Course Of Systemic Lupus Erythematosus Research Articles

Association of neutrophil extracellular trap levels with Raynaud’s phenomenon, glomerulonephritis and disease index score in SLE patients from Brazil

Neutrophil extracellular traps (NETs) are cell-extruded DNA strands coated with neutrophils' nuclear proteins and enzymes from cytotoxic granules, produced by NETosis, a cell death pathway. They perform an important defensive role in innate immunity, but their increased production and/or inefficient degradation expose new antigens, such as DNA or citrullinated histone peptides, triggering autoimmunity. This study aimed to access possible associations between serum NETs levels with epidemiological, clinical, and serological data from a well-characterized SLE Brazilian patients’ cohort. NET levels were evaluated in one hundred seventy serum samples of patients with Systemic Lupus Erythematosus (SLE) using an Immunoassay. Univariate and multivariate binary logistic regression used clinical patients' data as independent variables. Parametric and non-parametric tests compared log10 base serum NET levels transformed between patients' groups. SLE patients were also dichotomized into “High serum NET levels” and “Low serum NET levels” groups. All analyses were performed in R language 4.1.2, and p < 0.05 were considered significant. Increased susceptibility for high serum NET levels was observed in SLE patients with Raynaud's phenomenon (OR = 2.30, 95 % CI = 1.06–5.21 and p = 0.039), independently of any other risk factor. Also, SLE patients with Raynaud's phenomenon presented higher mean NET serum levels (mean = −0.13 vs. −0.51, p = 0.01). In addition, higher mean NET serum levels were associated with glomerulonephritis (mean = -0.45 vs. −0.12, p = 0.03). Ultimately, the SLEDAI index scored higher in the high NETs serum levels group (median = 2.0 vs. 0.0, p = 6 × 10−3). The formation of NETs might be implicated in Raynaud's phenomenon, glomerulonephritis, and disease index score in SLE patients. Our results highlight the importance of serum NET levels as a possible therapeutical target to modulate the clinical course of SLE.

Clinical and laboratory characteristics of early-onset and delayed-onset lupus nephritis patients: A single-center retrospective study.

Lupus nephritis (LN) manifests systemic lupus erythematosus (SLE) and is characterized by various clinical and laboratory features. This study aimed to comprehensively evaluate the characteristics of LN patients according to the time of LN diagnosis: early-onset (LN diagnosed within one year from SLE diagnosis) vs. delayed-onset (LN diagnosed more than one year after SLE diagnosis). We conducted a retrospective analysis of medical records from all SLE patients treated at the University Hospital in Kraków, Poland, from 2012 to 2022. We collected data on demographic, clinical, and laboratory characteristics, including histological findings, treatment modalities, and disease outcomes. Statistical analyses were performed to identify factors impacting LN development and prognosis. Among 331 LN patients, early-onset was diagnosed in 207 (62.54%) and delayed-onset was documented in 122 cases (36.86%). In 2 (0.6%) LN cases, the time of first kidney manifestation in the SLE course was unknown. Delayed-onset LN had a higher female-to-male ratio and younger age at SLE diagnosis. This group was associated with more severe clinical manifestations. In turn, studied subgroups did not differ in internist comorbidities, kidney histopathology, and family history regarding autoimmune diseases. Delayed-onset LN exhibited a higher frequency of anti-dsDNA, anti-Smith, anti-Ro, anti-RNP, and anti-cardiolipin IgG autoantibodies. During a 14-year follow-up period, 16 patients died. Mortality rate and causes of death were comparable in both analyzed subgroups. More severe clinical manifestations in delayed-onset LN prompt strict monitoring of non-LN SLE patients to diagnose and treat kidney involvement early. Also, recognizing the higher frequency of autoantibodies such as anti-dsDNA or anti-Smith in delayed-onset LN underscores the potential value of autoantibody profiling as a diagnostic and prognostic tool.

Clinical and laboratory assessment of the state of periapical tissues in systemic lupus erythematosus.

