Antibodies In Systemic Lupus Erythematosus Research Articles

Recurrent pulmonary thromboembolism with cardiac tamponade as initial manifestations of lupus and antiphospholipid syndrome: a case report

Systemic lupus erythematosus (SLE) is an inflammatory autoimmune disease with an important course due to systemic compromise. SLE is frequently associated with antiphospholipid syndrome, and pulmonary thromboembolism (PE) is particularly common. It is extremely rare for PE to be the initial clinical presentation and even more uncommon for it to coincide with cardiac tamponade, representing a challenge in diagnosis and management. We present a case of a 42-year-old woman with recurrent PE with severe pleural and pericardial effusion, hemodynamic instability, and cardiac tamponade. Laboratory workup revealed hypocomplementemia, leukopenia, negative SLE antibodies, and a positive lupus anticoagulant. This case emphasizes the importance of determining the etiology of PE, assessing risk classification, and implementing proper management, which are crucial for the patient's survival and outcome.

Beyond paraneoplastic neurological syndromes: Anti-neuronal antibodies in neuropsychiatric systemic lupus erythematosus.

Anti-neuronal antibodies target antigens produced by tumour cells and cells of nervous system. These antibodies are formed as a result of autoimmune response elicited by the underlying malignancy, when proteins restricted to immune privileged neurons are presented by the tumour. Previous studies have shown presence of anti-neuronal antibodies in systemic lupus erythematosus and neuropsychiatric lupus (NPSLE) but information on individual antibodies and their pathogenic role is lacking. To assess the frequency of anti-neuronal antibodies in our neuropsychiatric lupus cohort and to assess any significant association with specific neurological syndrome and to see if the antibodies were more likely to occur in active rather than inactive neuropsychiatric lupus. This cross-sectional study was conducted in our center from 2019 to 2022. Neuropsychiatric manifestations were defined according to 1999 American College of Rheumatology (ACR) nomenclature and case definitions for neuropsychiatric lupus. Samples were taken from active or inactive NPSLE patients with their informed consent. Testing was done on an anti-neuronal antigen panel which consisted of [Amphiphysin, CV2, GAD 65, PNMA2 (Ma-2/Ta), Ri, Yo, Hu, recoverin, SOX1, titin, Zic, Tr)] by semi-quantitative Line immune assay. Association between the categorical variables and antibody positivity group was established using chi-square/Fisher's exact test as appropriate. 65 patients were recruited, of which 23 (35%) patients had active NPSLE at the time of sample collection. Anti-neuronal antibodies were positive in 13/65 (20%) patients with anti-Gad 65 antibodies having the highest frequency (6.2%) followed by anti CV 2 (3.1%), anti Sox1 (3.1%), anti Amphiphysin (3.1%) anti recoverin (1.5%), anti Yo (1.5%) and anti Zic (1.5%). The panel of anti-neuronal antibodies did not show any specific association with NPSLE features.However, an interesting finding was that, patients with active disease had higher odds of having anti-neuronal antibodies with an OR = 10 (95% CI:2.38 -42) (p < 0.001) than inactive disease. Anti-neuronal antibodies were more likely to be positive in active neuropsychiatric lupus patients, and these antibodies which are commonly used to diagnose paraneoplastic syndromes may have a potential role in the diagnosis of NPSLE.

Anti-TCP1 Antibody Is a Potential Biomarker for Diagnosing Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease caused by autoantibodies. Serum samples from patients with SLE (n = 10) were compared with those from normal controls (NCs, n = 5) using 21K protein chip analysis to identify a biomarker for SLE, revealing 63 SLE-specific autoantibodies. The anti-chaperonin-containing t-complex polypeptide-1 (TCP1) antibody exhibited higher expression in patients with SLE than in NCs. To validate the specificity of the anti-TCP1 antibody in SLE, dot blot analysis was conducted using sera from patients with SLE (n = 100), rheumatoid arthritis (RA; n = 25), Behçet's disease (BD; n = 28), and systemic sclerosis (SSc; n = 30) and NCs (n = 50). The results confirmed the detection of anti-TCP1 antibodies in 79 of 100 patients with SLE, with substantially elevated expression compared to both NCs and patients with other autoimmune diseases. We performed an enzyme-linked immunosorbent assay to determine the relative amounts of anti-TCP1 antibodies; markedly elevated anti-TCP1 antibody levels were detected in the sera of patients with SLE (50.1 ± 17.3 arbitrary unit (AU), n = 251) compared to those in NCs (33.9 ± 9.3 AU), RA (35 ± 8.7 AU), BD (37.5 ± 11.6 AU), and SSc (43 ± 11.9 AU). These data suggest that the anti-TCP1 antibody is a potential diagnostic biomarker for SLE.

