Concurrent Systemic Lupus Erythematosus Research Articles

Diagnostic Dilemma: Immunoglobulin A Nephropathy Concurrent with Systemic Lupus Erythematosus

Abstract Renal involvement is a common feature of systemic lupus erythematosus (SLE), but cases of non-lupus nephritis occurring simultaneously with SLE are rare. IgA Nephropathy (IgAN) and SLE can present diagnostic challenges due to overlapping symptoms and laboratory findings. This abstract explores the complexities of diagnosing IgA Nephropathy in patients with concurrent SLE. While IgAN and lupus nephritis share some pathological traits, their laboratory and histopathological profiles, as well as extra-renal manifestations, differ significantly, indicating distinct pathogenesis. It highlights the importance of renal biopsy in distinguishing between these conditions to ensure accurate diagnosis and effective management.

An update on clinical trials for cutaneous lupus erythematosus.

Cutaneous lupus erythematosus (CLE) comprises dermatologic manifestations that may occur independently or with systemic lupus erythematosus (SLE). Despite advancements in refining CLE classification, establishing precise subtype criteria remains challenging due to overlapping presentations and difficulty in distinguishing morphology. Current treatments encompass preventive measures, topical therapies, and systemic approaches. Hydroxychloroquine and glucocorticoids are the sole US Food and Drug Administration (FDA)-approved medications for CLE, with numerous off-label treatments available. However, these treatments are often not covered by insurance, imposing a significant financial burden on patients. The exclusion of most CLE patients, particularly those without concurrent SLE, from trials designed for SLE has resulted in a lack of targeted treatments for CLE. To develop effective CLE treatments, validated outcome measures for tracking patient responsiveness are essential. The Cutaneous Lupus Erythematosus Disease Area and Severity Index is widely utilized for its reliability, validity, and ability to differentiate between skin activity and damage. In contrast, the FDA mandates the use of the Investigator's Global Assessment, a five-point Likert scale related to lesion characteristics, for skin-related therapeutic trials. It requires the disease to resolve or almost completely resolve to demonstrate improvement, which can be difficult when there is residual erythema or incomplete clearance that is meaningfully improved from a patient perspective. Various classes of skin lupus medications target diverse pathways, allowing tailored treatment based on the patient's lupus inflammatory profile, resulting in improved outcomes. Promising targeted therapeutic drugs include anifrolumab (anti-type 1 interferon), deucravacitinib (allosteric tyrosine kinase 2 inhibitor), litifilimab (plasmacytoid dendritic cell-directed therapy), iberdomide (cereblon-targeting ligand), and belimumab (B-cell directed therapy). Despite the significant impact of CLE on quality of life, therapeutic options remain inadequate. While promising treatments for cutaneous lupus are emerging, it is crucial to underscore the urgency for skin-focused treatment outcomes and the implementation of validated measures to assess therapeutic effectiveness in clinical trials.

A case report of systemic lupus erythematosus and intestinal tuberculosis with lower gastrointestinal bleeding: A treatment approach utilizing parenteral nutrition.

Limited literatures are available on lower gastrointestinal bleeding in systemic lupus erythematosus (SLE) combined with intestinal tuberculosis. Sharing the treatment experiences of a 26-year-old female patient diagnosed with this complex condition in this report may contribute valuable insights. The patient initially presented with abdominal pain and active gastrointestinal bleeding, leading to admission to the hospital. Over a 2-week period, she experienced persistent bleeding, with daily volumes ranging from 300 mL to 800 mL. Lower gastrointestinal bleeding was diagnosed in this patient with concurrent systemic lupus erythematosus and intestinal tuberculosis. As her symptoms rapidly progressed, food and water intake had to be completely restricted. The parenteral nutrition was implemented. The medical team effectively controlled the bleeding, leading to a notable improvement in the patient's condition. Consequently, she was able to resume oral intake and was discharged from the hospital. This case highlights the significance of using parenteral nutrition in the management of lower gastrointestinal bleeding in patients with concurrent systemic lupus erythematosus and intestinal tuberculosis. Close monitoring and collaborative efforts among healthcare professionals are crucial to achieve successful outcomes in similar cases.