Aim: To evaluate changes in clinical and laboratory parameters in patients with SLE depending on the severity and activity of the disease. Materials and Methods: The study included 50 patients with SLE of different age groups, 10 men and 40 women. Results: Systemic lupus erythematosus (SLE) is a chronic disease that belongs to the group of rheumatic diseases and is characterised by autoimmune tissue damage. Chronic inflammatory periodontal diseases remain one of the most common dental pathologies. Chronic gingivitis and periodontitis in SLE are described as one of the earliest and most striking symptoms of the disease. Approximately the same trend was found in patients with SLE depending on the degree of clinical and laboratory activity of the disease. Patients with minimal, 1 degree of activity (characterized by the longest duration of SLE) showed the greatest decrease in bone mineral density (up to 2.25 points), and patients with higher activity had a significantly shorter duration of SLE and, accordingly, a smaller decrease in bone mineral density: in patients with 2 degrees of activity (1.79 points), with 3 degrees of activity (1.94 points). Conclusions: In patients with acute SLE, the maximum value of the Muhlemann-Cowell index was 2.31 points, in patients with subacute SLE - 1.89 points, and in patients with chronic SLE - 1.58 points. CPITN values increase inversely with the nature of the course of SLE, which is associated with the duration of the underlying disease.

Ancestry-based differences in the immune phenotype are associated with lupus activity.

Systemic lupus erythematosus (SLE) affects 1 in 537 Black women, which is >2-fold more than White women. Black patients develop the disease at a younger age, have more severe symptoms, and have a greater chance of early mortality. We used a multiomics approach to uncover ancestry-associated immune alterations in patients with SLE and healthy controls that may contribute biologically to disease disparities. Cell composition, signaling, epigenetics, and proteomics were evaluated by mass cytometry; droplet-based single-cell transcriptomics and proteomics; and bead-based multiplex soluble mediator levels in plasma. We observed altered whole blood frequencies and enhanced activity in CD8+ T cells, B cells, monocytes, and DCs in Black patients with more active disease. Epigenetic modifications in CD8+ T cells (H3K27ac) could distinguish disease activity level in Black patients and differentiate Black from White patient samples. TLR3/4/7/8/9-related gene expression was elevated in immune cells from Black patients with SLE, and TLR7/8/9 and IFN-α phospho-signaling and cytokine responses were heightened even in immune cells from healthy Black control patients compared with White individuals. TLR stimulation of healthy immune cells recapitulated the ancestry-associated SLE immunophenotypes. This multiomic resource defines ancestry-associated immune phenotypes that differ between Black and White patients with SLE, which may influence the course and severity of SLE and other diseases.

Clinical and immunologic features of the phenotype of systemic lupus erythematosus combined with Sjögren's syndrome

The problem of clinical and immunological heterogeneity of systemic lupus erythematosus (SLE) is of great interest, especially the combination of SLE and Sjögren's syndrome (SjS) determines a more favorable prognosis and specifics of therapy.Objective: to characterize the clinical and immunological features of SLE combined with SjS.Material and methods. The retrospective study included 44 patients with SLE combined with SjS and 356 patients with SLE without SjS, hospitalized at the V.A. Nasonova Research Institute of Rheumatology between 2013 and 2021. The two groups of patients were compared in terms of life expectancy, clinical manifestations, laboratory parameters and therapy.Results and discussion. The following phenotypic features of the clinical and immunological variant of SLE combined with SjS were found: significantly longer duration of SLE (p&lt;0.01); higher incidence of polyarthritis (p=0.01) and Raynaud's syndrome (p&lt;0.003) at disease onset. Subacute cutaneous lupus erythematosus, Raynaud's syndrome, peripheral nervous system involvement (sensory polyneuropathy and distal sensory-motor polyneuropathy) were found more frequently in SLE combined with SjS than in SLE without SjS, p&lt;0.0001. Among laboratory abnormalities in SLE patients with SjS, leukopenia (p&lt;0.0001), antibodies to SSA/Ro, SSB/La, and rheumatoid factor (p&lt;0.0001) were observed in a greater number of cases. In the group of SLE combined with SjS, the chronic variant of SLE course according to the classification of V.A. Nasonova, and lower activity were observed, which didn't not require therapy with high doses of glucocorticoids; in this group, rituximab (p&lt;0.01), cyclophosphamide and methotrexate were used significantly more often.Conclusion. Thus, significant clinical and laboratory differences were found between SLE with SjS and without SjS. They determine prognosis, approaches to pathogenetic therapy and monitoring.