Cardiac Sarcoma Mimicking Libman-Sacks Endocarditis in a Patient with Systemic Lupus Erythematosus (SLE): A Case Report and Literature Review.

We present the case of a 39-year-old woman who was diagnosed with SLE and antiphospholipid antibodies 8 years ago. The chief manifestations of her disease included low-grade fever and polyarthritis. Eight months before presentation, she experienced symptoms attributed to a flare of SLE, leading to an increase in immunomodulatory treatment with no improvement. She presented to the emergency room with acute onset of dyspnea. Clubbing of her fingers and toes was noted. When questioned, she reported the onset of clubbing 5 months earlier. A CTA was performed to rule out pulmonary embolism, which was excluded, although it revealed a severely damaged mitral valve with severe insufficiency and a large mass on the valve, protruding into the left atrium. Antibiotics were started, with a working diagnosis of infectious endocarditis; however, the severe mitral valve dysfunction lead to emergency mitral valve replacement, revealing an organized thrombus. She was treated with anticoagulation, with a working diagnosis of Libman-Sacks endocarditis, with no improvement. Additional immunosuppression failed to improve her symptoms. Enlargement of the thrombotic mass and an increased gradient across the prosthetic mitral valve led to repeat surgery, culminating in a diagnosis of high-grade sarcoma within the left atrial mass. We further discuss cardiac sarcoma and describe the occurrence of clubbing in patients with sarcoma. This case highlights the importance of interdisciplinary collaboration and the need for vigilant monitoring in refractory cases, particularly when atypical presentations arise.

A Case of Immune-Mediated Coombs-Negative Hemolytic Anemia in a Patient with Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is an auto-immune connective tissue disorder that can involve any organ in the body. The American College of Rheumatology criteria (ACR) include autoimmune hemolytic anemia with reticulocytosis as one of the criteria in the diagnosis of SLE. Anti-erythrocyte antibodies in SLE are mainly warm-type IgG. We present a case of systemic lupus erythematosus presenting with immune-mediated Coombs-negative hemolytic anemia. This case highlights the possibility of autoimmune hemolysis occurring with a negative Coombs test, where the autoantibody levels are too low to be detected by a conventional direct antiglobulin test. (DAT)

Serum isolevuglandin IgG antibody concentrations are increased in patients with systemic lupus erythematosus and associated with lower 24-hour blood pressure

ObjectiveHypertension is frequent in patients with systemic lupus erythematosus (SLE) and is a major contributor to increased cardiovascular risk. Isolevuglandins (IsoLGs) are downstream products of oxidative stress that drive hypertension and SLE disease activity in animal models. Antibodies to IsoLGs (anti-IsoLGs) are present in human SLE and associated with disease activity, but it is not known if concentrations are higher compared to control subjects or if they are associated with blood pressure (BP).MethodsWe measured serum anti-IsoLG IgG antibody concentrations by sandwich ELISA in 23 patients with SLE and 30 controls who had participated in a cross-sectional 24-hour ambulatory BP study. We examined the association between anti-IsoLG IgG antibodies and BP measurements in patients with SLE and controls by Spearman Rho (rs) and linear regression analysis.ResultsSerum anti-IsoLG IgG antibody concentrations were higher in patients with SLE than controls (P = 0.007) and inversely associated with BP in SLE but not controls. In patients with SLE antibody concentrations were inversely associated with office (rs = −0.418) and diurnal systolic BP (rs = −0.421); the relationship was stronger among patients not taking anti-hypertensives (office: rs = −0.740, diurnal systolic BP: rs = −0.802) and every 20% increase in antibody concentration was associated with 10 mmHg decrease in 24-hour systolic BP (P = 0.004).ConclusionSerum anti-IsoLG IgG antibody concentrations are higher in patients with SLE than controls and are inversely associated with 24-hour BP measurements. Since IsoLGs promote hypertension, it is possible that in SLE, IsoLG antibodies could help clear these hypertension-inducing antigens.