Coexistence of Systemic Lupus and Antiphospholipid Syndrome in a Male Patient: Atypical Case Presentation

Abstract Systemic Lupus Erythematosus (SLE) and Antiphospholipid Syndrome (APS) are two distinct autoimmune disorders characterized by dysregulated immune responses, organ involvement, and the presence of autoantibodies in the bloodstream. Primary Antiphospholipid Syndrome (APS) can occur among individuals who are generally healthy and have no history of an underlying systemic autoimmune illness. In contrast, several other systemic autoimmune diseases, including SLE, have the potential to cause secondary Antiphospholipid Syndrome (APS). While Systemic Lupus Erythematosus (SLE) and Antiphospholipid Syndrome (APS) affect women of childbearing age, there are only a few reported case studies documenting the presence of SLE or APS individually in male patients. However, the coexistence of both conditions in a male patient is extremely rare, with only a few documented cases reports available to date. This case report presents a unique occurrence of concurrent SLE and APS in a forty-year-old male patient, highlighting the rarity of such a presentation. The patient presented with per rectal and mucosal bleeding and had been on warfarin therapy for 16 years due to a history of recurrent Deep Vein Thrombosis (DVT), but was not evaluated further. Immune-related laboratory results revealed positive antinuclear antibodies, anti-dsDNA antibodies, anticardiolipin antibodies, lupus anticoagulants, and direct Coombs test. Concurrent SLE and APS were diagnosed by meeting the classification criteria for both diseases. This report adds to the medical literature and emphasizes the significance of considering concurrent SLE and APS as a potential diagnosis in males. Further research is needed to enhance our understanding of the underlying mechanisms and the best approaches for managing this rare coexistence.

State of current management of the heightened risk for atherosclerotic cardiovascular events in an established cohort of patients with lupus erythematosus

ObjectivePatients with lupus erythematosus (LE) are at heightened risk for clinical events, chiefly heart attacks and strokes, from atherosclerotic cardiovascular disease (ASCVD). We recently proposed new guidelines to assess and...

Retrospective analysis of low-dose interleukin-2 therapy on chronic autoimmune thyroid disease with concurrent systemic lupus erythematosus

Introduction Low-dose interleukin-2 (IL-2) regulates the homeostasis of CD4+ T cells by modulating the proportions of effector and regulatory T cells, thus reducing disease activity in patients with systemic lupus erythematosus (SLE). However, to date, no research has been carried out on the efficacy of low-dose IL-2 for treating autoimmune thyroid disease (AITD). The aim of this study was to observe the effects of IL-2 on AITD patients with concurrent SLE, and explore potential mechanism of action. Methods A retrospective analysis was conducted on 29 SLE patients with concurrent AITD. Among them, 11 patients were in IL-2 therapy group and 18 patients without IL-2 treatment were considered as control group. Two groups had similar disease activities and were treated with comparable regular strategy. Free triiodothyronine (FT3), free thyroxine (FT4), thyroxine(T4), triiodothyronine(T3), thyroid stimulating hormone (TSH), thyroglobulin antibody (TG-Ab), thyroid peroxidase antibody (TPO-Ab) levels and immune cell subgroups were measured. Results After receiving low-dose IL-2 therapy, the TG-Ab and TPO-Ab levels decreased drastically (TG-Ab p = 0.008, TPO-Ab p = 0.007), and the majority of the AITD patients became seronegative, while there was no discernible change in control group. In IL-2 group, percentage of CD4+ T cells showed a significant increase after treatment (p = 0.029), with an upward trend in the ratio of regulatory T (Treg) cells to follicular helper T (Tfh) cells (Treg/Tfh). The percentage as well as absolute count of B cells demonstrated a decreasing trend. Conclusion Low-dose IL-2 may downregulate the levels of TG-Ab and TPO-Ab by modulating the immune balance of Treg/Tfh and B-cells, providing new avenue for clinical treatment of AITD.

Therapeutic Plasma Exchange in a Patient With Acute Motor Axonal Neuropathy Subtype of Guillain-Barre Syndrome and Systemic Lupus Erythematosus