Mental disorders in patients with systemic lupus erythematosus: association with activity and the course of rheumatic disease

To clarify the relationship between the clinical and psychopathological features of mental disorders, clinical and laboratory manifestations of activity and the nature of the course of systemic lupus erythematosus (SLE). The study included 119 patients - 98 (82.4%) women, mean age 36.5±12.4 years (M±SD) - with a reliable diagnosis of SLE (EULAR/ACR 2019 criteria), 51 (29.5%) of them - with secondary antiphospholipid syndrome - APS (International criteria of 2006). Among patients with SLE a high frequency of anxiety-depressive spectrum disorders (ADSD) and cognitive impairment (CI) was revealed. There was an association of greater severity of depression with high SLE activity index, acute/subacute onset of the disease course according to the classification of V.A. Nasonova, relapsing-remitting and chronic active current disease activity patterns of SLE according to the classification of S. Barr - M. Petri. Anxiety disorders were associated with subacute onset and relapsing-remitting disease activity patterns of SLE and were not associated with SLE activity index. Bipolar disorder was detected more often in patients with chronic SLE. Acute psychosis/delirium was associated with acute onset of SLE. Organic CI was associated with APS, chronic onset and long quiescent disease activity patterns of SLE. The episindrome and schizotypal disorder in patients with SLE are more often caused by concomitant APS. Patients with high SLE activity index should be of particular concern to rheumatologists regarding the diagnosis of depressive disorders. Patients with concomitant APS need timely diagnosis and treatment of CI and episindrome in order to improve the prognosis of the disease and the overall quality of life.

Lupus enteritis: A 10-year experience in a single Latin American center.

The objective is to compare the clinical and laboratory characteristics of systemic lupus erythematosus (SLE) patients with and without lupus enteritis (LE) and to identify the factors associated with the occurrence of LE. We performed a retrospective, case-control study in hospitalized patients with SLE who were admitted to our tertiary hospital between January 2012 and December 2021. Sixteen LE patients (cases) were matched (1:3 ratio) for sex and birth year with 48 non-LE patients (controls). Univariable and multivariable logistic regression analyses were used to identify the variables associated with LE. Of 2,479 SLE patients who were admitted to our hospital as inpatients, 16 (0.65%) were diagnosed as having LE. All patients, cases and controls, were of Mestizo ethnicity. SLE was diagnosed simultaneously with the first episode of LE in 10 (62.5%) patients. The median time from SLE diagnosis to the first episode of LE was 7 (IQR 0-78)months. LE patients had a shorter median disease duration [7 (0-78) vs 34 (9.5-79)months], and a significantly longer hospital stay (28.3 ± 15.8 vs 6.5 ± 7.9days, p < 0.001) than non-LE patients. Most LE patients (93.8%) had concomitant lupus nephritis. LE patients had higher SLEDAI-2K scores than those without LE (20.5 ± 9.4 vs 9.8 ± 10.4, p < 0.001). By multivariable analysis, a higher SLEDAI-2K score (OR 1.10, 95% CI 1.02-1.18; p = 0.015) was independently associated with LE occurrence after adjusting for cutaneous involvement, lymphocyte count, serum creatinine, and serum complement C4. Recurrence was observed in two patients (12.5%), both with a bowel wall thickening > 8mm. The two patients with large intestine-dominant LE developed intestinal pseudo-obstruction. No patient had life-threatening complications (intestinal hemorrhage, infarction, or perforation), and there were no deaths induced directly by LE itself. In patients of Mestizo ethnicity, LE occurs during the early course of SLE, frequently is one of the presenting manifestations of SLE, and in most cases, it presents with concomitant lupus nephritis. Higher levels of disease activity at diagnosis were independently associated with LE occurrence and when recurrences occur, they do so in the context of severe wall thickness.