Elevated expression of hsa_circ_0000479 in neutrophils correlates with features of systemic lupus erythematosus

Objective Accumulating evidence suggests that differentially expressed circular RNAs (circRNAs) play critical roles in immune cells of systemic lupus erythematosus (SLE) patients. Hsa_circ_0000479 has been studied in the field of cancer and infection, whereas seldom studied in autoimmune diseases. The aim of this study was to investigate the role and clinical value of neutrophil hsa_circ_0000479 in SLE. Methods The expression levels of hsa_circ_0000479 in both healthy individuals and SLE patients’ neutrophils were detected by qPCR and compared with those in peripheral blood mononuclear cells (PBMCs) . In addition, the correlation of hsa_circ_0000479 levels in neutrophils with the clinical and immunological features of SLE patients was also analysed. Results The expression levels of hsa_circ_0000479 in the patients with SLE were significantly higher in neutrophils than that of PBMCs, and also significantly higher than that in healthy controls (HCs). Moreover, the expression levels of hsa_circ_0000479 in neutrophils were negatively associated with absolute neutrophil count and complement 3 (C3), whereas positively correlated with anti-dsDNA and anti-nucleosome antibodies in SLE. In addition, SLE patients with higher levels of hsa_circ_0000479 demonstrated more several clinical manifestations, including Raynaud’s phenomenon, alopecia and leucopenia. Conclusions Hsa_circ_0000479 is up-regulated in neutrophils of SLE patients, and is also associated with several important laboratory indicators and clinical manifestations, suggesting that hsa_circ_0000479 in neutrophils was one of probable factors involved in the pathogenesis of SLE with potential clinical value.

Angiotensin II Type 2 Receptor Antibodies in Glomerular Diseases.

We evaluated the concentration of AT2R antibodies in 136 patients with primary and secondary glomerular diseases: membranous nephropathy (n = 18), focal and segmental glomerulosclerosis (n = 25), systemic lupus erythematosus (n = 17), immunoglobulin A (IgA) nephropathy (n = 14), mesangial (non-IgA) proliferative nephropathy (n = 6), c-ANCA vasculitis (n = 40), perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) vasculitis (n = 16), and compared it with a healthy control group (22 patients). Serum creatinine levels, proteinuria, serum albumin, and total protein concentrations were prospectively recorded for 2 years. The mean levels of AT2R antibodies in the lupus nephropathy group were significantly higher compared to the control group, 64.12 ± 26.95 units/mL and 9.72 ± 11.88 units/mL, respectively. There was no association between this level and the clinical course of the disease. The AT2R levels in other kinds of glomerular disease were no different from the control group. We found significant correlations between AT1R and AT2R in patients with membranous nephropathy (r = 0.66), IgA nephropathy (r = 0.61), and c-ANCA vasculitis (r = 0.63). Levels of AT2R antibodies in systemic lupus erythematosus are higher compared to other types of glomerulonephritis, vasculitis, and a healthy control group. Levels of AT2R antibodies correlate with AT1R antibodies in the groups of patients with membranous nephropathy, IgA nephropathy, and c-ANCA vasculitis. These kinds of AT2R antibodies have a stimulative effect on AT2R, but we have not found the influence of these antibodies on the clinical course of glomerular diseases.

Hydrolysis of Oligodeoxyribonucleotides on the Microarray Surface and in Solution by Catalytic Anti-DNA Antibodies in Systemic Lupus Erythematosus.

Anti-DNA antibodies are known to be classical serological hallmarks of systemic lupus erythematosus (SLE). In addition to high-affinity antibodies, the autoantibody pool also contains natural catalytic anti-DNA antibodies that recognize and hydrolyze DNA. However, the specificity of such antibodies is uncertain. In addition, DNA binding to a surface such as the cell membrane, can also affect its recognition by antibodies. Here, we analyzed the hydrolysis of short oligodeoxyribonucleotides (ODNs) immobilized on the microarray surface and in solution by catalytic anti-DNA antibodies from SLE patients. It has been shown that IgG antibodies from SLE patients hydrolyze ODNs more effectively both in solution and on the surface, compared to IgG from healthy individuals. The data obtained indicate a more efficient hydrolysis of ODNs in solution than immobilized ODNs on the surface. In addition, differences in the specificity of recognition and hydrolysis of certain ODNs by anti-DNA antibodies were revealed, indicating the formation of autoantibodies to specific DNA motifs in SLE. The data obtained expand our understanding of the role of anti-DNA antibodies in SLE. Differences in the recognition and hydrolysis of surface-tethered and dissolved ODNs need to be considered in DNA microarray applications.

