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Background: Systemic Lupus Erythematosus (SLE) may affect one or more organ systems; as time goes on, other manifestations may start to appear. Musculoskeletal, cutaneous, renal, and endocrine systems are all involved in SLE. The nervous system, hematological, vascular, pulmonary, gastrointestinal, and ocular imbalance of the immune response and the production of autoantibodies such as anti-oxidized Low-Density Lipoprotein LDL antibodies have a clear impact on the body’s organs and the development of complications of the disease. Objectives: To assess the levels of anti-oxLDL (IgM-Abs) and anti-oxLDL (IgG-Abs) as biomarkers for disease activity in SLE patients and their relationship to demographic characteristics. Patients and Methods: The study comprised 100 of SLE patients admitted to the Rheumatology Unit at Baghdad Teaching Hospital, age range (33.4 to 9.95) years, including 7 males and 93 females. An enzyme-linked immunosorbent Assay ELISA was used to measure the levels of serum oxLDL (IgM-Abs) and oxLDL (IgG-Abs). Results: The results of the present study showed that there was a significant difference between the levels of anti-oxLDL IgM antibodies(Abs) in SLE patients in obese and non-obese groups since the levels of anti-oxLDL-IgM Abs in obese patients were (3.14 µg/L) and non-obese patients were (5.13 µg/L) (P=0.005), while in SLE patients with Diabetes Mellitus (D.M.), the levels of anti-ox LDL-IgM Abs were (3.80 µg/L) and in SLE patients with no DM were (5.13 µg/L). Also, the results showed that there were no significant differences between levels of anti-oxLDL IgG Abs in obese patients with SLE (6.28 µg/L) and non-obese patients with SLE (10.25µg/L) P > 0.05. Conclusion: There was found a significant difference between levels of anti-oxLDL IgM Abs in obese and non-obese in patients with SLE, and no significant differences between the levels of anti-oxLDL IgG Abs in the same groups of patients.

Endometrial tuberculosis (TB) is an uncommon manifestation of disseminated TB. Rhupus is the coexistence of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). We describe a case of endometrial TB in rhupus patient an immunosuppressed. Contribution: We describe an uncommon presentation of disseminated TB, endometrial TB, in a rare rheumatic disease, rhupus. A high index of suspicion for TB is imperative in immunocompromised patients presenting with chronic urogenital symptoms especially in an endemic area.

In humans, insulin resistance is a physiological response to infections developed to supply sufficient energy to the activated immune system. This metabolic adaptation facilitates the immune response but usually persists after the recovery period of the infection and predisposes the hosts to type 2 diabetes and vascular injury. In patients with diabetes, superimposed insulin resistance worsens metabolic control and promotes diabetic ketoacidosis. Pathogenic mechanisms underlying insulin resistance during microbial invasions remain to be fully defined. However, interferons cause insulin resistance in healthy subjects and other population groups, and their production is increased during infections, suggesting that this group of molecules may contribute to reduced insulin sensitivity. In agreement with this notion, gene expression profiles (transcriptomes) from patients with insulin resistance show a robust overexpression of interferon- stimulated genes (interferon signature). In addition, serum levels of interferon and surrogates for interferon activity are elevated in patients with insulin resistance. Circulating levels of interferon- γ-inducible protein-10, neopterin, and apolipoprotein L1 correlate with insulin resistance manifestations, such as hypertriglyceridemia, reduced HDL-c, visceral fat, and homeostasis model assessment-insulin resistance. Furthermore, interferon downregulation improves insulin resistance. Antimalarials such as hydroxychloroquine reduce interferon production and improve insulin resistance, reducing the risk for type 2 diabetes and cardiovascular disease. In addition, diverse clinical conditions that feature interferon upregulation are associated with insulin resistance, suggesting that interferon may be a common factor promoting this adaptive response. Among these conditions are systemic lupus erythematosus, sarcoidosis, and infections with severe acute respiratory syndrome-coronavirus-2, human immunodeficiency virus, hepatitis C virus, and Mycobacterium tuberculosis., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Systemic Lupus Erythematosus (SLE) is a multifactorial chronic systemic autoimmune disease. It is characterized by a lack of immune tolerance to autoantigens such as nuclear antigens. The aim of the study is to assess the interferon-alpha (IFN-α) serum level in Iraqi patients with SLE and determine its potential relation to different clinical and laboratory parameters and disease activity. 100 SLE patients were all females and with a mean of age 31.3 ± 10 years (16- 63years) and disease duration of 5.8 ± 3.7years (1 month to 15 years). The average of SLEDAI score ranged from 2 to 22 with a mean of (8.53 ±3.42). Proteinuria, ESR, creatinine and AST were significantly higher (65% vs. 10% and 0.62±0.11 vs. 0.70±0.14 mg/dl respectively) while the PLT was significantly lower (231.9±88.8 vs. 282.3±67.3 103/mL) (p< 0.001) among SLE patients as compared to control. Serum levels of IFN-α were increased in the SLE patients compared to control, and no significant difference has been observed (208.7±530.0 vs. 63.7±34.8 pg/ml) respectively (P=0.245). Interferon-alpha showed a significant negative correlation with the SLE Disease Activity Index (SLEDAI) in the active and inactive groups. There were no significant variations in all study parameters across IFN-α serum levels (p greater than 0.05). In conclusion, the results suggest a risk effect for female gender and age in etiology of SLE. IFN-α could not be considered as biomarker or to have a risk effect in SLE patients or perpetuate the disease activity. No evidence for any correlation between the IFN-α serum level and any clinical manifestations or laboratory investigation of the disease in current study except for age and disease duration, which suggests them as a risk factor for increasing the IFN-α serum level. [ABSTRACT FROM AUTHOR]

Objectives: This study aims to determine the frequency of personality disorders in patients with systemic lupus erythematosus (SLE) and healthy volunteers and to compare SLE patients with and without personality disorders in terms of quality of life (QoL) and other clinical and laboratory findings. Patients and methods: Between January 2021 and March 2021, a total of 64 patients (17 males, 47 females; mean age: 42.9±10.8 years; range, 21 to 62 years) who were diagnosed with SLE and 68 age- and sex-matched healthy volunteers (20 males, 48 females; mean age: 40.9±10.6 years; range, 21 to 65 years) without any known disease were included. The Nottingham Health Profile (NHP) was filled in to evaluate the QoL for all participants. For the diagnosis of personality disorder, the Structured Clinical Interview For DSM-5 Personality Disorders (SCID-5PD) form was used. Clinical and laboratory findings of patients with SLE were noted and disease activity index (SLEDAI) was calculated. Clinical and laboratory variables that may affect personality disorder were evaluated. Results: The prevalence of personality disorder in SLE patients was significantly higher than the control group (39.1% vs. 11.8%, respectively; p<0.001). In terms of the subgroups of personality disorders detected in SLE, only the prevalence of obsessive-compulsive personality disorder was significantly higher than the control group (26.6% vs. 10.3%, respectively; p=0.015). The frequency of personality disorder increased, as the education level decreased, the duration of SLE disease increased, and with antiphospholipid autoantibodies positivity in patients with SLE (p<0.05). The mean NHP total score was 126.1±55.1 in SLE patients with personality disorder and 62.9±43.8 in patients without personality disorder, indicating that the QoL of SLE patients with personality disorder was worse than those without personality disorder (p<0.001). Conclusion: The frequency of personality disorder in SLE seems to be higher than in the control group. Quality of life is adversely affected in SLE patients with personality disorders. Therefore, clinicians should be alert for personality disorders that may accompany SLE and fight with personality disorder with early diagnosis and optimal treatment. [ABSTRACT FROM AUTHOR]