Objective To highlight treatment resistance of concurrent Guillain-Barre syndrome (GBS) and active systemic lupus erythematosus (SLE). Background Coincidence of SLE and GBS is an uncommon and complicated course of autoimmune disease. Treatment is further complicated by pregnancy exacerbation of SLE. Design/Methods We report a case of acute motor axonal neuropathy (AMAN) subtype of GBS in a young pregnant woman in her early 20s presenting with SLE. Patient was 10 weeks pregnant at presentation. Lumbar puncture study and electrical muscle stimulation (EMS) were consistent with acute motor axonal neuropathy subtype of GBS. Patient also had increased proteinuria and renal biopsy performed that was consistent with lupus nephritis. Despite treatment with IVIG and pulse dose corticosteroids, the patient had minimal neurological improvement with respiratory decline requiring intubation. Her pregnancy was terminated at this point and a course of therapeutic plasma exchange (TPE) was started followed by cyclophosphamide. Results The patient responded to the combination of therapy and had slow but gradual neurologic recovery as well as improvement of proteinuria. Conclusions Concurrent GBS and active SLE in the setting of pregnancy may be more treatment resistant, and combination therapy including TPE, immunosuppression, and termination of pregnancy may be indicated. For patients with concurrent GBS and SLE, especially axonal subtype of GBS and during pregnancy, TPE should be considered as a primary treatment option with respect to both efficacy and safety.

148 Management of the heightened risk for clinical events from atherosclerotic cardiovascular disease (ASCVD) in an established cohort of lupus erythematosus patients

Lupus erythematosus (LE) patients are at heightened risk of clinical events, chiefly heart attacks and strokes, caused by ASCVD. To address this problem, we recently proposed new guidelines for categorization of levels of risk for ASCVD events in LE patients, with corresponding recommendations for management of conventional risk factors, chiefly hypercholesterolemia, hypertension, smoking, and diabetes mellitus (Keyes E et al. 2021). Here, we performed a single-center study of our established cohort of cutaneous LE patients without or with concurrent systemic LE (n=370).

Small plaque psoriasis re-visited: A type of psoriasis mediated by a type-I interferon pathway.

TNFα-inhibitor-induced psoriasis is mediated by the type-I interferon pathway, of which IFNα, LL37 and IL-36γ are major players. A subset of patients treated with TNFα inhibitors develop small plaque psoriatic lesions. Small plaque psoriasis is similarly observed in patients on immune checkpoint inhibitors (ICI), and with concurrent systemic lupus erythematosus (SLE) or positive antinuclear antibody (ANA). Small plaque psoriasis is also the predominant phenotype in Asian populations. The association between small plaque psoriasis morphology in various clinical scenarios and the type-I interferon pathway has not been previously studied. A cross-sectional study was conducted of patients who developed small plaque psoriasis and had a biopsy for diagnostic clarification between 2009 and 2017. We obtained skin specimens from 14 adults with small plaque psoriasis: four patients taking anti-TNFα treatment, four patients with antecedent SLE, three patients with concurrent ANA positivity and three patients taking ICI. Controls included three patients with chronic plaque psoriasis. Histology confirmed psoriasiform epidermal hyperplasia with focal lichenoid and spongiotic features. Immunohistochemical analysis revealed higher expression of IFNα-induced MXA, LL37 and IL-36γ in all clinical scenarios of small plaque psoriasis compared to chronic plaque psoriasis. There wasdecreased CD8 T-cell migration to the epidermis and variability in the number of LAMP3+ cytoplasmic dendritic cells in the dermis of small plaque psoriasis. The findings suggest that small plaque psoriasis is a unique type of psoriasis with a distinct morphology and immune-phenotype, primarily mediated by the type-I interferon pathway. Associating morphology and disease pathogenesis may help identify therapeutic targets for better disease control.

Pediatric discoid lupus erythematosus: Short report.

Pediatric discoid lupus erythematosus (DLE) is a rare inflammatory skin disorder. This article aims to review all the available clinical and therapeutic data on reported cases of pediatric DLE. A systematic review of the literature was conducted using Pubmed and Embase with no limitation on publication date, sex, or nationality. Thirty-two articles were included with 201 cases, a mean age of 8.9 years (2 months-16 years) and an F:M ratio of 1.8. Lesions were located on the head and neck in 58.5% and were disseminated in 36.5% of the cases. Associated symptoms were pruritus (10.1%) and alopecia (8.7%). 12% progressed to systemic lupus erythematosus (SLE) and 14.5% had concurrent SLE. The only statistically significant predictor for progression to SLE was the onset of symptoms before or at the age of 10 years (p= 0.004). Treatments consisted mainly of sunscreens (26.3%), topical corticosteroids (24.3%), and oral antimalarials (25.3%). Retrospective nature of the included studies, small sample size, short duration of follow-up and limited data on the patients' demographics. Pediatric DLE affects mostly the head and neck, with a female predominance, a possible association with inflammatory and autoimmune diseases, and overall good treatment response and prognosis.