An Emerging Role for Anti-DNA Antibodies in Systemic Lupus Erythematosus.

Anti-DNA antibodies are hallmark autoantibodies produced in systemic lupus erythematosus (SLE), but their pathogenetic role is not fully understood. Accumulating evidence suggests that some anti-DNA antibodies enter different types of live cells and affect the pathophysiology of SLE by stimulating or impairing these cells. Circulating neutrophils in SLE are activated by a type I interferon or other stimuli and are primed to release neutrophil extracellular traps (NETs) on additional stimulation. Anti-DNA antibodies are also involved in this process and may induce NET release. Thereafter, they bind and protect extracellular DNA in the NETs from digestion by nucleases, resulting in increased NET immunogenicity. This review discusses the pathogenetic role of anti-DNA antibodies in SLE, mainly focusing on recent progress in the two research fields concerning antibody penetration into live cells and NETosis.

Neurological complexity in systemic lupus erythematosus: insights into autoimmune encephalitis

Background: Autoimmune encephalitis (AE) is an inflammatory syndrome characterized by autoantibodies targeting the central nervous system (CNS), leading to a wide range of neurological and psychiatric manifestations. Objective: This systematic review aims to investigate the coexistence of AE and systemic lupus erythematosus (SLE) to shed light on potential underlying mechanisms. Materials and Methods: Following PRISMA guidelines, a comprehensive search was conducted using Boolean logic in research databases i.e., PubMed, ProQuest, ScienceDirect, and Web of Science to identify relevant studies on AE, SLE, and their potential correlation. After the initial screening, exclusion, and quality assessment using the Joanna Briggs Institute Critical Appraisal Checklist, 14 studies were included for analysis. Among 18 patients, 88.9% were female whose an average age was 31.9 ± 12.7 years. Clinical presentations were varied, including cognitive impairment (66.67%), seizures (61.1%), altered mental status (50%), and movement disorders. Neuroimaging and neurophysiological findings consistently aligned with encephalitis, with MRI abnormalities observed in 94.4% of cases. Various autoantibodies were identified, including anti-NMDAR, anti-AMPA-R2, and anti-GluR antibodies. Conclusion: The identification of neuronal antibodies is crucial, particularly in scenarios involving both neuropsychiatric SLE (NPSLE) and encephalitis. This investigation aims to reveal etiological insights. The ongoing debate revolves around whether SLE-related encephalitis is associated with NPSLE through SLE antibodies or involves distinct antibodies. Further clinical research is essential to clarify the pathogenic role of autoantibody in SLE-linked AE.

Clinical value of chemiluminescence method for detection of antinuclear antibody profiles.