Changes in Clinical Manifestations and Course of Systemic Lupus Erythematosus and Secondary Antiphospholipid Syndrome over Three Decades.

Systemic lupus erythematosus (SLE) is often associated with antiphospholipid syndrome (APS), which potentially results in a more severe disease course and reduced life expectancy. Since the therapeutic guidelines have been refined in the last 15 years, we assumed that the diseases course has become more favorable. In order to shed light on these achievements, we compared the data of SLE patients diagnosed before and since 2004. In our retrospective study, we assessed a wide spectrum of clinical and laboratory data of 554 SLE patients who received regular follow-up care and therapy at our autoimmune center. Among these patients, 247 had antiphospholipid antibodies (APAs) without clinical signs of APS, and 113 had definitive APS. In the APS group, among patients diagnosed since 2004, deep vein thrombosis (p = 0.049) and lupus anticoagulant positivity (p = 0.045) were more frequent, while acute myocardial infarction was less frequent (p = 0.021) compared with patients diagnosed before 2004. Among the APA positive patients without definitive APS, anti-cardiolipin antibody positivity (p = 0.024) and development of chronic renal failure (p = 0.005) decreased in patients diagnosed since 2004. Our study demonstrates that the disease course has changed in recent years; however, in the presence of APS, we have to expect repeated thrombotic events despite adequate anticoagulant therapy.

Short- and mid-term outcomes in systemic lupus erythematosus patients presenting with disease exacerbation after SARS-CoV-2 mRNA vaccination: A cohort study from Puerto Rico.

To determine if SARS-CoV-2 mRNA vaccination has an impact on the clinical course of systemic lupus erythematosus (SLE). Puerto Ricans with SLE who received mRNA COVID-19 vaccines were studied. Demographic parameters, clinical manifestations, disease activity (per Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), disease damage (per Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), emergency room visits, hospitalizations, and pharmacologic therapy were determined. Baseline variables (prior to vaccination) were compared between patients with and without exacerbation after SARS-CoV-2 vaccination. Among those with exacerbation, clinical outcomes were determined up to 1year after vaccination. Of the entire cohort (n = 247), 14 (5.7%) had post-vaccination exacerbations. Photosensitivity, oral ulcers, anti-Ro antibodies, higher SLEDAI score, and corticosteroids exposure were associated with post-vaccination flares. Among those with post-vaccination flares, 10 (71.4%) had major organ involvement. No significant differences were observed for mean SLEDAI scores, emergency room visits, hospitalizations, disease damage, and exposure to immunosuppressive drugs before and after SARS-CoV-2 mRNA vaccination. At 12months of follow-up, all patients were fully controlled without evidence of active disease. In our group of SLE patients, 5.7% had a disease flare after SARS-CoV-2 mRNA vaccination. Most had exacerbations involving major organs/systems. Mucocutaneous manifestations, anti-Ro antibodies, disease activity, and corticosteroids were associated with flares. Awareness of these factors and the possibility of a major lupus flare after vaccination with COVD-19 vaccines is critical to provide timely and effective therapy.

CAR T Cell Therapy: A New Hope for Refractory Lupus?

Systemic lupus erythematosus (SLE) is a complex multisystem, autoimmune disorder. The disease involves multiple inflammatory and immune pathways, posing several treatment challenges. The role of B cells in the pathogenesis and disease activity of SLE is well described, and treatments targeting B cells are now well established. Despite advances in therapeutics for SLE, the clinical course of SLE can be associated with significant morbidity and mortality. For many individuals, the management of SLE requires lifelong immunosuppression. Chimeric antigen receptor (CAR) T cell therapy is an emerging treatment for haematological malignancies that uses CAR T cells to target CD19+ B lymphocytes. CAR T cell therapy has shown promising results in murine models of SLE, and a case report and subsequent case series in people with SLE reporting drug-free remission following CAR T cell therapy against CD19+ B cells have recently been published. This review discusses CAR T cell therapy, comparing it with established B cell depletion therapies, such as rituximab, and exploring its safety, limitations and promise in the management of patients with SLE.