Background: In different studies, the effectiveness of compassion-focused therapy and acceptance and commitment therapy on reducing psychological problems of patients with chronic disease has been investigated, but no research has compared the effectiveness of compassion-focused therapy and acceptance and commitment therapy on distress tolerance in women with Systemic lupus Erythematosus (SLE). Aims: The present research was aimed to compare the effectiveness of compassion-focused therapy and acceptance and commitment therapy on distress tolerance in women with SLE. Method: The current study was a semi-experimental research in which a pretest-posttest with control group design was used. The research population included all the women with SLE referring to the Rheumatology Clinic of Imam Khomeini Hospital and Tooba Clinic in Sari city, Iran, in the first half of 2019 who were156 individuals. Among this statistical population, a sample of 60 subjects were selected by purposeful sampling method who were assigned into two experiment groups and one control group by random assignment method. The research tool was Distress Tolerance Scale of Simons and Gaher (2005). The first experiment group received the compassion-focused therapy (Gilbert, 2010) and the second experiment group received the acceptance and commitment therapy (Hayes, Strosahl and Wilson, 2016) both in eight 120-minute sessions. For analyzing the data, the variance analysis with repeated measures and the Bonferroni ad hoc test were used. Results: Results indicated that compassion-focused therapy and acceptance and commitment therapy led to increase in distress tolerance and its components (tolerance, Absorption, Appraisal and regulation) at the end of the interventions and the follow-up stage. Also, compassion-focused therapy was more effective on increasing distress tolerance and its components than acceptance and commitment therapy (p< 0/01). Conclusions: The results of the present study confirmed the effectiveness of compassion-focused therapy and acceptance and commitment therapy on increasing distress tolerance among with SLE.

Nephrotic syndrome (NS) can be a symptom of many autoimmune, metabolic, or infectious diseases. Kidney involvement is often observed in the course of diabetes mellitus (DM) and systemic lupus erythematosus (SLE). The development of NS with coexisting SLE and DM generates serious diagnostic problems. In this paper, the authors present diagnostic and therapeutic dilemmas in a patient with long-lasting DM, SLE, and secondary antiphospholipid syndrome, in whom NS symptoms appeared. Histopathological examination of the kidney confirmed the diagnosis of lupus nephritis. Immunosuppressive and anticoagulant drugs were used. The authors demonstrated that the character of morphologic lesions in the kidney biopsy can help in diagnosis, nephropathy classification, and further therapeutic decisions, which are distinct in both diseases.

Iman M Eissa, Ghada A Nassar, Shaimaa A Arfeen, Ahmed A Dahab Department of Ophthalmology, Cairo University, Giza, EgyptCorrespondence: Ahmed A DahabCairo University, 27 Madinat Al-Mabouthin, Giza, EgyptTel +966559231865Email dr.adahab@yahoo.comPurpose: To evaluate the corneal pachymetric and topographic parameters of systemic Lupus Erythematosus (SLE) patients using Dual Scheimpflug Imaging.Methods: This observational cross-sectional controlled study included the right eye of 30 SLE patients and 30 age-matched controls. Corneal measurements were acquired by dual Scheimpflug imaging including anterior and posterior corneal curvatures, central, mid-peripheral corneal thickness (measured at the 5 mm zone) and peripheral pachymetry (measured at the 7 mm zone). SLE disease activity index (SLEDAI) was calculated and correlated with corneal pachymetry.Results: SLE patients had significantly thicker corneal periphery than controls. Mean central corneal pachymetry was 530.4± 27.3 microns (SD) in SLE and 547.5± 31.5 microns (SD) in control group, p = 0.032. The corneal periphery – except superiorly – was significantly thicker in SLE patients than controls (p ˂0.001). Nasal peripheral corneal thickness positively correlated with disease activity index SLEDAI (p=0.03).Conclusion: SLE patients present with thicker corneal periphery than controls characteristically sparing the superior quadrant. Possible corneal photosensitivity leading to peripheral immune complex deposition as well as flatter posterior corneal surface at the periphery are proposed explanations for these findings.Keywords: cornea, corneal pachymetry, corneal topography, SLEDAI score, systemic lupus erythematosus

Immunoinflammatory rheumatic diseases (IIRDs) are a large group of pathological conditions with impaired immunological tolerance to autogenous tissues, leading to inflammation and irreversible organ damage. The review discusses current ideas on the role of type I interferons in the immunopathogenesis of IIRDs, primarily systemic lupus erythematosus, and new possibilities for personalized therapy.

Peripheral ulcerative keratitis (PUK) is a group of diseases that manifest with ulceration and/or thinning in the peripheral cornea. Although this group of diseases can occur as a result of infection, most cases are of immunological origin and are associated with autoimmune diseases (AID). The most common form of AID associated with PUK is rheumatoid arthritis (RA). However, PUK may also be seen with other AIDs, such as systemic lupus erythematosus and Wegener's granulomatosis. There are immunological differences between the peripheral and central cornea that may explain the localization of PUK in the peripheral cornea. For example, the proximity to limbal blood vessels and conjunctival lymphatics is different. Both humoral and cellular immunity play a role in the development of PUK. Although the number of CD8+ T cells did not vary significantly in RA patients, the number of CD4+ T cells was significantly greater. As PUK is very serious, early diagnosis and treatment are very important. Local and systemic corticosteroids, immunosuppressants, biological agents, bandage contact lenses, tissue adhesives, and in some cases, surgical treatment can be applied. Adjacent conjunctival excision, patch lamellar grafts, and keratoplasty are options in surgical treatment. It is very important not to forget the possibility of disease progression despite surgery, and the treatment of underlying disease is crucial. Keratoplasty in PUK cases may not be successful as a result of several factors, such as dry eye and the underlying immunological disease.

Coexistence of ankylosing spondylitis with connective tissue diseases is very rare. Here, in this study, we describe a coexistence of ankylosing spondylitis and discoid lupus erythematosus in a 35-year-old man. He presented with a 5-year history of low back pain and concurrent development of a discoid rash. Inflammatory low back pain, HLA-B27 positivity and bilateral active sacroiliitis confirmed the diagnosis of ankylosing spondylitis. Discoid lupus erythematosus was diagnosed based on a skin biopsy. There are reports of discoid lupus associated with medications, particularly with tumour necrosis factor-alpha (TNF-α) blocking drugs. However, the patient presented here had coexistence of ankylosing spondylitis and discoid lupus before starting such treatments.

Introduction: Histoplasmosis is an opportu­nistic granulomatous fungal infection. Peritoneal histoplasmosis (PH) is a rare form of dissem­inated histoplasmosis (DH). Case description: A 44-year-old male pa­tient with history of HIV infection, systemic lupus erythematosus with lupus nephritis, and chronic kidney disease on peritoneal dialysis, presented with recurrent ascites without clear etiology in the serial study of ascitic fluid. Con­trast computed tomography of the abdomen showed a nonspecific peritoneal nodule, for which laparoscopy and biopsy were performed, identifying Histoplasma capsulatum infection. The subject received 15 days of in-hospital treatment with amphotericin B deoxycholate with good evolution, no recurrence of ascites, and indefinite outpatient management with itraconazole. Discussion: The first case of PH was described in 1970 but this is the first case reported in Co­lombia. Most cases of PH have been associated with peritoneal dialysis. Its diagnosis is based on the study of ascitic fluid and its treatment follows the guidelines of other forms DH. Conclusions: PH is a rare entity that requires high clinical suspicion, especially in immuno­compromised patients.