Neuropsychiatric Events in Systemic Lupus Erythematosus: Predictors of Occurrence and Resolution in a Longitudinal Analysis of an International Inception Cohort.

To determine predictors of change in neuropsychiatric (NP) event status in a large, prospective, international inception cohort of patients with systemic lupus erythematosus (SLE). Upon enrollment and annually thereafter, NP events attributed to SLE and non-SLE causes and physician-determined resolution were documented. Factors potentially associated with the onset and resolution of NP events were determined by time-to-event analysis using a multistate modeling structure. NP events occurred in 955 (52.3%) of 1,827 patients, and 593 (31.0%) of 1,910 unique events were attributed to SLE. For SLE-associated NP (SLE NP) events, multivariate analysis revealed a positive association with male sex (P=0.028), concurrent non-SLE NP events excluding headache (P < 0.001), active SLE (P = 0.012), and glucocorticoid use (P = 0.008). There was a negative association with Asian race (P = 0.002), postsecondary education (P = 0.001), and treatment with immunosuppressive drugs (P = 0.019) or antimalarial drugs (P = 0.056). For non-SLE NP events excluding headache, there was a positive association with concurrent SLE NP events (P < 0.001) and a negative association with African race (P = 0.012) and Asian race (P < 0.001). NP events attributed to SLE had a higher resolution rate than non-SLE NP events, with the exception of headache, which had comparable resolution rates. For SLE NP events, multivariate analysis revealed that resolution was more common in patients of Asian race (P = 0.006) and for central/focal NP events (P < 0.001). For non-SLE NP events, resolution was more common in patients of African race (P = 0.017) and less common in patients who were older at SLE diagnosis (P < 0.001). In a large and long-term study of the occurrence and resolution of NP events in SLE, we identified subgroups with better and worse prognosis. The course of NP events differs greatly depending on their nature and attribution.

Cross-sectional study of plasma Axl, ferritin, IGFBP4 and sTNFR2 as biomarkers of disease activity in childhood-onset SLE: A study of the Pediatric Nephrology Research Consortium.

To evaluate the performance of 4 plasma protein markers for detecting disease activity in childhood-onset systemic lupus erythematosus (SLE) patients. Eighty-three consecutive pediatric patients fulfilling ≥4 ACR criteria for SLE and twenty-five healthy controls were prospectively recruited for serological testing of 4 protein markers identified by antibody-coated microarray screen, namely Axl, ferritin, IGFBP4 and sTNFR2. SLE disease activity was assessed using SLEDAI-2000 score. Fifty-seven patients had clinically active SLE (SLEDAI score ≥4, or having a flare). The plasma concentrations of Axl and ferritin were significantly higher in patients with active SLE than inactive SLE. Plasma Axl levels were significantly higher in active renal versus active non-renal SLE patients. Levels of Axl, ferritin and IGFBP4 correlated significantly with SLEDAI scores. Levels of Axl, IFGBP4 and sTNFR2 inversely correlated with plasma complement C3 levels. Only plasma Axl and ferritin levels correlated with degree of proteinuria. These markers were more specific, but less sensitive, in detecting concurrent SLE activity than elevated anti-dsDNA antibody titer or decreased C3. Ferritin and IGFBP4 levels were more specific for concurrent active lupus nephritis than anti-dsDNA or C3. Plasma ferritin was the best monitor of global SLE activity, followed by C3 then Axl, while both Axl and C3 were best monitors of clinical lupus nephritis activity. In childhood-onset SLE patients, plasma ferritin and Axl perform better than traditional yardsticks in identifying disease activity, either global or renal. The performance of these plasma markers should be explored further in longitudinal cohorts of SLE patients.

Comment on: Successful hydroxychloroquine treatment for familial Mediterranean fever in a Japanese patient with concurrent systemic lupus erythematosus: reply.

Comment on: Successful hydroxychloroquine treatment for familial Mediterranean fever in a Japanese patient with concurrent systemic lupus erythematosus: reply.

Comment on: Successful hydroxychloroquine treatment for familial Mediterranean fever in a Japanese patient with concurrent systemic lupus erythematosus.

Comment on: Successful hydroxychloroquine treatment for familial Mediterranean fever in a Japanese patient with concurrent systemic lupus erythematosus.