LABORATORY BIOMARKERS FOR SYSTEMIC LUPUS ERYTHEMATOSUS

The development of innovative technologies using mono- and multiplex methods of immune analysis contributed to an increase in the sensitivity and specificity of laboratory tests and a significant expansion of the range of laboratory biomarkers of systemic lupus erythematosus (SLE). The article presents modern aspects of laboratory diagnosis of SLE. Screening and confirmatory methods for the study of antinuclear antibodies (ANA), the main immunological marker of SLE, are considered in detail. New classification criteria for SLE are discussed (EULAR/ACR, 2019), which for the first time single out “seropositivity” for the antinuclear factor as a mandatory “incoming” criterion that allows diagnosing the disease as SLE. The characteristics of clinical and laboratory subtypes of SLE associated with the detection of various autoantibodies in serum (antibodies to dsDNA, histones, nucleosomes, Sm, Ro/SSA and La/SSB, U1 ribonucleoprotein,ribosomal protein P, antiphospholipid antibodies, antibodies to C1q) are given. The diagnostic value of defects in the components of the complement system in SLE is indicated. The relationship between ANA and cytokine profiles in SLE patients using multiplex immunoassay of these biomarkers based on xMAP microarray technology is described. It has been shown that the formation of ANA and high activity of SLE are associated with overexpression of the IP-10 and MCP-1 chemokines induced by IFN. The clinical and pathogenetic significance of subpopulations of B-lymphocytes and CD4+ CD25high+ FoxP3+ regulatory T-cells (Treg) of peripheral blood in SLE was noted. SLE is characterized by an increase in the number of double negative memory B cells (CD19+ CD27-IgD-) and plasma cells, a decrease in the levels of naive and transitional B cells in the blood. A positive correlation was found between the number of double negative B cells and the activity of SLE and ESR. The effect of therapy with genetically engineered biological agents (rituximab and belimumab) on B-cell subpopulations in patients with SLE was analyzed. The decrease in the number of Tregs is most pronounced in patients with an acute variant of the course of the disease, high activity of the pathological process, hyperproduction of IgG and expansion of autoreactive B-cells. The study of modern molecularcellular biomarkers allows diagnosing, assessing activity, the nature of the course, clinical and laboratory subtypes of SLE, and predicting the effectiveness of therapy for this disease at a qualitatively new level.

LESIONS OF THE HEMATOPOIETIC SYSTEM IN A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND THE EFFECTIVENESS OF TREATMENT WITH MYCOPHENOLATE MOFETIL

The article presents a clinical case of the use of mycophenolate mofetil in a patient with high activity of systemic lupus erythematosus (SLE) and suppression of hematopoietic systems. The patient had all the characteristic signs of a severe course of SLE with the development of a complication - severe anemia (frequent exacerbations of the main disease, a significant and constant decrease in the number of erythrocytes and the level of hemoglobin in the general blood test against the background of glucocorticosteroid therapy, the severity of clinical symptoms). Damage to the hematopoietic system is characteristic of such patients and prevailed in the clinical picture of the disease and became the direct reason for seeking medical help. The appointment of mycophenolate mofetil made it possible to reduce the activity of manifestations of the main disease, reduce the development of possible complications, and in the future achieve clinical and laboratory remission. The clinical feature of this case is the effective use of mycophenolate mofetil in a patient with high activity of the course of SLE and the constant occurrence of a severe complication - severe anemia, against the background of glucocorticoid therapy. Damage to the hematopoietic system in the patient prevailed in the clinical picture of the disease and became the direct reason for seeking medical help. According to the literature, the use of mycophenolate mofetil in patients gives a positive clinical result in the treatment of such patients: reducing the activity of the course of the main disease, preventing complications, extending the period of clinical and laboratory remission. This approach will improve the quality of life of patients with this group of diseases with the additional advantage of reducing the burden on health care due to the reduction in the frequency of hospitalizations of patients with severe SLE.