Based on the recent evidence of association between hyperprolactinemia and systemic lupus erythematosus disease activity (SLEDAI), a study was conducted to analyze the association of hyperprolactinemia with lupus nephritis disease activity. In this cross-sectional study, the analysis was conducted on SLE patients who visited the University Kebangsaan Malaysia Medical Centre (UKMMC) Nephrology Clinic from August 2015 till February 2016. The disease activity was measured using the SLEDAI score, with more than 4 indicating active lupus nephritis. Basal resting prolactin level was analyzed in 43 patients with lupus nephritis, in 27.9% of them had raised serum prolactin. The median of serum prolactin level at 0 minutes was 19.91 ng/mL (IQR: 15.95-22.65 ng/ mL) for active lupus nephritis, which was significantly higher compared to the median of serum prolactin level of 14.34 ng/mL (IQR: 11.09-18.70 ng/mL) for patients in remission (p=0.014). The serum prolactin level positively correlated with SLEDAI (rhos: 0.449, p=0.003) and the UPCI level in lupus nephritis patients (rhos: 0.241, p=0.032). The results were reproduced when the serum prolactin was repeated after 30 minutes. However, the serum prolactin levels at 0 minutes were higher than those taken after 30 minutes (p=0.001). An assessment of serum IL-6 levels found that the active lupus nephritis patients had a higher median level of 65.91 pg/ mL (IQR: 21.96-146.14 pg/mL) compared to the in-remission level of 15.84 pg/mL (IQR: 8.38-92.84 pg/mL), (p=0.039). Further correlation analysis revealed that there was no statistical correlation between the interleukin (IL)-6 levels with serum prolactin, SLEDAI and other lupus nephritis parameters. An ROC curve analysis of serum prolactin at 0 minutes and serum prolactin after 30 minutes and IL-6 levels for prediction of SLE disease activity provided the cutoff value of serum prolactin at 0 minutes, which was 14.63 ng/mL with a sensitivity of 91.7% and specificity of 58.1% and AUC of 0.74 (p=0.015). This study concurred with the previous findings that stated that hyperprolactinemia is prevalent in SLE patients and correlated with clinical disease activity and UPCI level. The baseline of the fasting serum prolactin level was found to be a sensitive biomarker for the evaluation of lupus nephritis disease activity.

Objectives: Aim of the study was to perform a systemic review and meta-analysis of the current case-control studies based on the assessment of the left ventricular (LV) systolic function with standard and advanced echocardiographic methods., Materials and Methods: Objectives of the study, methods of statisticalanalysis, literature search strategy, inclusion andexclusion criteria, and outcome measurementswere defined according to Cochrane Collaborationsteps, 13 including recommendations for metaanalysisof observational studies in epidemiology (MOOSE)., Results: A total of 850 papers were collected. Of those, eight papers (10 groups) including 174,442 SLE patients and 45,608,723 controls with heart failure (HF), 20 papers including 1,121 SLE patients and 1,010 controls with an evaluated LV ejection fraction (LVEF), and eight studies (nine groups) including 462 SLE patients and 356 controls with a measured LV global longitudinal strain (LVGLS) met the predefined inclusion criteria. HF rate in SLE patients was 2.39% (4,176 of 174,442 patients with HF), and SLE patients showed a 3.4 times higher risk for HF compared to controls. SLE patients had a lower LVEF compared to controls. LVGLS was more impaired in SLE patients compared to controls, irrespective of two-dimensional or three-dimensional speckle tracking echocardiography., Conclusion: Heart failure rate in SLE patients is high, and SLE patients showed a 3.4 times higher risk in patients with SLE compared to controls. LV systolic function, as measured by LVEF and LVGLS, is significantly affected in SLE patients, and LVGLS potentially represents a new tool for the early assessment of LV function., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2024, Turkish League Against Rheumatism.)

Objectives: This study aimed to evaluate the early effectiveness and safety of belimumab in addition to standard therapy in patients with systemic lupus erythematosus (SLE) for 24 weeks., Patients and Methods: This retrospective study was conducted with 60 adult patients with active SLE between June 2020 and August 2022. The patients either received intravenous belimumab in addition to standard therapy (n=31; 24 females, 7 males; mean age: 33.7±14.1 years; range, 18 to 52 years) or only standard therapy (n=29; 22 females, 7 males; mean age: 34.1±13.4 years; range, 19 to 66 years) for 24 weeks. Outcome measures, including safety and effectiveness (Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Erythematosus Disease Activity Index [SELENA-SLEDAI]), changes in biomarkers (double-stranded DNA [deoxyribonucleic acid]), serum complement levels, and immunoglobin G (IgG) were recorded. Adverse events were recorded., Results: Baseline demographic and clinical characteristics were similar between the two groups. More patients in the belimumab group achieved a reduction of ≥4 points in SELENA-SLEDAI at weeks 12 and 24 (week 12, 77.4% vs. 41.4%, p=0.008; week 24, 87.1% vs. 48.3%, p=0.002). The mean score of SELENA-SLEDAI was significantly lower in the belimumab group compared to the standard therapy group at week 12. However, a significant difference was not reached at week 24. Moreover, mean levels of serum C3 and C4 in the belimumab group were significantly higher than those in the standard therapy group at weeks 12 and 24. A higher proportion of patients in the belimumab group had a normal C3 level than in the standard therapy group. In addition, belimumab treatment resulted in a significant decrease in IgG levels at both weeks 12 and 24. At week 24, the belimumab group had a higher reduction in prednisone dose than the standard therapy group. Furthermore, the percentages of patients with more than 50% reduction in prednisone over baseline were significantly greater for belimumab versus standard therapy at week 12 (p=0.002). The occurrence of adverse events was similar between the two groups (standard therapy group, 44.8%; belimumab group, 51.6%)., Conclusion: Intravenous belimumab was well tolerated and significantly improved disease activity in Chinese patients with SLE at the early stage of treatment. More importantly, belimumab treatment could result in a rapid reduction in prednisone dose as early as week 12., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2024, Turkish League Against Rheumatism.)

Background: Kikuchi-Fujimoto Disease (KFD) is a rare condition, distinguished by its hallmark presentation of regional lymphadenopathy in young adult females. While initially observed to exclusively affect cervical lymph nodes in females under 40 years old, KFD is now known to impact individuals of any age or gender and manifest with adenopathy in various anatomical sites. Nonspecific imaging findings for KFD include enlarged lymph nodes, often exhibiting abnormal morphology., Case Presentation: In this study, we present the case of a 49 year old asymptomatic woman, in whom several enlarged left axillary lymph nodes were incidentally noted during routine mammography. The diagnosis of KFD was determined via ultrasound-guided core needle biopsy. Histological examination of the biopsied lymph node revealed necrotizing lymphadenitis, consistent with KFD., Conclusion: The uncommon and broad presentation of KFD highlights the significance of acquiring tissue samples to distinguish this condition from resembling malignancies or autoimmune disorders.

Background: Pregnancy in women with systemic lupus erythematosus (SLE) is still introduced as a major challenge. Consulting before pregnancy in these patients is essential in order to estimating the risk of undesirable maternal and fetal outcomes by using appropriate information. The purpose of this study was to develop an artificial neural network for prediction of pregnancy outcomes including spontaneous abortion and live birth in SLE. Methods: In a retrospective study, forty-five variables were identified as effective factors for prediction of pregnancy outcomes in systemic lupus erythematosus. Data of 104 pregnancies in women with systemic lupus erythematosus in Shariati Hospital and 45 pregnancies in a private specialized center in Tehran from 1982 to 2014 in August and September, 2014 were collected and analyzed. For feature selection, information of the 149 pregnancies was analyzed with a binary logistic regression model in SPSS software, version 20 (SPSS, Inc., Chicago, IL, USA). These selected variables were used for inputs of neural networks in MATLAB software, version R2013b (MathWorks Inc., Natick, MA, USA). A Multi-Layer Perceptron (MLP) network with scaled conjugate gradient (trainscg) back propagation learning algorithm has been designed and evaluated for this purpose. We used confusion matrix for evaluation. The accuracy, sensitivity and specificity were calculated from the confusion matrix. Results: Twelve features with P