Blaschkoid lichen planus occurring in childhood systemic lupus erythematosus

Lupus erythematosus-lichen planus (LE-LP) overlap syndrome represents a rare disorder with clinical and histopathological features of both lichen planus and lupus erythematosus presenting in the same lesions. However, lichen planus and lupus erythematosus can also coexist in the same patient. Blaschkoid lichen planus in the setting of concurrent childhood-onset systemic lupus erythematosus has not been previously described.

Validity and sensitivity to change of laser Doppler imaging as a novel objective outcome measure for cutaneous lupus erythematosus.

The objectives of this study were to assess the reliability of a novel objective outcome measure, laser Doppler imaging (LDI), its validity against skin biopsy histology and other clinical instruments, including localized cutaneous lupus disease area and severity index (L-CLASI) and visual analogue scale (VAS) score of photographs, and its responsiveness to clinical change with therapy. A prospective observational cohort study was conducted in 30 patients with active cutaneous lupus erythematosus (CLE). At baseline and 3 months, disease activity was assessed using L-CLASI and a high resolution LDI system by two assessors. Skin biopsy was scored as 0 = non-active, 1 = mild activity and 2 = active. Photographs were assessed by two clinicians using 100 mm VAS. Inter-rater reliability was analyzed using Bland-Altman limits of agreement. Correlation between histology and LDI, L-CLASI and VAS and sensitivity to change of LDI with physician subjective assessment of change (PSAC) at 3 months were analyzed using Kendall's tau-a. Of 30 patients with CLE, 28 (93%) were female, mean (SD) age 48.4 (11.5) y, 25 (83%) were Caucasians, 25 (83%) had concurrent systemic lupus erythematosus and 16 (53%) were smokers. CLE subtypes were acute = 9, subacute = 8 and chronic = 13. Inter-rater agreement for LDI was fair but for VAS score of photographs was poor. In 20 patients with biopsy, correlation with histology was better for LDI (tau-a = 0.53) than L-CLASI (tau-a = 0.26) (difference = 0.27; 90% CI 0.05-0.49) or VAS score of photographs (tau-a = 0.17) (difference = 0.36; 90% CI 0.04-0.68). There was a moderate correlation between PSAC score and change in LDI (tau-a = 0.56; 90% CI 0.38-0.74; p < 0.001, n = 15). LDI provides a reliable, valid and responsive quantitative measure of inflammation in CLE. It has a better correlation with histology compared to clinical instruments. LDI provides an objective outcome measure for clinical trials.

Leptospirosis as Unusual Trigger of Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder of an unknown origin. The role of leptospirosis as a triggering factor for SLE is unknown. This paper reports an uncommon case of SLE following a leptospira infection. A 29-year-old female was referred due to fevers, myalgia, and facial edema with rash. Laboratory investigations revealed a hepatic dysfunction, significantly raised lactate dehydrogenase with marked leukopenia and thrombocytopenia. A diagnosis of leptospirosis was confirmed. The patient was treated with antibiotic therapy for leptospirosis. She developed dyspnea after one week. The echocardiogram revealed global hypokinesia with a decreased ejection fraction. A positivity of antinuclear, anti-DNA, and anti-Smith antibodies, together with clinical and laboratory improvement by steroid therapy, led to the diagnosis of SLE. This case highlights the presence of concurrent SLE and leptospirosis. As the symptoms of SLE are similar to leptospirosis, accurate diagnosis through high suspicion is essential for appropriate treatment.

Bone marrow as a target organ of systemic lupus erythematosus: analysis of cases with myelofibrosis.

Cytopenia in the course of systemic lupus erythematosus (SLE) may be due to multiple factors. In this study, we aimed primarily to evaluate the detailed results of bone marrow (BM) biopsies of SLE patients, secondly to determine the myelofibrosis (MF) frequency and thirdly to compare BM morphologic findings as well as the clinical and laboratory parameters between groups (with MF and without MF) in cytopenic SLE patients. We retrospectively analyzed 224 SLE patients' files. Patients were divided into two groups according to whether they had MF or not. Concurrent SLE organ involvements, medical therapy and detailed BM findings were recorded. Forty-five (20%) of 224 SLE patients were found to have undergone BM biopsy due to cytopenia. Four patients were excluded (two drug-induced cytopenia, one lymphoma, one insufficient BM biopsy samples). While MF was detected in 29 (70.7%) of the 41 patients, 12 patients did not have MF. Between the two groups, no differences were identified in terms Systemic Lupus Erythematosus Disease Activity Index, BM cellularity, or BM dysplastic changes (P = 0.788, P = 0.672 and P = 0.494, respectively). In the SLE-associated MF group, 27 patients responded to immunosuppressive therapy and corticosteroids, but two patients were unresponsive. The response time was longer for the SLE-associated MF group compared to the without MF group (3.3 ± 3.1 months vs. 1.7 ± 1.2 months, P = 0.091). Correlation analysis revealed that increased degree of BM fibrosis delayed the response time (r = 0.471, P = 0.002). MF is common in SLE patients. SLE-associated MF as an additional factor for cytopenia in SLE patients may lead to delayed response to appropriate therapy, which may be dependent on the increased grade of BM fibrosis.

