Lupus Disease Activity Research Articles

A Severe Case of Lupus Cerebritis

Objective Lupus cerebritis is a rare but potentially fatal complication of SLE. Prompt diagnosis and rapid initiation of therapy can prove lifesaving. Background We present the case of a 31-year-old woman who presented to the emergency department with one week of fevers, headaches and confusion. Review of systems was notable for diffuse muscle and joint pains, weakness and a rash. SLE and Sjogren's were both diagnosed six months prior to admission and treatment included prednisone, methotrexate, and hydroxychloroquine. She self-discontinued therapy six weeks prior to seek natural remedies in her native Ecuador. Vitals on admission were 102.2 F, 111 beats/min, 96/67 mmHg, 16 breaths/min and 95% SO2 on 2 L/min via nasal cannula. Physical exam was notable for conjunctival injection, hypopyon (OD), and oral ulcers with visible bleeding. Pelvic exam showed shallow ulcerations of the vaginal mucosa. Neurological exam was significant for diffuse weakness. There were punched-out ulcers on the digits of both hands. Laboratory markers of lupus disease activity were markedly abnormal. An MRI of the brain showed numerous punctate foci of restricted diffusion in the supra and infratentorial brain parenchyma. Few of the lesions showed subtle rim-enhancement and microhemorrhagic foci. CSF analysis showed 220 WBC/mL (70% PMNs), glucose 13 mg/dL and protein 63.7 mg/dL. No CSF oligoclonal bands were detected. CSF/serum albumin index showed mild impairment of the blood brain barrier. Cultures of CSF, blood, urine and sputum showed no growth. Design/Methods NA. Results The patient improved significantly upon initiation of pulsed corticosteroids, plasma exchange, and cyclophosphamide. She was transitioned to steroid-sparing agents and is doing well. Conclusions Lupus cerebritis can be the dominant syndrome in a patient presenting with uncontrolled SLE. Imaging and CSF findings can be dramatic and evoke infectious syndromes. Once alternative diagnoses have been ruled out lupus cerebritis should be managed aggressively to ensure good outcomes.

LBODP074 Autoimmune Hypophysitis: Diagnosis Beyond Imaging

Abstract Background Lymphocytic (LH) or autoimmune hypophysitis (AH) is a rare inflammatory disorder of the hypophyseal gland, often miss-diagnosed. LH is predominant in females, occasionally related with other rheumatic disorders (1-20%). The disease can lead to pituitary dysfunction and symptoms of hypopituitarism, the most affected axes are the ACTH and TSH. Definitive diagnosis of AH can be based only on pathological examination of a pituitary biopsy sample but such invasive procedure is seldom needed. Different imaging modalities, especially MRI, can be useful but up to 30% can be unremarkable, thus clinical manifestations and biochemical evaluation is of great importance in guiding the diagnosis. Hormonal replacement therapy is the main treatment strategy. Immunosuppressive drugs are indicated by the severity of the symptoms and the underlying autoimmune disease. Clinical Case A 36 year old patient with active lupus and Sjogren disease presented to the emergency department with syncope and hypotension preceded by a 6 month history of intense fatigue. She did not take any steroids in the 6 months prior. Initial tests were consistent with secondary adrenal insufficiency; low plasma cortisol (<1ug/dl n 5-23 ug/dl) and ACTH-concentrations below detection (< 4 ng/l), central hypothyroidism; abnormal low TSH (0. 04uU/ml n 0.4-4.5 uU/ml) and low free T4 (0.56 ng/dl n 0.8-2 ng/dl), and growth hormone deficiency (IGF1 below age and sex normal values). Craneal CT and pituitary MRI didn't show any anomaly. Based on symptoms, clinical context and test results the pressumtive diagnosis was AH related to her lupus. The patient was treated with hydrocortisone and levothyroxine with excellent response and complete resolution of her symptoms. Clinical Lesson: This case demonstrates that hypophysitis is a clinical entity that can be suspected and treated based on clinical and past medical history even in the context of normal imaging were an invasive procedure such as a biopsy would likely not change the final outcome. Presentation: No date and time listed

Serum beta-2 microglobulin as a predictor of nephritis, disease activity, and damage score in systemic lupus erythematosus: a cross-sectional study