Background: Cytotoxic lymphocyte antigen-4 (CTLA-4) plays an important role in regulating T cell activation. CTLA-4 gene polymorphisms are related with genetic susceptibility to various autoimmune diseases, including systemic lupus erythematosus (SLE). We analyzed the role of CTLA-4 polymorphisms at positions -318CT in patients who suffer from SLE. Methods: This study was performed on 180 SLE patients referred to 5th Azar University Hospital in Gorgan, Iran. Three hundred and four ethnically-and age-matched healthy controls with no history of autoimmune diseases entered the study between 5th May 2008 and 23rd October 2009. DNA was extracted from blood samples according to the standard procedure. Polymerase chain reaction- restriction fragments length polymorphism (PCR-RFLP) was used to analyze the genotype and allele frequencies of this polymorphism. PCR was carried out using the following primers: forward 5′-AAATGAATTGGACTGGATGGT-3′ and reverse 5′-TTACGAGAAAGGAAGCCGT G-3′. The frequency of alleles and genotypes were assessed using direct counting. Chi-square test and Fisher’s exact test were used to compare the association between the alleles and genotype frequencies and SLE. P

Fever of unknown origin is defined as a clinical spectrum of patients who consulted for presenting fever as a single or predominant manifestation, greater or equal to 38.3°C, more than 3 weeks of evolution, and in whom clinical evaluation results and initial paraclinical studies have not identified its cause. Five categories group the causes of fever of unknown origin which include infectious, neoplastic, inflammatory noninfectious, miscellaneous and of unknown origin. Systemic lupus erythematosus is an autoimmune disease that affects connective tissue and has a wide range of clinical manifestations, among which the presence of polyarthritis, mucocutaneous ulcers and malar erythema stand out at the beginning; systemic lupus erythematosus is one of the non-infectious inflammatory causes of fever of unknown origin. A clinical case report is made, of a 51-year-old woman, who consulted to Internal Medicine, with a clinical picture of 30 days of evolution, with intermittent fever, daily and quantified between 38.5 and 39°C, associated with significant weight loss; to which it was diagnosed by clinical and paraclinical findings systemic lupus erythematosus, treated with hydroxychloroquine and prednisolone, achieving disappearance of the fever and improvement of the general state of the patient. [ABSTRACT FROM AUTHOR]

Ilknur Erdem,1 Senay Elbasan Omar,1 Ridvan Kara Ali,1 Hayati Gunes,2 Aynur Eren Topkaya2 1Department of Infectious Diseases, 2Department of Medical Microbiology, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey Objective: Infections are among the most important causes of morbidity and mortality in patients with systemic lupus erythematosus (SLE) but are rare initial presentation of the disease. Therefore, in this study, we describe a case of Streptococcus pneumoniae sepsis in a young woman with previously undiagnosed SLE. Case report: A 23-year-old female patient was admitted to our outpatient clinic complaining of high fever (40°C), chills, fatigue, generalized myalgia, and cough with brown sputum for 5 days. Blood cultures grew gram-positive coccus defined as S. pneumoniae using standard procedures. Antinuclear antibody was positive at a titer of 1/1,000, and anti-double-stranded DNA was positive at 984 IU/mL. She was diagnosed with SLE. Her respiratory symptoms and pleural effusion were considered to be due to pulmonary manifestation of SLE. Conclusion: The underlying immunosuppression caused by SLE could have predisposed the patient to invasive pneumococcal disease. It may also occur as a primary presenting feature, although a rare condition. Keywords: Streptococcus pneumoniae, sepsis, systemic lupus erythematosus

Pregnancy in patients with lupus nephritis is a challenging clinical situation. Although not absolutely contraindicated, it is associated with increased risk for foetal and maternal complications, including foetal loss, preterm delivery, intrauterine growth retardation, hypertension, pre-eclampsia, nephritis flare, and, rarely, maternal death. The complication rate is further increased in the presence of antiphospholipid antibodies or the antiphospholipid syndrome. Proliferative classes of nephritis (III and IV) also appear to confer excess risk for complications. Immunosuppressives such as cyclophosphamide and mycophenolate, and antihypertensives such as angiotensin-converting-enzyme (ACE) inhibitors and angiotensin receptor blockers need to be stopped due to teratogenic effects. Agents like corticosteroids, azathioprine, and probably calcineurin inhibitors are considered compatible with gestation. Lupus activity needs to be assessed and carefully monitored. Thrombotic risk due to antiphospholipid antibodies, thrombotic events, or nephrosis needs to be evaluated and managed accordingly, with the use of aspirin and/or unfractioned or low molecular weight heparin. Differentiating between severe pre-eclampsia and lupus nephritis flare might require a renal biopsy, which might not always be feasible, for example after the 32nd gestational week or in a setting of uncontrolled hypertension or thrombocytopaenia. A 6-month history of quiescent disease on non-teratogenic agents seems to be associated with best chance for favourable outcomes. Pregnancy is optimally managed by a multidisciplinary team of experienced specialists, and close monitoring for disease activity during gestation; additionally, follow-up for maternal flare postpartum is also advised.

Objectives: This study aimed to assess the ex vivo impact of Lactobacillus delbrueckii (L. delbrueckii) and Lactobacillus rhamnosus (L. rhamnosus) on inflammatory and anti-inflammatory cytokines as well as their related molecules on the peripheral blood mononuclear cells (PBMCs) of systemic lupus erythematosus (SLE) patients., Patients and Methods: This study was conducted with 20 newly diagnosed SLE patients (18 females, 2 males; mean age: 33.3±12.4 years; range, 18 to 68 years) between September 2017 and September 2018. Extracted PBMCs from each patient were divided into 4 cell groups in our study. Three cell groups act as treatment groups receiving L. rhamnosus (10 7 CFU/mL), L. delbrueckii (10 5 CFU/mL) or a mixture of both, and one group act as our untreated control group in the absence of any probiotic agents. All cell groups were cultured in RPMI 1460 medium for 48 h. Then, total RNA was extracted, and cDNA was synthesized., Results: The gene expression levels of forkhead box P3 (FOXP3), transforming growth factor beta (TGF-β), interleukin (IL)-6, IL-10, and IL-2 were evaluated by a quantitative real-time polymerase chain reaction. The results revealed that expression levels of FOXP3, TGF-β, IL-10, and IL-2 increased and the level of IL-6 decreased in probiotics-receiving groups compared to the control group. Lactobacillus delbrueckii and L. rhamnosus enhanced the expression of regulatory T cell-related molecules such as FOXP3 and IL-2 and also increased the expression of IL-10. These probiotics also reduced the expression of IL-6 as proinflammatory cytokines in the PBMCs of SLE patients., Conclusion: The results of the present study show that these probiotics could be effective in regulating the balance of cytokine gene expression ex vivo , and due to their beneficial effects, they can be an intriguing option in the production of new complement drugs for SLE., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2023, Turkish League Against Rheumatism.)

Objectives: The aim of this study was to investigate the frequencyof pulmonary function abnormalities in clinically asymptomaticchildren with Systemic Lupus Erythematosus and to determinethe relationship of these abnormalities to clinical, laboratory, andimmunological parameters as well as to disease activity.Methods: Forty-two children with childhood onset SystemicLupus Erythematosus were included in this study. Demographic,clinical, laboratory and immunological parameters, as well asdisease activity were assessed. Pulmonary function tests (PFT)were performed routinely to screen for subclinical lung disease.Results: Out of the 42 children, 19�0(n=8) had clinical evidence ofpulmonary involvement. The patients with no clinical evidence ofpulmonary involvement (n=34) represent the study cohort. Fromour cohort of patients with no clinical evidence of pulmonaryinvolvement 79�0(n=27) had PFT abnormality; including 62�=21) had reduced FVC, 71�0(n=24) had reduced FEV1, and67�0(n=12) had reduced DLCO. Similarly, 56�0(n=15) hada restrictive PFT pattern, and 2.6�0(n=2) had an obstructivePFT pattern, while 33�0(n=7) had an isolated impairment ofdiffusion capacity. Due to small sample size; it was not possibleto find a statistically significant difference between the cohort ofasymptomatic SLE patients with abnormal PFT findings (n=27)and those with normal PFT findings (n=7) in terms of clinical,laboratory, immunological or disease activity index score.Conclusion: Subclinical lung disease, as demonstrated byabnormal PFT in patients with normal radiographs, may becommon but should be interpreted with caution as an early signof lung disease. Although PFT studies do not correlate well withpulmonary symptoms in patients with childhood onset SLE,they nevertheless provide objective quantification of the type andseverity of the functional lesions.