Antinuclear antibodies (ANAs) are crucial in diagnosing autoimmune diseases, mainly systemic lupus erythematosus (SLE). This study aimed to compare the performance of chemiluminescence assay (CLIA) and line immunoassay (LIA) in detecting ANAs in patients with autoimmune diseases, evaluate their diagnostic accuracy for SLE, and develop a novel diagnostic model using CLIA-detected antibodies for SLE. Specimens from patients with autoimmune diseases and physical examination specimens were collected to parallel detect specific antibodies. Individual antibodies' diagnostic performance and a model combining multiple antibodies were assessed. The findings provide valuable insights into improving the diagnosis of SLE through innovative approaches. To compare the performance of CLIA and LIA in detecting ANAs in patients with autoimmune diseases, assess their accuracy for SLE, and develop a novel diagnostic model using CLIA-detected antibodies for SLE. Specimens have been obtained from 270 patients with clinically diagnosed autoimmune disorders, as well as 130 physical examination specimens. After that, parallel detection of anti-double-stranded DNA (dsDNA) antibody, anti-histone (Histone) antibody, anti-nucleosome (Nuc) antibody, anti-Smith (Sm) antibody, anti-ribosomal P protein (Rib-P) antibody, anti-sicca syndrome A (Ro60) antibody, anti-sicca syndrome A (Ro52) antibody, anti-sicca syndrome (SSB) antibody, anti-centromere protein B (Cenp-B) antibody, anti-DNA topoisomerase 1 (Scl-70) antibody, anti-histidyl tRNA synthetase (Jo-1) antibody, and anti-mitochondrial M2 (AMA-M2) antibody was performed using CLIA and LIA. The detection rates, compliance rates, and diagnostic performance for SLE were compared between the two methodologies, followed by developing a novel diagnostic model for SLE. CLIA and LIA exhibited essentially comparable detection rates for anti-dsDNA antibody, anti-Histone antibody, anti-Nuc antibody, anti-Sm antibody, anti-Rib-P antibody, anti-Ro60 antibody, anti-Ro52 antibody, anti-SSB antibody, anti-Cenp-B antibody, anti-DNAScl-70 antibody, anti-Jo-1 antibody and anti-AMA-M2 antibody (P > 0.05). The two methods displayed identical results for the detection of anti-dsDNA antibody, anti-Histone antibody, anti-Nuc antibody, anti-Sm antibody, anti-Ro60 antibody, anti-Ro52 antibody, anti-SSB antibody, anti-Cenp-B antibody, anti-Scl-70 antibody, and anti-AMA-M2 antibody (Kappa > 0.7, P < 0.05), but showed a moderate agreement for the detection of anti-Rib-P antibody and anti-Jo-1 antibody (Kappa = 0.671 and 0.665; P < 0.05). In addition, the diagnostic performance of these antibodies detected by both methods was similar for SLE. The diagnostic model's area under the curve values, sensitivity, and specificity, including an anti-dsDNA antibody and an anti-Ro60 antibody detected by CLIA, were 0.997, 0.962, and 0.978, respectively. These values were higher than the diagnostic performance of individual antibodies. CLIA and LIA demonstrated excellent overall consistency in detecting ANA profiles. A diagnostic model based on CLIA-detected antibodies can successfully contribute to developing a novel technique for detecting SLE.

Limited Association between Antibodies to Oxidized Low-Density Lipoprotein and Vascular Affection in Patients with Established Systemic Lupus Erythematosus.

Patients with systemic lupus erythematosus (SLE) are at an increased risk of cardiovascular disease. We aimed to evaluate whether antibodies to oxidized low-density lipoprotein (anti-oxLDL) were associated with subclinical atherosclerosis in patients with different SLE phenotypes (lupus nephritis, antiphospholipid syndrome, and skin and joint involvement). Anti-oxLDL was measured by enzyme-linked immunosorbent assay in 60 patients with SLE, 60 healthy controls (HCs) and 30 subjects with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Intima-media thickness (IMT) assessment of vessel walls and plaque occurrence were recorded using high-frequency ultrasound. In the SLE cohort, anti-oxLDL was again assessed in 57 of the 60 individuals approximately 3 years later. The levels of anti-oxLDL in the SLE group (median 5829 U/mL) were not significantly different from those in the HCs group (median 4568 U/mL), while patients with AAV showed significantly higher levels (median 7817 U/mL). The levels did not differ between the SLE subgroups. A significant correlation was found with IMT in the common femoral artery in the SLE cohort, but no association with plaque occurrence was observed. The levels of anti-oxLDL antibodies in the SLE group were significantly higher at inclusion compared to 3 years later (median 5707 versus 1503 U/mL, p < 0.0001). Overall, we found no convincing support for strong associations between vascular affection and anti-oxLDL antibodies in SLE.

Antibodies against neutrophil extracellular traps (NETs) potentiate clinical performance of anti-double-stranded DNA antibodies in systemic lupus erythematosus

Autoantibodies against NETs (ANETA) are present in SLE patients. We aimed to determine the clinical relevance of ANETA in SLE. Serum from 129 SLE patients, 161 patients with various rheumatoid diseases (DC), and 53 healthy controls (HC) were tested by a home-made ANETA ELISA platform. ANETA showed a sensitivity of 35.7% and a specificity of 92.5%, respectively, in the diagnosis of SLE. The combination of ANETA with anti-dsDNA antibody increased the diagnostic sensitivity from 49.6% to 62.8% for SLE. The presence of ANETA potentiates the clinical utility of anti-dsDNA antibodies in identifying a subset of SLE patients with higher disease activity and hematological abnormalities. The binding of ANETA to NETs did not inhibit the immunostimulatory effect of NETs. Our findings suggested that ANETA have potential as clinically relevant biomarkers that potentiate the clinical performance of anti-dsDNA antibodies in the diagnosis, risk stratification and subtyping of patients with SLE.