A strong correlation between lupus nephritis (LN), disease activity, and serum beta 2-microglobulin (b2MG) was observed. The current study examines the correlation between serum b2MG and renal involvement, damage score, and disease activity in systemic lupus erythematosus (SLE) patients. One hundred SLE patients from Ain Shams University Hospital were enrolled and categorized into two groups. Group I had 40 patients with negative b2MG, while Group II had 60 patients with positive b2MG levels. Medical history, clinical examination, and assessing disease activity based on SLE disease activity index (SLEDAI-2 K), and damage score were recorded for all patients. Laboratory examinations, such as serum b2MG, complete blood count, blood urea nitrogen (BUN), serum creatinine, glomerular filtration rate (GFR), urine analysis, 24 h urinary protein excretion, Antinuclear antibodies (ANA), anti-dsDNA antibody, and serum complement (C3, C4). BUN, 24 h urinary protein, serum creatinine, active urinary sediment, SLEDAI score, and damage score were all elevated in group II compared to group I (p < 0.001). There is a positive correlation between serum b2MG and 24 h urinary protein, BUN, serum creatinine, disease activity, and damage score (p < 0.001), while it was negatively correlated with GFR, C3, and C4 (p < 0.001). Serum b2MG has proven to be a predictor of LN in SLE patients (Sensitivity 92.45%, Specificity 74.47%), also being a predictor of the activity of the disease as well as damage index (Sensitivity 96.67%, Specificity 85%) (Sensitivity 92.45%, Specificity 74.47%), respectively. Serum b2MG level can be used as a valuable predictor for LN, clinical disease activity, and damage score.

Scrub Typhus-Induced Macrophage Activation Syndrome with Flare of Systemic Lupus Erythematosus: A Rare Case Report

Aims: Infection is a major trigger for both Lupus flare and Macrophage activation syndrome. Scrub typhus infection associated with lupus flare or MAS has been very rarely reported. We report a case of scrub typhus-induced MAS along with a flare of disease activity in a neuropsychiatric lupus patient presenting after discontinuation of medications.&#x0D; Presentation of Case: 13-year-old girl, a known case of neuropsychiatric lupus with lupus nephritis, on immunosuppressant for the last 1 year had stopped all medications for 3 months. She had presented with a fever and altered sensorium. Based on clinical and laboratory investigations, a diagnosis of scrub typhus-induced macrophage activation syndrome with a flare of lupus disease activity was made and the patient was treated with injection doxycycline and glucocorticoids. There was rapid clinical improvement with treatment.&#x0D; Discussion: The case emphasizes the importance of identifying the exact trigger behind the deterioration of a patient with lupus and its therapeutic implications. An infection alone requires antibiotics only, flare without infection requires up titrating immunosuppressants but the presence of life-threatening complications like MAS along with infection requires prompt treatment of both facets simultaneously.&#x0D; Conclusion: Scrub typhus infection should be ruled out apart from other common infections in any patient with lupus flare or MAS.

Efficiency of Disease and Disease Activity Diagnosis Models of Systemic Lupus Erythematosus Based on Protein Array Analysis.

Background Systemic lupus erythematosus (SLE) has become increasingly common in the clinic and requires complicated evidence of both clinical manifestations and laboratory examinations. Additionally, the assessment and monitoring of lupus disease activity are challenging. We hope to find efficient biomarkers and establish diagnostic models of SLE. Materials and Methods We detected and quantified 40 proteins using a quantitative protein array of 76 SLE patients and 21 healthy controls, and differentially expressed proteins were screened out by volcano plot. Logistic regression analysis was used to recognize biomarkers that could be enrolled in the disease diagnosis model and disease activity diagnosis model, and a receiver operating characteristic (ROC) curve was drawn to evaluate the efficiency of the model. A nomogram was depicted for convenient and visualized application of our models in the clinic. Decision curves and clinical impact curves were also plotted to validate our models. Results The protein levels of TNF RII, BLC, TNF RI, MIP-1b, eotaxin, MIG, MCSF, IL-8, MCP-1, and IL-10 showed significant differences between patients with SLE and healthy controls. TNF RII and MIP-1b were included in the SLE diagnosis model with logistic regression analysis, and the value of the area under the ROC curve (AUC) was 0.914 (95% confidence interval (CI), 0.859-0.969). TNF RII, BLC, and MIP-1b were enrolled in the disease activity diagnosis model, and the AUC value was 0.823 (95% CI 0.729-0.916). Both of the models that we established showed high efficiency. Additionally, the three protein biomarkers contained in the disease activity distinguish model provided additional benefit to conventional biomarkers in predicting active lupus. Conclusions The disease diagnosis model and disease activity diagnosis model that we developed based on protein array chip results showed high efficiency in differentiating patients with SLE from healthy controls and recognizing SLE patients with high disease activity, and they have also been validated. This implied that they might greatly benefit clinical decisions and the treatment of SLE.