Objective: To determine the relationship between demographic variables and the level of disease awareness among patients of Systemic lupus erythematosus (SLE). Study Design: Cross-sectional study Place and Duration of Study: Fauji Foundation Hospital, Rawalpindi Pakistan, from Mar 2019 to Jan 2020. Methodology: The study included 150 patients, diagnosed with systemic lupus erythematosus according to SLICC criteria. A self-made questionnaire was used, including three sets of questions. The first set collected information on socio-demographic characteristics, and the second set gathered information regarding the symptoms and treatment. While, the third was based on aetiology, aspects of life affected and sources of disease knowledge. Results: A total of 150 participants' data was analysed; the majority of those were females. The mean age was 36.19±12.08 years and 17.3% of the patients had adequate knowledge of the disease. There were no statistically significant differences i n awareness based on gender, education level and geographical distribution. Conclusion: SLE is a two-pronged sword that affects patients' mental and physical health. Patient awareness remains an important aspect of disease management which is currently not emphasized appropriately. At the community and national levels, measures must be taken to promote and create awareness regarding SLE. [ABSTRACT FROM AUTHOR]

Objectives: This study investigated the correlation between serum and urinary B cell-activating factor (BAFF) levels and systemic lupus erythematosus (SLE) disease activity., Patients and Methods: This case-control study was conducted with 87 participants between December 2020 and September 2021. Sixty-two SLE patients who fulfilled the eligibility criteria were enrolled. SLE patients were categorized into active (n=34) and inactive (n=28) groups based on their Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores. The control group consisted of 25 healthy subjects. Serum and urine samples were collected for the measurement of BAFF levels. Finally, the relationship between these variables and SLE disease activity was investigated., Results: The mean age of active (SLEDAI-2K >4) and inactive (SLEDAI-2K ≤4) SLE patients and healthy individuals were 32.8±7.8, 32.5±6.8, and 31.7±7.8 years, respectively (p=0.62). The median serum BAFF (s-BAFF) and urinary BAFF (u-BAFF) in active lupus patients (10.4 [2.3] ng/mL and 8.2 [3.7] ng/mL, respectively) were significantly higher than in inactive lupus patients (6 (7.1) ng/mL and 1.7 (4.7) ng/mL, respectively; p<0.001) and the control group (3 (3.7) ng/mL and 1.6 (2.2) ng/mL, respectively; p<0.001). However, s-BAFF (p=0.07) and u-BAFF (p=0.43) did not significantly differ between the inactive group and the control group. A significant positive correlation was observed between s-BAFF (r=0.41 and p=0.001) and u-BAFF (r=0.78 and p<0.001) levels and the SLEDAI-2K score., Conclusion: There is a significant positive correlation between serum and urinary BAFF levels and SLE disease activity. Furthermore, significantly higher levels of s-BAFF and u-BAFF have been observed in patients with active lupus compared to inactive and healthy subjects, indicating a possible role for BAFF in the pathogenesis of SLE disease activity., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2023, Turkish League Against Rheumatism.)

Background: Systemic lupus erythematosus (SLE) is currently the most prevalent autoimmune disorder worldwide. A previous study reported the frequency of sarcopenia in patients with chronic inflammation and found a higher rate of sarcopenia in patients with SLE than in control. A preview study found that exercises management given to SLE patients can reduce fatigue and increase vitality., Objective: The objective of this study is to understand the relationship between sarcopenia and SLE from Physical Medicine and Rehabilitation (PM&R) standpoint and its intervention., Methods: Using the PubMed computer-aided search engine specific keywords: "sarcopenia" AND "Systemic lupus erythematosus" OR "lupus" OR "SLE" OR "physical medicine and rehabilitation" OR "rehabilitation" OR "physical therapy" OR "exercises" OR "physical activity" OR "training" OR "nutrition" OR "OR "diet.", Results: Exercise rehabilitation can increase energy level, cardiovascular fitness, functional status, and physical capabilities of muscle strength and are safe to be performed by patients with SLE. Resistance training has been shown to improve muscle strength and size, increase mitochondrial content, and reduce oxidative stress. Resistance exercise impacts sarcopenia through several mechanisms in the muscular and neuromotor levels. Aerobic exercises are also beneficial for skeletal muscles to increase mitochondrial bioenergy, improve insulin sensitivity, and reduce oxidative stresses. Nutritional interventions such as protein, amino acids, essential fatty acids, and vitamin D produce biological effects that will enhance the physiological adaptation of exercise., Conclusion: Intervention for maintaining muscle function in the prevention and management of sarcopenia in SLE and its complications is a combination of resistance training and nutritional intake through adequate protein intake., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Background: Platelet-activating factor (PAF) seems to be implicated in systemic lupus erythematosus (SLE) patients with associated renal diseases.Aims: In this study, we ensured the role of PAF in SLE patients without renal complications.

ABSTRACT Objectives: To evaluate the clinical features, outcome and poor prognostic factors of COVID-19 in rheumatic disease patients. Study Design: Cross-sectional study. Place and duration of Study: Department of Rheumatology, Fauji Foundation Hospital, Rawalpindi, from Mar to Sep 2020. Methodology: The study included rheumatic disease patients with COVID-19. Patients' age, gender, smoking status; details of rheumatic disorder; method of COVID-19 diagnosis, treatment and outcomes were recorded. Results: The study included 46 patients. Overall mortality rate was 23.8%. The most common symptoms were fever (35, 83.3%), cough (26, 61.9%) and myalgia (23, 54.8%). Dyspnea was associated with mortality (p=0.013), ICU admission (p<0.001), ventilation (p=0.02) and hospitalization (p<0.001). NSAIDs increased the risk of ventilatory support (p= 0.02). Long term steroids predicted mortality (p=0.02), hospitalization (p=0.014) and intensive care admission (p=0.004). Steroid and Hydroxychloroquine treatment for COVID-19 was associated with intensive care admission (p= 0.001 and 0.006, respectively) and ventilation (p=0.007 and 0.03, respectively). Mycophenolate Mofetil was related to intensive care admission, ventilation and hospitalization (p=0.03, 0.03 and 0.02, respectively), whereas Cyclophosphamide was related to hospitalization (p= 0.03). Systemic lupus erythematosus was associated with all poor outcomes except ventilation (p<0.05) Conclusion: Systemic lupus erythematosus, long-term steroids, Mycophenolate Mofetil, Cyclophosphamide and Dyspnea are associated with severe COVID-19. [ABSTRACT FROM AUTHOR]

Bruton tyrosine kinase (BTK) is an important protein of the tyrosine kinase family and plays a key role in signal transduction, proliferation, migration, and survival in B lymphocytes. The inhibition of BTK is a promising therapy for various autoimmune diseases (AD) involving abnormal B cell function, such as rheumatoid arthritis (RA), multiple sclerosis (MS), and systemic lupus erythematosus (SLE). This article briefly summarizes the role of BTK in the BCR signaling pathway, the development process of BTK inhibitors, and especially the latest progress of their clinical trials for the treatment of AD., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Objectives: The study aimed to examine the frequency, causes, and predictors of mortality in a cohort of Egyptian systemic lupus erythematosus (SLE) patients and compare mortality causes and the survival rate in our cohort to African, Arabic, and Mediterranean studies., Patients and Methods: In this retrospective study, a review of medical records of 563 SLE patients (516 females, 47 males; median of age: 32 [IQR: 26-38 years]; range, 14 to 63 years) fulfilling the 1997 American College of Rheumatology (ACR) criteria between January 2015 and December 2019 was done. The data extracted included demographic, clinical, and laboratory features, treatments used, disease activity as measured by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and damage index as measured by Systemic Lupus International Collaborating Clinics (SLICC) damage index. Causes of mortality were also reported., Results: Out of 563 reviewed medical records, 50 (8.9%) patients died. Infection (28%) and organ damage (18%) were the most commonly reported causes of death. Multivariate Cox regression analysis showed that patients with cardiac manifestations, renal failure, those receiving higher doses of either oral (in their last visit) or intravenous (higher cumulative pulse steroids) steroids were at increased risk of mortality (p=0.011, p<0.001, p=0.01, and p<0.001, respectively; 95% confidence intervals 7.2, 63.9, 1.2, and 1.09, respectively). The overall survival at 5, 10, 15, and 20 years was 96.6%, 93.3%, 91.0%, and 83.2%, respectively, and 56.2% at 25 years until the end of the follow-up., Conclusion: Cardiac manifestations, renal failure, and higher steroid doses were independent predictors of mortality in our cohort. As in most African countries, infection was the main cause of death in our study; however, the mortality rate and the five-year survival among our cohort were better than in African (sub-Saharan) countries and similar to Arabic and Mediterranean countries., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2023, Turkish League Against Rheumatism.)