Structural Features of Patients with Drusen-like Deposits and Systemic Lupus Erythematosus.

Background: The relevance of drusen-like deposits (DLD) in patients with systemic lupus erythematosus (SLE) is to a large extent uncertain. Their genesis is proposed to be correlated to immune-complex and complement depositions in the framework of SLE. The intention of this study was to determine potential morphological differences in the choroid and retina as well as potential microvascular changes comparing two cohorts of SLE patients divergent in the presence or absence of DLD using multimodal imaging. Methods: Both eyes of 16 SLE patients with DLD were compared to an age- and sex-matched control-group consisting of 16 SLE patients without detectable DLD. Both cohorts were treated with hydroxychloroquine (HCQ) and did not differ in the treatment duration or dosage. Using spectral-domain optical coherence tomography (SD-OCT) choroidal volume measures, choroidal vascularity indices (CVI) and retinal layer segmentation was performed and compared. In addition, by the exploitation of optical coherence tomography angiography vascular density, perfusion density of superficial and deep retinal capillary plexuses and the choriocapillaris were analyzed. For the choroidal OCT-scans, a subset of 51 healthy individuals served as a reference-group. Results: CVI measures revealed a significant reduction in eyes with DLD compared to healthy controls (0.56 (0.54–0.59) versus 0.58 (0.57–0.59) (p = 0.018) and 0.56 (0.54–0.58) versus 0.58 (0.57–0.60) (p < 0.001)). The photoreceptor cell layer presented significant thinning in both eyes of subjects with DLD compared to control subjects without DLD (68.8 ± 7.7 µm vs. 77.1 ± 7.3 µm for right eyes, p = 0.008, and 66.5 ± 10.5 µm vs. 76.1 ± 6.3 µm for left eyes, p = 0.011). OCTA scans revealed no significant changes, yet there could be observed numerically lower values in the capillary plexuses of the retina in eyes with DLD than in eyes without DLD. Conclusions: Our results illustrated significant alterations in the choroidal and retinal analyzes, suggesting a correlation between DLD and the progression of inflammatory processes in the course of SLE leading to retinal degeneration. For this reason, DLD could serve as a biomarker for a more active state of disease.

Gout in the course of systemic lupus erythematosus: Literature review and case study report

Gout is one of the relatively common inflammatory diseases of the joints. It is caused by the deposition of uric acid crystals in the tissues, which induces an acute or chronic inflammatory process. Elevated serum uric acid levels are usually found long before symptoms appear, and it is worth emphasizing that not every hyperuricemic patient will ever develop gout symptoms. The onset of gout is characterized by periodic joint inflammation, which may be triggered by various stress factors (trauma, infection), certain medications, dietary mistakes, and excessive exercise. Over time, repeated joint inflammation causes permanent joint damage. Most often, deposits of urate crystals are located in places with poorer blood supply and exposed to increased pressure, such as joints and soft tissues (e.g., auricles). The coexistence of gout and autoimmune diseases is relatively rare. While for many years it was believed that gout was not associated with other systemic connective tissue diseases, gout has been described in the course of systemic lupus erythematosus, systemic sclerosis, mixed connective tissue disease, psoriatic arthritis, ankylosing spondylitis, and rheumatoid arthritis. The presented systematic review also describes a case of a patient with longlasting systemic lupus erythematosus who was diagnosed with gout.

NET Formation in Systemic Lupus Erythematosus: Changes during the COVID-19 Pandemic.