Evolving phenotype of systemic lupus erythematosus patients and it's correlation with antibody against c1q

Backgrounds: Autoantibodies against C1q are strongly linked to immune-complex disorders like systemic lupus erythematosus (SLE). Although anti-C1q antibodies have received much interest in the recent years, their biological functions remain unclear. Aim and objectives: The main aim of this study was to analyze the phenotype of systemic lupus erythematosus at first presentation and during follow-up at Qena university hospital. Subjects and methods: This cross-sectional study was conducted at Qena university hospital. The study was conducted on 100 systemic lupus erythematosus patients. Results: Regarding the relation between lupus activity and anti-C1q antibody we found that there were highly statistically significant differences between lupus activity groups according to anti-C1q antibody. We also found that there were highly statistically significant differences between lupus activity groups according to anti-C1q with highly increasing in anti-C1q in patients with lupus activity when comparing with patients without lupus activity. Conclusion: Anti-C1q antibodies were strongly associated with lupus disease activity in SLE patients, suggesting that it may be a reliable, sensitive and non-invasive serological marker for identification of SLE patients with active SLE disease.

Urine inter-alpha-trypsin inhibitor family-related proteins may serve as biomarkers for disease activity of lupus.

BackgroundSystemic lupus erythematosus (SLE) is a chronic inflammatory disease involving multiple tissues. Inter‐Alpha‐Trypsin Inhibitor (ITI) family proteins have a role in maintaining tissue homeostasis, but their possible clinical significance in the SLE patients has not been reported. The aim of this study was to analyze and verify the expression of ITI‐related proteins in the urine of SLE patients, further explore the features of these proteins in disease activity.MethodsBased on label‐free proteomics technology and bioinformatics technology, we analyzed the expression of ITI family‐related proteins in the urine of lupus. Subsequently, Western‐blot and targeted proteomics were used to qualitatively and quantitatively verify the expression of these proteins, respectively.ResultsA total of seven ITI family‐related proteins were screened and identified; and six of these proteins were differentially expressed in the urine of SLE patients. Further quantitative analysis showed that the expressions of ITIH2, ECM1, and ITIH5 in urine between active SLE group and stable SLE group were consistent with the preliminary screening results. The expression of ITIH2 and ECM1 in the renal damage group were also consistent with the screening results. Moreover, ITIH2 and ECM1 have a good correlation with disease activity and have a certain correlation with renal damage.ConclusionsIn this exploratory study, we evaluated the expression of ITI family‐related proteins in the urine of SLE and found that urine ITIH2 and ECM1 were closely related to SLE activity, especially kidney damage, providing an experimental basis for further exploration of the potential roles in monitoring lupus and lupus nephritis activity.

Therapeutic potential of interleukin-2 in autoimmune diseases.

Autoimmune diseases are characterized by dysregulation and aberrant activation of cells in the immune system. Therefore, restoration of the immune balance represents a promising therapeutic target in autoimmune diseases. Interleukin-2 (IL-2) can promote the expansion and differentiation of different immune cell subsets dose-dependently. At high doses, IL-2 can promote the differentiation and expansion of effector and memory T cells, whereas at low doses, IL-2 can promote the differentiation, survival, and function of regulatory T (Treg) cells, a CD4+ T cell subset that is essential for the maintenance of immune homeostasis and immune tolerance. Therefore, IL-2 exerts immunostimulatory and immunosuppressive effects in autoimmune diseases. The immunoregulatory role of low-dose IL-2 has sparked excitement for the therapeutic exploration of modulating the IL-2-Treg axis in the context of autoimmune diseases. In this review, we discuss recent advances in the therapeutic potential of IL-2 or IL-2-derived molecules in the treatment of autoimmune diseases.