Objectives: This study aims to analyze the relationship between complement receptor 2 (CR2) gene mutation and the clinical phenotype in Chinese familial systemic lupus erythematosus (SLE)., Patients and Methods: A total of one Chinese familial SLE patients (median age: 30.25 years; range, 22 to 49 years) were included between January 2017 and December 2018. The clinical features and diagnoses of familial SLE patients were analyzed using whole-exome sequencing (WES) of genomic deoxyribonucleic acid (DNA) samples. Sanger sequencing was used to verify candidate mutations detected in the examined family., Results: The mother and her three daughters were diagnosed with SLE. The clinical characteristics showed that the patient and her mother were diagnosed with lupus nephritis. The eldest daughter had decreased renal function and lower serum albumin levels. Immunological index analysis showed that all four patients were positive for anti-SSA and antinuclear antibody (ANA), but that only the second daughter was positive for anti-double-stranded DNA (dsDNA). Complement 3 (C3) was significantly decreased in all patients, while evaluation of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) showed that the second and third daughters had mild active SLE. The mother and eldest daughter were treated with prednisolone combined with cyclophosphamide, while the other two daughters were treated with prednisolone alone. The WES and Sanger sequencing analyses revealed an unreported missense T>C mutation c.2804 in the 15 th exon of the CR gene in all four patients., Conclusion: We identified a novel c.2804 (exon 15) T>C mutation in the CR gene of Chinese familial SLE. This mutation was previously reported, suggesting that the CR gene c.2804 (exon 15) T>C mutation is the probable cause of SLE in this family., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2022, Turkish League Against Rheumatism.)

Objectives: This study aims to the prevalence of antinuclear antibody (ANA)-negative systemic lupus erythematosus (SLE) and their clinical characteristics in a large single-center SLE inception cohort to provide guidance for early diagnosis., Patients and Methods: Between December 2012 and March 2021, the medical records of a total of 617 firstly diagnosed SLE patients (83 males, 534 females; median age [IQR]: 33+22.46 years) who fulfilled the selection criteria were retrospectively analyzed. The patients were divided into groups with ANA-negative SLE and ANA-positive SLE, or with prolonged use of glucocorticoids or immunosuppressants (SLE-1) and without (SLE-0). Demographic, clinical characteristics, and laboratory features were collected., Results: The total prevalence of ANA-negative SLE patients was 2.11% (13/617). The prevalence of ANA-negative SLE in SLE-1 (7.46%) was significantly higher than that in SLE-0 (1.48%) (p<0.01). The ANA-negative SLE patients had a higher prevalence of thrombocytopenia (84.62%) than ANA-positive SLE patients (34.27%). As with ANA-positive SLE, ANA-negative SLE also had a high prevalence of low complement (92.31%) and anti-double-stranded deoxyribonucleic acid (anti-dsDNA) positivity (69.23%). The prevalence of medium-high titer anti-cardiolipin antibody (aCL) IgG (50.00%) and anti-ß2 glycoprotein I (anti-ß2GPI) (50.00%) of ANA-negative SLE was significantly higher than that of ANA-positive SLE (11.22% and 14.93%, respectively)., Conclusion: The prevalence of ANA-negative SLE is very low, but it exists, particularly under the influence of prolonged use of glucocorticoids or immunosuppressants. Thrombocytopenia, low complement, positive anti-dsDNA, and medium-high titer antiphospholipid antibody (aPL) are the main manifestations of ANA-negative SLE. It is necessary to identify complement, anti-dsDNA, and aPL in ANA-negative patients with rheumatic symptoms, particularly thrombocytopenia., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2022, Turkish League Against Rheumatism.)

Objectives: In this study, we aimed to better understand the expression of pro-apoptotic Bad and Bax in the pathogenesis of systemic lupus erythematosus (SLE) and their relationship with the disease activity., Patients and Methods: Between June 2019 and January 2021, a total of 60 female patients with SLE (median age 29 years; IQR, 25.0-32.0) and 60 age- and sex-matched healthy female controls (median age: 30 years; IQR, 24.0-32.0) were included. The Bax and Bad messenger ribonucleic acid (mRNA) expression was measured by real-time polymerase chain reaction., Results: The expression of Bax and Bad was significantly lower in SLE group than the control group. The median value of mRNA expression of Bax and Bad was 0.72 and 0.84, respectively versus 0.76 and 0.89 in the control group. The median value of (Bax*Bad)/β-actin index was 17.8 in the SLE group and 19.64 in the control group. The expression of both Bax, Bad and (Bax*Bad)/β-actin index had a good significant diagnostic utility (area under the curve [AUC]= 0.64, 0.70, and 0.65, respectively). The Bax mRNA expression showed a significant upregulation with disease flare-up. The efficacy of Bax mRNA expression in predicting SLE flare-up was good (AUC= 73%). In the regression model, the probability of flare-up reached 100%, with increasing Bax/β-actin as well, and the likelihood of flare-up increased 10,314 times with every unit increase of Bax/β-actin mRNA expression., Conclusion: Deregulation of the mRNA expression of Bax may have a role in the susceptibility to SLE and may be associated with disease flare. A better understanding of the expression of these pro-apoptotic molecules may carry a great potential for the development of specific effective therapies., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2023, Turkish League Against Rheumatism.)

Loss of B-cell tolerance is a hallmark feature of the pathogenesis in systemic lupus erythematosus (SLE), an autoimmune disease that is characterized by hypergammaglobulinemia and autoantibody production. These autoantibodies lead to formation of immune-complex deposition in internal organs causing inflammation and damage. Autoreactive B-cells are believed to be central in the pathophysiology of SLE. Other than its role in the production of antibodies that mediate humoral immune response, B-cells also function as antigen-presenting cells and are capable of activating T-cells. Activated B-cells may also produce pro-inflammatory cytokines that aggravate local inflammation. Abnormal B-cell homeostasis has been described in SLE patients. This may occur as a result of intrinsic B-cell defect or from aberrant regulation by maturation and survival signals. B-cell-based therapy is the current mainstream of research and development of novel therapies in SLE patients with severe and refractory disease. Potential cellular and molecular targets for B-cell therapies include cell surface molecules such as CD20 (rituximab) and CD22 (epratuzumab); co-stimulatory molecules involved in B-cell–T-cell interaction such as CTLA4 and B7 molecules (abatacept); maturation and growth factors such as B-cell activating factor and a proliferation-inducing ligand (belimumab, briobacept, atacicept) and B-cell tolerogen (abetimus). This article provides an overview on normal B-cell physiology and abnormal B-cell biology in SLE that form the immunological basis of B-cell-targeted therapy in the treatment of these patients with refractory diseases. [ABSTRACT FROM AUTHOR]