The severity of the coronavirus disease in 2019 (COVID-19) is strongly linked to a dysregulated immune response. This fuels the fear of severe disease in patients with autoimmune disorders continuously using immunosuppressive/immunomodulating medications. One complication of COVID-19 is thromboembolism caused by intravascular aggregates of neutrophil extracellular traps (NETs) occluding the affected vessels. Like COVID-19, systemic lupus erythematosus (SLE) is characterized by, amongst others, an increased risk of thromboembolism. An imbalance between NET formation and clearance is suggested to play a prominent role in exacerbating autoimmunity and disease severity. Serologic evidence of exposure to SARS-CoV-2 has a minor impact on the SLE course in a Swedish cohort reportedly. Herein, we assessed NET formation in patients from this cohort by neutrophil elastase (NE) activity and the presence of cell-free DNA, MPO-DNA, and NE-DNA complexes and correlated the findings to the clinical parameters. The presence of NE-DNA complexes and NE activity differed significantly in pre-pandemic versus pandemic serum samples. The latter correlated significantly with the hemoglobin concentration, blood cell counts, and complement protein 3 and 4 levels in the pre-pandemic but only with the leukocyte count and neutrophil levels in the pandemic serum samples. Taken together, our data suggest a change, especially in the NE activity independent of exposure to SARS-CoV-2.

Clinical Study on Systemic Lupus Erythematosus Complicated with Knee Bone Infarction.

Objective The present study aims to (1) analyze the clinical characteristics and related influencing factors of knee bone infarction in systemic lupus erythematosus (SLE) and (2) improve the understanding of SLE complicated with knee bone infarction. Methods The data of patients with SLE complicated with knee bone infarction were retrospectively analysed; patients with SLE during the same period who matched in age, gender, and disease duration were selected as control subjects, with a 1 : 1 ratio with the SLE group. The clinical data were collected to analyze the risk factors for SLE complicated with knee bone infarction. Results In a total of 36 (6.4%) of 563 patients aged 19–33 (25.8 ± 4.8) years who had SLE during the same period, the disease was complicated with knee bone infarction. The diagnosis of knee bone infarction was made at an SLE duration of 7–65 (26.2 ± 15.7) months. During the SLE course, knee bone infarction occurred within 1 year in 6 cases (16.7%), within 1–5 years in 28 cases (77.8%), and in >5 years in 2 cases (5.6%). Raynaud's phenomenon incidence and anti-nRNP antibody positivity were significantly higher in the knee bone infarction group than in the control group (P < 0.01 and P < 0.05, respectively). The cumulative glucocorticoid dose at 1, 3, and 6 months was significantly higher in the knee bone infarction group than in the control group (P < 0.05). SLE complicated with knee necrosis had a statistically significant rank correlation with Raynaud's phenomenon (r = 0.445, P < 0.001), anti-nRNP antibody (r = 0.309, P=0.008), and renal injury (r = 0.252, P=0.032). The multivariate analysis of SLE complicated with knee bone infarction showed that Raynaud's phenomenon was an independent influencing factor for the complicated knee bone infarction in SLE patients (OR = 4.938, P=0.004), and the probability of SLE complicated with knee bone infarction in Raynaud's phenomenon positive patients was 4.938 times that of Raynaud's phenomenon negative patients. Conclusions The risk of knee bone infarction was relatively high in patients with SLE within a 5-year disease course and in young patients. The risk factors were Raynaud's phenomenon, anti-nRNP antibody positivity, and early high-dose glucocorticoid therapy.

Psychiatric manifestations of rheumatic diseases

Introduction and purpose of the work: Rheumatic diseases are chronic diseases that cause symptoms in many systems of the human body. Their most common symptoms include pain and symptoms of arthritis, their deformities, fatigue, and malaise. The aim of the article is to present the symptoms and mental disorders occurring in the course of selected rheumatic diseases.&#x0D; State of knowledge: The psychological symptoms are characteristic of systemic lupus erythematosus. Specified is a form of lupus called NSPLE (neuropsychiatric systemic lupus erythematosus), which includes neuropsychiatric symptoms in the course of systemic lupus erythematosus. Psychiatric symptoms are also present in the course of other rheumatic diseases. Neuropsychiatric symptoms may affect up to 80% of patients with primary Sjogren's syndrome, with 50% to 80% ahead of diagnosis. It has been proven that systemic slcerosis causes microvascular damage, which may cause neuropsychiatric symptoms in the form of mood disorders, anxiety and cognitive disorders. In one study, 59% of patients with fibromyalgia experienced mania, which was more than twice as high as in the control group.&#x0D; Summary: In the course of all rheumatic diseases presented by us, there are symptoms and mental disorders. They are often mood, cognitive and sleep disturbances. It should be emphasized that the etiology of psychiatric symptoms is multifactorial.