Hypomethylation of miR-17-92 cluster in lupus T cells and no significant role for genetic factors in the lupus-associated DNA methylation signature

ObjectivesLupus T cells demonstrate aberrant DNA methylation patterns dominated by hypomethylation of interferon-regulated genes. The objective of this study was to identify additional lupus-associated DNA methylation changes and determine the...

α-2,6-sialic acid/IgG anti-dsDNA ratios correlate with human lupus disease activity and possible mechanisms: A pilot study.

To study the association of α2,6-sialic acid (SIA) content in serum IgG anti-dsDNA with human systemic lupus erythematosus disease activity index (SLEDAI) and the effect of sialylated and desialylated (deSIA) IgG anti-dsDNA on lupus B cells. Blood from lupus patients was collected to determine the ratio of SIA in isolated IgG anti-dsDNA over serum IgG anti-dsDNA (SIA/IgG anti-dsDNA) ratios, which were plotted against SLEDAI using a receiver-operating-characteristics curve. Lupus B cells were cultured in vitro with chimeric sialylated IgG anti-dsDNA and its deSIA form. Culture supernatants were assayed for anti-inflammatory IL-10 and SIA/IgG anti-dsDNA ratios, which were compared among different pre-treatment groups using t-tests. The area-under-the-curve (AUC) for anti-dsDNA levels against SLEDAI was 0.791 positively (95% confidence interval [C.I.]: 0.699-0.884) and SIA/IgG anti-dsDNA ratios against SLEDAI yielded an AUC of 0.705 inversely (95% C.I: 0.601-0.809): not significantly different. SIA/IgG anti-dsDNA ratios discriminated significantly between patients without and patients with proteinuria (p = .046). SIA/IgG anti-dsDNA ratios correlated significantly and positively with serum C3c and C4 levels. Pre-treatment with IgG anti-dsDNA and its immune complexes (dsDNA/IgG anti-dsDNA IC) induced higher IL-10 from lupus B cells than medium pre-treatment (most p < .01 from day 2 to day 5 culture). DeSIA IgG anti-dsDNA IC induced lower IL-10 (p < .05) and lower SIA/IgG anti-dsDNA ratios (p < .001) from lupus B cells than medium and dsDNA pre-treatment. α2,6-SIA/IgG anti-dsDNA ratios inversely forecasted SLEDAI scores. Possible mechanisms may be due to the different effects of sialylated and deSIA IgG anti-dsDNA on lupus B cells in terms of IL-10 secretion and SIA/IgG anti-dsDNA ratios.

2022 Systemic lupus erythematosus remission in clinical practice. Message for Polish rheumatologists.

Systemic lupus erythematosus (SLE) is a complicated multiorgan disease and can lead to organ damage and increased risk of morbidity and mortality. The strategy of management while avoiding complications, especially caused by chronic glucocorticoid therapy, improves outcomes. Different definitions of the treatment goal in different configurations of lupus activity indexes have appeared over the years. In 2021 the definition of remission and recommendations for its achievement were published and it become a way to implement a treat-to-target strategy. The main goal of treatment has become DORIS (definition of remission in SLE) remission and the alternative LLDAS (low lupus disease activity state).Prolonging remission with clinical and immunological lupus activity restrictions and minimizing or stopping steroid doses reduced flares and damage accrual. The analysis and neutralization of poor prognosis predictive factors in lupus could be the most beneficial for less morbidity and mortality and better quality of life.

Advances in the clinical use of hydroxychloroquine levels.

This review summarizes the recent literature exploring hydroxychloroquine levels and their relationship with disease activity and risk of toxicity. There is no clear correlation between weight-based dosing of hydroxychloroquine and the resulting blood levels of the medication. Recent studies have shown that increased hydroxychloroquine levels are associated with lower lupus disease activity and likely also increased risk of medication toxicity. Mounting evidence supports use of hydroxychloroquine levels in clinical practice to document adherence and ensure safety.