Nervous system involvement in systemic lupus erythematosus (SLE) is typically diagnosed on the basis of clinical psychiatric and/or neurologic syndromes (NPSLE). Neuropsychological tests can be used to assess nervous system integrity even in the absence of major NP syndromes. Their application has uncovered significant cognitive dysfunction, ranging from mild to severe, in a sizeable proportion of SLE patients irrespective of clinical NP status. Cognitive dysfunction has now been accepted as a bona fide manifestation of NPSLE. The heterogeneity of clinical NPSLE manifestations is paralleled by the diversity of cognitive deficits reported in different studies and within different patients. The success of attempts to explain these deficits on the basis of potential pathogenetic mechanisms, such as antibrain antibodies and proinflammatory cytokines, has been uneven. To date, the most robust findings have emerged in relation to antiphospholipid antibodies, which carry with them important therapeutic implications. [ABSTRACT FROM AUTHOR]

Objectives: This study aims to investigate the expression patterns of mitochondrially encoded cytochrome c oxidase 1 (MT-CO1) in different organs and tissues of MRL/lpr mice aged six and 18 weeks., Materials and Methods: Six-week-old female MRL/lpr mice (n=10) were considered young lupus model mice, and 18-week-old MRL/lpr mice (n=10) were considered old lupus model mice. Additionally, six-week-old (n=10) and 39-week-old (n=10) female Balb/c mice were used as the young and old controls, respectively. The messenger ribonucleic acid (mRNA) and protein expression levels of MT-CO1 in nine organs/tissues were detected via quantitative polymerase chain reaction (qPCR) and Western blot. Malondialdehyde (MDA) levels were determined with thiobarbituric acid colorimetry. The correlation coefficient of MT-CO1 mRNA levels and MDA levels in each organ/tissue at different ages was analyzed by Pearson correlation analysis., Results: The results showed that most non-immune organs/tissues (heart, lung, liver, kidneys, and intestines) showed increased MT-CO1 expression levels in younger MRL/lpr mice (p<0.05) and decreased MT-CO1 expression in older mice (p<0.05). Expression of MT-CO1 in the lymph nodes was low in younger mice but high in older mice. In other immune organs (spleen and thymus), MT-CO1 expression was low in older MRL/lpr mice. Lower mRNA expression and higher MDA levels were observed in the brains of MRL/lpr mice. However, all MRL/lpr mice showed higher MDA levels than Balb/c mice in every organ no matter younger or older MRL/lpr mice., Conclusion: Our study results suggest that lymphoid mitochondrial hyperfunction at organ level may be an important intrinsic pathogenesis in systemic lupus erythematosus activity, which may affect mitochondrial dysfunction in non-immune organs., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2022, Turkish League Against Rheumatism.)

A distinct annular erythema developed on the cheeks of three East Asian women who had anti-SS-A/Ro (SSA) antibodies. The erythema was characterized by a wide, elevated border and central pallor. Histologically, there was a coat-sleeve-like infiltration of lymphocytes around the blood vessels, appendages, and secretory gland cells in the dermis. Immunohistological analysis clarified that the majority of infiltrating lymphocytes were CD4-positive T cells. Abnormal expression of HLA-DR antigens in the perivascular, appendage, and secretory gland cells in the dermis was also observed. The differential diagnosis of the three patients lay between Sjögren syndrome (SjS), Sjögren/systemic lupus erythematosus overlap syndrome and an asymptomatic clinical state. These results are consistent with recent findings of major histocompatibility complex class II expression on target organs in various autoimmune diseases. Based on these findings, erythema appears to represent a broad cutaneous manifestation of these diseases. Furthermore, the presence of SSA antibodies, aberrant HLA-DR expression, and sun exposure may be responsible for the development of erythema. [ABSTRACT FROM AUTHOR]

CD28 on T cells provides a potent costimulatory signal for T cell activation. Down-regulation of CD28 on peripheral T cells has been reported in certain clinical conditions, but full studies on the mechanism and biological significance have not been performed. Our extensive phenotype analysis of peripheral blood lymphocytes (PBL) from SLE patients revealed that the absolute number of CD28+ T cells of both CD4 and CD8 phenotypes was selectively decreased, while that of CD28- T cells was maintained. CD28+ T cells from SLE patients exhibited mostly normal proliferative responses to both CD28-dependent and -independent stimulations. In contrast, CD28- T cells were hyporesponsive to anti-CD3 stimulation in both SLE and normal controls. These results implied that the selective decrease of CD28+ T cells in SLE does not result from a hyporesponsiveness of CD28+ T cells. To investigate the reason for the selective loss of CD28+ T cells, we determined the appearance of apoptotic cells in culture with or without anti-CD3 stimulation. Apoptotic cells defined by merocyanine(MC)540 were gradually increased from 12h to 24h. Anti-CD3-induced apoptosis of CD28+ T cells was significantly accelerated in SLE, whereas apoptosis of CD28- T cells was hardly detected in both SLE and normal controls. Comparative analysis between CD28+ and CD28- T cells on CD95 (Fas) and Bcl-2 expression, which are related to activation-induced cell death (AICD), did not show a major difference, although CTLA4, which has been demonstrated to transmit an apoptosis-inducing signal, was expressed only on CD28+ T cells. Our results suggest that CD28-mediated costimulation influences T cell susceptibility to AICD and may be involved in T cell lymphopenia in SLE. [ABSTRACT FROM AUTHOR]

The availability of MoAbs now allows the accurate quantification of the individual C4 isotypes. C4A and C4B. Using a sensitive two-site immunoradiometric technique to measure serum levels of C4A and C4B, we studied the relationship between genotype and phenotype and physiological factors affecting C4 expression in 129 fully genotyped healthy subjects. Our results confirm that there is extensive phenotypic overlap between genotypic groups and it was not possible to determine the presence of single null alleles from total serum C4. Of the factors which may influence C4 expression, we found that age contributes a very small influence but that gender has no effect and there was no evidence for the presence of feedback of null alleles on the expression of remaining genes. Potential problems in quantifying C4 arising from the complex relationship between isotypic identity and serotypic recognition were highlighted by the finding of reversed antigenic expression of a C4B*5 molecule which was recognized as C4A by the anti-Rg:1 monoclonal used in these studies. We also confirmed that the extended MHC haplotype associated with Felty's syndrome, HLA-B44, C4A*3, C4BQ*O, HLA-DR4, encodes an expressed, duplicated. C4A gene. [ABSTRACT FROM AUTHOR]

B lymphocyte studies, in vivo and in vitro, were performed in 28 patients with systemic lupus erythematosus (SLE). After immunization with the primary test antigen haemocyanin, a decreased antigen specific humoral immune response was observed for all three Ig classes, irrespective of disease activity or the use of corticosteroids. Levels of antibodies against (recall) viral and nuclear antigens were increased during active-disease. The in vitro spontaneous production of IgM and IgG, determined in the supernatant of 8 days cultures by ELISA, was highly increased in patients with active disease (reversely correlated with levels of complement Cr,r=0.74). Pokeweed mitogen-induced synthesis was decreased in all patients. The distribution of T cell subsets (OKT3, Leu 3a and OKT8 positive cells) was not different form controls, irrespective of disease activity. It is concluded that the primary humoral immune response is decreased in SLE, whereas a polyclonally activated B cell appears to be present. The normal T cell subset distribution does not support and primary role of the cell in the state of hyperactivity of the B cell. [ABSTRACT FROM AUTHOR]