Methods of assessment of joint involvement in various systemic connective tissue diseases.

Joint involvement is one of the most common clinical manifestations of systemic connective tissue diseases (CTD). Joint symptoms can take various forms, ranging from joint pain to mono-arthritis or symmetrical poly-arthritis. In most cases, arthritis takes a non-destructive form, such as in the course of systemic lupus erythematosus or primary Sjögren’s syndrome, to destructive arthritis in overlap syndromes of CTD with rheumatoid arthritis. In addition, apart from the wide variety of forms of joint involvement, it should be noted that joint symptoms may be one of the domains suggesting a severe course of the disease.The study attempts to present the methods of assessing the involvement of the locomotor system. The search for appropriate scales to determine the degree of joint involvement is important in assessing the severity of joint changes, has an impact on the overall degree of disease activity, and allows for timely implementation of appropriate treatment.

Biologic Agents and Other Emerging Therapies for Childhood SLE.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by remissions and flares. Twenty percent of SLE presents in childhood where the course of SLE is often more severe with significant morbidity and mortality. Several biologic agents have been developed recently for the treatment of lupus, and although some have proven to be safe and efficacious, many have failed to demonstrate significant benefit in clinical trials. There continues to be a desperate need for safe, effective medications that target specific pathway abnormalities seen in SLE. This is an area of intense research that is changing clinical practice in the treatment of childhood SLE. In this article, we discuss the use of B-cell inhibitors, including belimumab and rituximab, as well as the anti-complement drug eculizumab. Promising treatments on the horizon include the jak-stat inhibitors as well as anifrolumab, which targets interferon. [Pediatr Ann. 2022;51(2):e63-e71.].

Cardiovascular Manifestations of Systemic Lupus Erythematosus: An Overview

Systemic Lupus Erythematosus(SLE) is a multisystem autoimmune disease with variable presentations. A proposed mechanism for the etiology of SLE involves the development of autoantibodies that result from a defect in apoptosis. The specific defect involves the “find-me” (adenosine triphosphate [ATP]/uridine triphosphate [UTP]) or “eat-me” (phosphatidylserine) signals activated upon release of red cell nuclei. In the absence of apoptosis, the nuclei break down, causing inflammation and contributing to the development of autoimmunity. Many signs and symptoms of SLE are caused by either circulating immune complexes or direct effects of antibodies on cells. A genetic predisposition for SLE exists, and the concordance rate in monozygotic twins is between 25% and 70%. If a mother has SLE, her daughter’s risk of developing the disease is 1:40, and her son’s risk is 1:250. The course of SLE consists of intermittent remissions punctuated by disease flares, and organ damage often progresses over time. Pericarditis is the most frequent cardiac manifestation; it can be documented by ECG, auscultation of a friction rub, or evidence of pericardial effusion. It usually responds to anti-inflammatory therapy and infrequently leads to tamponade. More serious cardiac manifestations are myocarditis and fibrinous endocarditis of Libman-Sacks. The endocardial involvement can lead to valvular insufficiencies, most commonly of the mitral or aortic valves, or embolic events. It has not been proven that glucocorticoid or other immunosuppressive therapies improve lupus myocarditis or endocarditis. Still, it is usual practice to administer a trial of high-dose steroids and appropriate supportive treatment for heart failure, arrhythmia, or embolic events. As discussed above, patients with SLE are at increased risk for myocardial infarction, usually due to accelerated atherosclerosis, which probably results from immune attack, chronic inflammation, and chronic oxidative damage to arteries.