Concomitant systemic lupus erythematosus and HIV infection

Rationale:Coexisting systemic lupus erythematosus (SLE) and human immunodeficiency virus (HIV) infection cases are rare worldwide. Great challenges are posed on the diagnosis and treatment of such concurrent cases.Patient concern:We report the case of a 44-year-old Chinese man with edema, hematuria, and fever who presented at West China Hospital, Sichuan University, Chengdu, Sichuan, China, in 2013.Diagnoses:An initial diagnosis of SLE was made from the clinical manifestations and laboratory findings based on the Systemic Lupus International Collaborating Clinics classification criteria. Immunosuppressant therapy relieved him of the edema and hematuria, but he regained the symptoms after a cold. Workup, including electrochemiluminescence immunoassay, western blot, and polymerase chain reaction analysis, revealed that he was concurrently infected with HIV after hospitalization.Interventions:The treatment plan included methylprednisolone and cyclophosphamide, with gastroprotective and hepatoprotective agents, simultaneously aiming to reduce urinary protein. After HIV infection confirmed, cyclophosphamide was stopped. He was referred to the local Centers for Disease Control and Prevention for combination antiretroviral therapy (ART). He was suggested to continue monitoring CD4 T-cell count for an appropriate dose of immunosuppressive drugs.Outcomes:In the last follow-up in May 2017, he had been stable in terms of both SLE and HIV infection.Lessons:The case highlights the presence of concurrent SLE and HIV infection. Laboratory technicians and clinicians should be cautious on diagnosis, especially in eliminating the false-positive results. Attention should be paid to the dose of immunosuppressants and the ART procedure.

Axl, Ferritin, Insulin-Like Growth Factor Binding Protein 2, and Tumor Necrosis Factor Receptor Type II as Biomarkers in Systemic Lupus Erythematosus.

To evaluate the performance of 4 serum protein markers for detecting concurrent clinical activity in patients with systemic lupus erythematosus (SLE). Consecutive patients who fulfilled ≥4 American College of Rheumatology classification criteria for SLE and healthy controls were recruited for serologic testing of 4 protein markers identified by antibody-coated microarray screen, namely Axl, ferritin, insulin-like growth factor binding protein 2 (IGFBP-2), and tumor necrosis factor receptor type II (TNFRII). SLE disease activity was assessed by the Safety of Estrogens in Lupus Erythematosus National Assessment version of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and physician's global assessment (PGA). Levels of these markers were correlated with SLEDAI scores, and their sensitivity and specificity for clinical SLE activity were determined. A total of 94 SLE patients (98% women, mean ± SD age 28.7 ± 9.4 years, mean ± SD disease duration 5.4 ± 5.0 years) and 49 healthy controls were studied. Fifty-two patients had clinically active SLE (defined as SLEDAI score ≥6 or having a flare). The serum concentrations of Axl, ferritin, IGFBP-2, and TNFRII were significantly higher in patients with active SLE than in those with inactive SLE or in controls. The levels of these markers correlated strongly and significantly with anti-double stranded DNA (anti-dsDNA), C3, and clinical SLEDAI and PGA scores. These markers were more specific, but less sensitive, in detecting concurrent SLE activity than elevated anti-dsDNA or depressed C3. Levels of Axl, TNFRII, and IGFBP-2, but not ferritin, could differentiate active renal from active nonrenal or inactive SLE. The specificity of Axl and IGFBP-2 for concurrent active lupus nephritis was higher than anti-dsDNA or C3. Serum proteomic markers are potentially useful for diagnosing SLE and monitoring disease activity. The performance of Axl and IGFBP-2 in lupus nephritis should be further explored in a longitudinal cohort of SLE patients.