Tumor necrosis factor receptor 2 and anti-ribosomal P antibodies as biomarkers in juvenile neuropsychiatric systemic lupus erythematosus

BackgroundNeuropsychiatric systemic lupus erythematosus (NPSLE) is more frequent in adolescents than in adults and is indistinguishable from regular mood disorders. AimTo investigate the levels of soluble tumor necrosis factor receptor 2 (sTNFR2) and anti-ribosomal P (anti-rib-p) antibodies in the serum of adolescents suffering from juvenile lupus and their correlation with neuropsychiatric manifestations (NP) and disease activity. MethodsSixty adolescents diagnosed with systemic lupus erythematosus (SLE) visiting Ain Shams University hospitals were assessed for NPSLE. Subjects were divided into 3 groups. Group 1 comprised 30 patients suffering from NP manifestations, while group 2 included 30 patients without NP manifestations. Computerized Axial Tomography (CAT) scans of the brain were done for NPSLE patients to exclude organic disease. Group 3 comprised 30 age and gender-matched healthy adolescents. Lupus activity was evaluated by the Systemic Lupus Disease Activity Index (SLEDAI). Levels of sTNFR2 and anti-rib-P antibodies were analyzed using enzyme-linked immunosorbent assay (ELISA). ResultsThe levels of sTNFR2 and anti-rib-P antibodies were significantly higher (P < 0.0001) in lupus patients compared to healthy subjects. Anti-rib-P antibodies levels were significantly higher in patients with NPSLE compared to those without NPSLE. Levels of sTNFR2 correlated positively with SLEDAI and ESR. Similar correlations were observed for anti-rib-P antibodies. ConclusionThe study showed that anti-rib-P antibodies could be used as biomarkers for the early diagnosis of NPSLE and sTNFR2 could be a valuable marker for the evaluation of juvenile lupus activity.

Central Nervous System Systemic Lupus Erythematosus: Pathophysiologic, Clinical, and Imaging Features

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by multiple immunologic abnormalities and has the potential to involve the central nervous system (CNS). The prevalence of SLE seems to be growing, possibly because of earlier diagnosis and improved survival; however, the associated mortality is still high. The mortality is associated with disease-related risk factors such as lupus disease activity, young age, and organ damage or with antiphospholipid syndrome (APS). Neuropsychiatric SLE (NPSLE), which is caused by SLE-related CNS involvement, comprises a broad range of neurologic and psychiatric manifestations with varying severity, which can make this disease indistinguishable from other conditions that are unrelated to SLE. No unifying pathophysiology has been found in the etiology of NPSLE, suggesting that this condition has multiple contributors such as various immune effectors and the brain-intrinsic neuroimmune interfaces that are breached by the immune effectors. The postulated neuroimmune interfaces include the blood-brain barrier, blood-cerebrospinal fluid barrier, meningeal barrier, and glymphatic system. On the basis of the immunologic, pathologic, and imaging features of NPSLE, the underlying pathophysiology can be classified as vasculitis and vasculopathy, APS, demyelinating syndrome, or autoimmune antibody-mediated encephalitis. Each pathophysiology has different imaging characteristics, although the imaging and pathophysiologic features may overlap. Moreover, there are complications due to the immunocompromised status caused by SLE per se or by SLE treatment. Radiologists and clinicians should become familiar with the underlying mechanisms, radiologic findings, and complications of NPSLE, as this information may aid in the diagnosis and treatment of NPSLE. Online supplemental material is available for this article.©RSNA, 2022.

Immunogenicity and safety of inactivated and mRNA COVID-19 vaccines in patients with systemic lupus erythematosus.

