure, respectively. Add-on rituximab is an effective and safe rescue therapy for cLN patients with life-/organ-threatening manifestations or treatment-resistance. A higher resolution version of the Graphical abstract is available as Supplementary information.

#4844 BELIMUMAB AND MULTITARGET THERAPY AS INDUCTION THERAPY OF SEVERE ACTIVE LUPUS NEPHRITIS: CASE SERIES FROM CHINA

Abstract Background and Aims Belimumab (BLM) has been approved for the treatment of active systemic lupus erythematosus (SLE) and active lupus nephritis (LN). Multitarget therapy (MT) consisting of tacrolimus (FK506), mycophenolate mofetil (MMF), and steroid has been proved that can provide good curative effect as induction therapy for LN. Because of the limitations of BLM's phase III clinical trails, the exact curative effect in severe active LN of BLM is not clear yet. To assess the safety and generate preliminary efficacy data on MT followed by BLM for severe active Chinese LN patients, a case series were investigated. Method Six patients with severe active LN were reported. Among the 6 patients, 2 were treated with MT followed by BLM infusions, 2 were treated with MMF / (FK506) followed by BLM infusions, and 2 were treated with MT. BLM was given 10 mg/kg every 2 weeks for 3 times, then every 4 weeks till Week 24. Primary renal response index, SLEDAI, and safety data were analyzed. Results The patients affected by LN ISN/RNP Class III/IV±V with high disease activity are 3 de no LN and 3 refractory LN (each regimen included 1 de no LN and 1 refractory LN). At baseline, the SLEDAI score were 15,20,17,16,15,15 respectively. 4 of the 6 patients (case 1,case 2,case 3, and case 5) accepted intravenous (IV) methylprednisolone, followed by MT/MMF/FK506 after admission. The initial oral prednisone was 30-45 mg/day. Within 2 weeks of starting treatment, IV BLM was introduced. At Week 24, the SLEDAI score decreased to 2,8,8,10,8,10 respectively. 2 patients accepted BLM and MT achieved complete renal remission, and the other 4 patients achieved partial renal remission (Fig. 1). MT and BLM therapy reduced SLEDAI more rapidly than MMF/FK 506 +BLM or MT (Fig. 2). Prednisone was reduced to 10-15 mg/d. No adverse events occurred. Conclusion The addition of BLM to basic immunosuppressants was effective and safe for severe active LN. The BLM and MT regimen seems to lead a faster and more pronounced remission. Further prospective clinical studies with larger samples are needed to evaluate the benefits of belimumab combined with MT in severe active LN.

Predictors of treatment outcomes in lupus nephritis with severe acute kidney injury and requirement of dialytic support.

Acute kidney injury (AKI) with the requirement of kidney replacement therapy (KRT) portends a poor prognosis for kidney function in lupus nephritis (LN). This study evaluated the kidney function recovery rates, the rates of reinitiation of KRT, and factors associated with these outcomes in LN. All consecutive patients hospitalized for LN with KRT requirement between 2000 and 2020 were included. Their clinical and histopathologic characteristics were retrospectively registered. The outcomes and associated factors were evaluated by multivariable Cox regression analysis. Among 140 patients, 75 (54%) recovered kidney function, with recovery rates of 50.9% and 54.2% by 6 and 12months of therapy. The factors associated with a lower probability of recovery included a previous history of LN flares, worse eGFR and higher proteinuria at presentation, immunosuppression with azathioprine, and hospitalizations within 6months of therapy initiation. There was no difference in the kidney function recovery rates between mycophenolate and cyclophosphamide treatment schemes. Out of 75 patients who recovered kidney function, 37 (49%) reinitiated KRT, with KRT reinitiation rates of 27.2% and 46.5% by 3 and 5years. Seventy-three (52%) patients had at least one hospitalization within 6months of initial therapy, 52 (72%) of them secondary to infectious events. Approximately 50% of patients with LN and KRT requirement recover kidney function within 6months. The risk-to-benefit ratio decisions may be aided by clinical and histological factors. These patients require close follow-up as ≈50% of those who recover kidney function will reinitiate dialysis in the long term. Key Points • Approximately 50% of patients with severe acute lupus nephritis with the need for kidney replacement therapy requirement recover their kidney function. • The factors associated with a lower probability of recovery of kidney function include a previous history of LN flares, worse eGFR and higher proteinuria at presentation, immunosuppression with azathioprine, and hospitalizations within 6 months of therapy initiation. • Patients who recover kidney function will require close follow-up as around 50% of them will eventually reinitiate kidney replacement therapy.

Significance of co-positivity for anti-dsDNA, -nucleosome, and -histone antibodies in patients with lupus nephritis

Objective The aim of this study was to define the clinical, histopathologic, and prognostic features associated with simultaneous positivity for anti-dsDNA, -nucleosome, and -histone antibodies (3-pos) in Korean patients with biopsy-proven lupus nephritis (LN). Methods The 102 patients included in the study had undergone kidney biopsy prior to the start of induction treatment, were treated with immunosuppressives, and followed-up for >12 months. Results In total, 44 (43.1%) of the 102 LN patients were 3-pos. Patients with 3-pos had a higher SLEDAI-2K score (p = .002), lower lymphocyte count (p = .004), and higher rates of proteinuria > 3.5 g/24 h (p = .039) and positivity for urinary sediments (p = .005) at the time of renal biopsy than non-3-pos patients. 3-pos patients had a more proliferative form of LN (p = .045) in the renal histopathologic findings, and as co-positivity gradually increased from 0 to 3, the total activity score in the renal biopsy findings increased significantly (p = .033). In addition, 3-pos patients had a more rapid eGFR decline than non-3-pos patients after a follow-up of 83.2 months (p = .016). Conclusions Our findings suggest that 3-pos is related to severe LN and that 3-pos patients are more likely to experience a rapid decline of renal function than non-3-pos patients. KEY MESSAGE Patients with co-positivity for anti-dsDNA, -nucleosome, and -histone antibodies (3-pos) had higher disease activity and a worse renal histopathology than those without co-positivity. 3-pos patients had a more rapid decline of renal function than non-3-pos patients.

A case report of systemic lupus erythematosus combined with central serous chorioretinopathy treated with glucocorticoids

Glucocorticoids are generally contraindicated for use in central serous chorioretinopathy (CSC) because their use is considered to be an independent risk factor for CSC. There are rare reports regarding the treatment of systemic lupus erythematosus (SLE) combined with CSC. This current case report describes a rare case of a 24-year-old female patient with severely active SLE combined with CSC, whose vision was significantly restored after she was administered 120 mg methylprednisolone intravenously once a day for 3 days. This case report presents the clinical characteristics for the first time in terms of distinguishing between typical CSC and lupus chorioretinopathy. It also provides a review of the relevant literature. In patients with clinically severe active lupus nephritis combined with bilateral lupus chorioretinopathy, timely systemic application of appropriate doses of glucocorticoids is the preferred method to control the primary disease and serious ocular complications.