Various allergic diseases have been described in patients with systemic lupus erythematosus (SLE). In the present study, we evaluated the prevalence of allergic disease and serum IgE levels in 36 children of 26 mothers with SLE. None of the subjects had any rheumatic symptoms. There was at least one type of allergic disease in 28 (78%) of the 36 children of mothers with SLE, as compared with 30% in Japanese control children (P< 0.01). The prevalence of atopic dermatitis and bronchial asthma was higher in children of mothers with SLE (64% and 28%) than in controls (19% and 9%) (P< 0.01, respectively). Fourteen of the 36 subjects (39%) had higher levels of serum IgE than those of normal range for age-matched healthy Japanese children with no atopic family disease in the immediate family history, and these children had atopic disease. Among the 14 children with high serum-IgE levels, seven had neither immediate nor remote family history of atopic disease, while the others had an immediate family history of atopic disease. We think that genetic factors may influence the presence of allergic disease in children of mothers with SLE, and that the increased serum IgE levels in children of SLE mothers with no allergic family history may be a part of subclinical immunologic abnormalities related to SLE. [ABSTRACT FROM AUTHOR]

Summary Expression of the human erythrocyte C3b receptor (CR1-CD35) and its Hind III Rap was studied in a group of 37 patients with SLE, 15 consanguineous relatives of the patients and 48 healthy normal subjects. The CR1 number on erytbrocytes was quantitated by ELISA using a mAb to CR1. Serum levels of complement proteins (C3, C4, C3d) and circulating immune complexes (CIC) were estimated simultaneously in controls and relatives. The patients were followed up during the course of the treatment. The CR1/ erythrocyte (CR1/E) in patients were found to be significantly low in comparison to controls. The gene frequencies for the alleles H and L (7.4 and 6.9 kb Hind Ill restriction fragments) in the patients were 0.75 and 0.25, respectively, which did not differ significantly from the controls (0.77 and 023 in normal subjects and 0.79 and 0.21 in consanguineous relatives of the patients). However, patients expressed fewer CR1/E within each genotype than their relatives and healthy subjects. CR1/E was found to be stable in consecutive samples in controls. In patients, the numbers varied between low and high during the course of the treatment. The variation in the numbers was significantly correlated with C3d and CIC as well as with the severity of the disease. Our results suggest that low levels of CR1 on erythrocytes in SLE patients are acquired during the course of the disease and that the 6.9 kb restriction fragment does not play a role in causing susceptibility to the disease. [ABSTRACT FROM AUTHOR]

Objectives: 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)., Patients and Methods: 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., Results: 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)., Conclusion: Pregnancy outcomes in SLE patients who achieved modified clinical remission and modified LLDAS were comparable., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2022, Turkish League Against Rheumatism.)

Incidence of ANCA antibodies in patients with systemic lupus erythematosus (SLE) is described in 24-31 %, but they are not related to the distribution and severity of organ involvement in SLE; the routine monitoring is not recommended. Overlap syndrome of systemic lupus erythematosus and ANCA associated vasculitis (AAV) is rare. The difficult diagnosis and treatment of this syndrome is described in this case report of the patient with SLE and severe kidney involvement resulting from AAV.

Objectives: This study aims to estimate predicted factors for maternal and fetal outcomes in Hakka pregnant women with systemic lupus erythematosus (SLE) in the southern China., Patients and Methods: Between June 2014 and February 2020, we retrospectively analyzed the data of a total of 123 singleton pregnant women with SLE (mean age: 27.1±4.1 years; range, 19 to 39 years) who were referred to our rheumatology clinic. Demographic, clinical, and laboratory data of the patients were recorded. Adverse pregnancy outcomes (APOs) were assessed., Results: Multivariate logistic regression analysis revealed that preeclampsia was associated with the increased odds of APOs (odds ratios [OR]=9.538, 95% confidence interval [CI]: 2.055-44.271, p=0.004), premature birth (OR=14.289, 95% CI: 3.596-56.777, p<0.001) and low birth weight (OR=8.275, 95% CI: 2.117-32.345, p=0.002). Anti-double-stranded deoxyribonucleic acid (anti-dsDNA) antibody positivity was the predictor of APOs (OR=2.165, 95% CI: 1.034-4.532, p=0.040), premature birth (OR=2.849, 95% CI: 1.220-6.657, p=0.016) and pregnancy loss (OR=3.004, 95% CI: 1.086-8.305, p=0.034). The use of hydroxychloroquine and prednisone was associated with the decreased odds of APOs (OR=0.412, 95% CI: 0.198-0.860, p=0.018) and pregnancy loss (OR=0.304, 95% CI: 0.111-0.831, p=0.020)., Conclusion: Our study results indicate that preeclampsia, anti-dsDNA antibody positivity, and the use of hydroxychloroquine and prednisone are independent predictors of pregnancy outcomes., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2022, Turkish League Against Rheumatism.)

Hereditary deficiency of complement component C1q is a rare genetic disorder with susceptibility to recurrent infections with polysaccharide-containing encapsulated microorganisms and a high prevalence of autoimmune diseases, most often systemic lupus erythematosus (SLE). Here, we report a 29- month-old boy who presented with facial rash and history of early death of a sibling with infections, who was found to have a selective deficiency of C1q. The facial rash was composed of patchy erythematous plaques and centrally hypopigmented macules and desquamation. Two siblings had died of severe bacterial infections and his uncle had died of meningitis. Molecular study disclosed a homozygous point mutation in the C1qA chain gene. Five members of the family, including the parents and three healthy siblings, were heterozygous for this mutation. [ABSTRACT FROM AUTHOR]

Background: Lupus nephritis (LN) represents 40%-50% of all systemic lupus erythematosus (SLE) patients, and rapidly progressive glomerulonephritis is associated with significant morbidity and mortality. Antineutrophil cytoplasmic antibody (ANCA) might be involved in the pathogenesis of LN., Objective: We evaluated the role of myeloperoxidase (MPO)-ANCA, proteinase 3 (PR3)-ANCA, and anti-glomerular basement membrane autoantibodies (anti-GBM autoAb) for the diagnosis of LN., Methods: In this cross-sectional study, 95 SLE patients were divided into 2 subgroups: LN group (n = 60) and non-LN group (n = 35). For further analysis, we subclassified the LN group into ANCA- positive (n = 16) and ANCA-negative (n = 44) LN patients. The entire Non-LN group was ANCA- negative. The SLE disease activity index (SLEDAI) was reported for each patient. Determination of MPO-ANCA, PR3-ANCA, and anti-GBM autoAb was performed using a novel multiplex bead-based technology in all patients. Data analyses were done using SPSS, version 20. Approval was obtained from the institutional review board of Zagazig University (ZU-IRB#6000)., Results: Of 95 patients with SLE, 16 patients (16.84%) had ANCA-positive LN, all of which were MPO-ANCA. There was a positive correlation between MPO-ANCA and SLEDAI, as well as with class IV LN. Receiver operating characteristic analyses revealed that the sensitivity and specificity of MPO-ANCA were 81.3% and 99.8%, respectively, in discriminating LN from systemic lupus without nephritis., Conclusion: MPO-ANCA level was significantly correlated with SLEDAI, inflammatory markers, kidney function tests, and LN class IV., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Background/aim: Avascular necrosis (AVN) is the death of bone due to compromise of blood flow. The etiology of AVN is multifactorial; corticosteroid usage is the second most significant factor after trauma, and systemic lupus erythematosus (SLE) is the most common underlying disease. The objective of this study was to assess the factors of AVN in SLE patients., Materials and Methods: The study included 127 patients with SLE who fulfilled 1997 American College of Rheumatology (ACR) revised criteria. Demographic data, age at SLE diagnosis, disease duration, disease activity, body mass index, clinical findings, antiphospholipid syndrome, steroid usage, dose and duration, comorbid diseases, and smoking history were recorded., Results: AVN was found in 11 of 127 (8.7%) SLE patients. Hyperlipidemia (P < 0.001), cushingoid body habitus (P < 0.001), and proteinuria (P = 0.013) were found at higher rates in the AVN group. All of the 11 AVN cases had osteoporosis (P < 0.02). In multivariate regression analysis, daily steroid usage was the only factor for development of AVN in SLE., Conclusion: The hypothesis of our study was that an alternate day steroid regimen may decrease AVN frequency in SLE patients., Competing Interests: None declared, (This work is licensed under a Creative Commons Attribution 4.0 International License.)