Objective:To evaluate the effects and side effects of both inactivated and mRNA COVID-19 vaccines in patients with systemic lupus erythematosus (SLE).Methods:This was a prospective, single-center, observational study. Patients with SLE planning to receive COVID-19 vaccines were recruited and matched 1:1 with healthy controls. The immunogenicity of the COVID-19 vaccines was assessed by a surrogate neutralization assay at 28 days after the second dose. The main outcome was the antibody response comparing SLE patients and controls. Other outcomes included reactogenicity, disease activity and predictors of antibody responses in patients with SLE.Results:Sixty-five SLE patients received 2 doses of COVID-19 vaccines (Comirnaty: 38; CoronaVac: 27) were recruited. Many of them were on systemic glucocorticoids (76%) and immunosuppressants (55%). At day 28 after the second dose of vaccines, 92% (Comirnaty: 100% vs CoronaVac: 82%, p = 0.01) of the patients had positive neutralizing antibody. However, compared to the age, gender, vaccine type matched controls, the level of neutralizing antibody was significantly lower (p < 0.001). The self-reported adverse reactions after vaccines in lupus patients were common but mild, and were more frequent in the Comirnaty group. There was no significant change in lupus disease activity up to 28 days after vaccination. The independent predictors of neutralizing antibody level included the dosage of systemic glucocorticoids, use of mycophenolate and type of vaccines.Conclusions:COVID-19 vaccines produced satisfactory but impaired humoral response in SLE patients compared to controls which was dependent on the immunosuppressive medications use and type of vaccines received. There was no new short-term safety signal noted. Booster dose is encouraged.

Pregnancy outcomes between pregnant systemic lupus erythematosus patients with clinical remission and those with low disease activity: A comparative study.

This study aims to compare pregnancy outcomes between systemic lupus erythematosus (SLE) patients who attained clinical remission based on the Definition of Remission in SLE (DORIS) and those with lupus low disease activity based on Low Lupus Disease Activity State (LLDAS). Between January 1993 and June 2017, a total of 90 pregnancies (one twin pregnancy) from 77 patients (mean age: 26.9±4.8 years; range, 17.9 to 37.3 years) were included in the study. The clinical remission and the LLDAS groups were modified into modified clinical remission and LLDAS groups, respectively by omitting Physician Global Assessment (PGA). The clinical SLE disease activity index (cSLEDAI) score was used for LLDAS. Pregnancies in 49 patients occurred, when they were in modified clinical remission and in 57 in modified LLDAS. There was no significant difference in demographic characteristics, disease activity, or medication received at conception between the two groups. Pregnancy outcomes were similar between the modified clinical remission and the modified LLDAS groups in terms of successful pregnancy (83.67% vs. 84.21%), full-term births (38.78% vs. 38.60%), fetal losses (16.33% vs. 15.79%), spontaneous abortions (14.29% vs. 14.04%), small for gestational age infants (18.37% vs. 19.30%), low birth weight infants (42.86% vs. 40.35%), maternal complications (46.94% vs. 49.12%), and maternal flares (36.73% vs. 40.35%). The agreement of pregnancy outcomes was very high between the two groups (91.11% agreement). Pregnancy outcomes in SLE patients who achieved modified clinical remission and modified LLDAS were comparable.

Impact of glucocorticoids on the incidence of lupus-related major organ damage: a systematic literature review and meta-regression analysis of longitudinal observational studies

ObjectiveIn systemic lupus erythematosus (SLE), disease activity and glucocorticoid (GC) exposure are known to contribute to irreversible organ damage. We aimed to examine the association between GC exposure and organ...

B Cell Signatures Distinguish Cutaneous Lupus Erythematosus Subtypes and the Presence of Systemic Disease Activity.

Cutaneous lupus erythematosus (CLE) is a chronic inflammatory skin disease characterized by a diverse cadre of clinical presentations. CLE commonly occurs in patients with systemic lupus erythematosus (SLE), and CLE can also develop in the absence of systemic disease. Although CLE is a complex and heterogeneous disease, several studies have identified common signaling pathways, including those of type I interferons (IFNs), that play a key role in driving cutaneous inflammation across all CLE subsets. However, discriminating factors that drive different phenotypes of skin lesions remain to be determined. Thus, we sought to understand the skin-associated cellular and transcriptional differences in CLE subsets and how the different types of cutaneous inflammation relate to the presence of systemic lupus disease. In this study, we utilized two distinct cohorts comprising a total of 150 CLE lesional biopsies to compare discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE), and acute cutaneous lupus erythematosus (ACLE) in patients with and without associated SLE. Using an unbiased approach, we demonstrated a CLE subtype-dependent gradient of B cell enrichment in the skin, with DLE lesions harboring a more dominant skin B cell transcriptional signature and enrichment of B cells on immunostaining compared to ACLE and SCLE. Additionally, we observed a significant increase in B cell signatures in the lesional skin from patients with isolated CLE compared with similar lesions from patients with systemic lupus. This trend was driven primarily by differences in the DLE subgroup. Our work thus shows that skin-associated B cell responses distinguish CLE subtypes in patients with and without associated SLE, suggesting that B cell function in skin may be an important link between cutaneous lupus and systemic disease activity.

Association of Mean Platelet Volume (MPV) in Relation to Disease Activity in Lupus

Introduction: Among the various clinical and biochemical parameters which are employed to monitor the disease activity in Systemic Lupus Erythematosus (SLE), Mean Platelet Volume, which is a platelet activation biomarker has been recently studied. We intended at evaluating the MPV in patients with active SLE and comparing it with the same patients who were in clinical remission, and to study the correlation between MPV and SLEDAI. We also studied the correlation between MPV and ESR, complements C3 and C4.&#x0D; Methods: This is a prospective study conducted for a period of 12 months, in which 50 consecutive patients who were recently diagnosed with SLE according to the SLICC classification criteria were included. Complete blood count with MPV levels along with ESR, were measured at the first visit to the hospital and were repeated at every visit till they attained clinical remission. Analysis of MPV was done at first visit and at remission. Complements C3 and C4 was done at the first visit and at remission. The results were then tabulated and statistically analysed using SPSS 25. A Pearson’s correlation test was done to assess the relation between MPV, ESR and SLEDAI.&#x0D; Results: Patients with active disease had a decreased MPV as compared to those in clinical remission (10.91fl, 13.11fl, p=0.011). We observed a weak positive correlation between MPV and SLEDAI (r=0.034, p=0.011). We observed no correlation between MPV and ESR, C3 and C4. Meanwhile, SLEDAI showed a positive correlation with C3 and C4; there was no correlation with ESR though.&#x0D; Conclusion: MPV is reduced in patients with active SLE and displayed an inverse correlation with SLEDAI.

Patient-reported outcome measures in systemic lupus erythematosus by a web-based application: A randomized, crossover, agreement study.

Patient-reported outcome measures (PROMs) are evaluated in randomized controlled trials (RCTs) in patients with systemic lupus erythematosus (SLE), but not widely used in clinical practice. However, interest in incorporating PROMs into the management of SLE is increasing as PROMs provide a unique insight into the patient's perception of lupus disease activity. The objective was to assess agreement in PROMs answered using a web app versus an outpatient touchscreen among patients with SLE. In a crossover RCT, SLE patients answered the following PROMs in a random order using the web app and the outpatient touchscreen: Systemic Lupus Erythematosus Activity Questionnaire (SLAQ) Global Health, SLAQ Symptom, SLAQ Total, SLAQ Worsening, Pain Visual Analog Scale (VAS), Fatigue VAS, Patient Global Health VAS, Health Assessment Questionnaire Disability Index (HAQ-DI), Patient Acceptable Symptom State (PASS), and an Anchoring Question. Equivalence between the two device types was demonstrated if the 95% confidence interval (95% CI) of the difference in PROM scores was within the prespecified equivalence margin. Agreement between the two device types was assessed using mixed linear models. Thirty-four patients with SLE were included. Equivalence was demonstrated between the two device types for SLAQ Global Health with a difference of -0.21 (95% CI: -0.65 to 0.23). Moreover, equivalence was also found for HAQ-DI, Pain VAS, and Fatigue VAS whereas only comparability within the limits of the Minimal Clinically Important Difference (MCID) was demonstrated for VAS Patient Global Health. Statistical comparability was demonstrated for SLAQ Total, SLAQ Worsening, PASS, and Anchoring Question (no predefined MCID/equivalence margins available). However, a statistically significant difference between device types was observed for the SLAQ Symptom of -0.56 (95% CI: -1.10 to -0.01). The difference was, however, very small when considering the scale range of 0-24; thus, it was not judged to be of clinical relevance. Preference for the web app was very high (91.2%). For the first time ever, equivalence and comparability between two electronic device types for various PROMs were demonstrated among patients with SLE. Implementation of the device is expected to improve the management of SLE.