Patient with Systemic Lupus Erythematodes, high pANCA antibodies and end-stage renal failure

A 47-year-old female patient with active Systemic lupus erythematosus (SLE) - skin, joint, hematological, and renal involvement was admitted for rapidly progressive renal failure at the background of very high anti-dsDNA and pANCA antibodies. Active pathogenetic treatment was started pulse therapy with methylprednisolone, intravenous immunoglobulins (IVIG) and cyclophosphamide, blood transfusions were performed, symptomatic treatment with infusions ad diuretic was initiated. Due to progression of renal failure with volume overload and increase in urea and creatinine levels renal-replacement therapy was initiated. The patient developed E. faecalis and E. durans sepsis with disseminated intravascular coagulation and ground-glass-type lung changes and the condition further deteriorated to fatal multiorgan failure. The authors discuss the role of pANCA antibodies in SLE with renal and lung involvement.

A cross-sectional study to know the prevalence of thyroid dysfunction in systemic lupus erythematosus

Background: Systemic lupus erythematosus (SLE) is a persistent autoimmune disease, the pathogenesis of which remains elusive. Autoimmune factors may be a cause of SLE and thyroid dysfunction. Many studies have revealed that the prevalence of thyroid disorder is higher in SLE patients than in the general population. SLE is a multisystem and hypothyroidism is an organ specific autoimmune disorder and can occur successively or simultaneously. Aims and Objectives: The aim of the study was to study the prevalence of thyroid disorder in patients with SLE. Materials and Methods: Patients admitted with definite clinical features of SLE and Antinuclear Antibodies positive, in medicine ward and healthy blood donors are taken as control. Sample was tested by fully automated analyzer. Results: Subclinical hypothyroidism was found in 24% of study group and 8% of control group which is statistically significant. Central and secondary hyperthyroidism was found in 10% of study group and 12% of control group but it was statistically insignificant. Several studies have documented an association between SLE and other autoimmune diseases such as Sjogren’s syndrome, autoimmune hemolytic anemia, and antiphospholipid syndrome. Subclinical hypothyroidism was higher than another thyroid dysfunction such as primary, central, and subclinical hypothyroidism was found to be higher in frequency, probably depicting the slow destructive process which is pathognomic of autoimmune thyroiditis. Conclusion: Subclinical hypothyroidism was more prevalent in SLE than that of overt hypothyroidism as compared with general population.

Challenges in Family Planning and Pregnancy for Women with Rheumatic Diseases

Systemic rheumatic diseases (RDs) often affect women in their reproductive years and may complicate family planning and pregnancy. For women with RD who are not pursuing pregnancy, especially those at the highest risk of maternal morbidity or on teratogenic medications, effective contraception is important. For women with active systemic lupus erythematosus (SLE) and/or antiphospholipid antibodies, oestrogen-containing contraceptive methods are generally not recommended. Emergency contraception and induced abortion are safe for women with RD and should be discussed when clinically appropriate. Women with RD commonly have questions and concerns about the impact of their disease on fertility and conception. RD-associated factors may contribute to difficulties in conceiving and decreased family size. Assisted reproductive technology (ART) is often a safe option for women with RD. However, precautions and specific treatment modifications may be needed, particularly in women with SLE and/or antiphospholipid antibody positivity. SLE and antiphospholipid syndrome (APS) are the RDs associated with the greatest risk of maternal and foetal pregnancy complications. Regardless of the specific RD, having an active disease is a significant risk factor for poor pregnancy outcomes. With appropriate preconception planning and multidisciplinary management during and after pregnancy, most women with RD can have successful pregnancies.

Systemic Lupus Erythematosus (SLE): An often-overlooked cause of Cytopenias

Background: Haematological manifestations are common in SLE, but cytopenias are rarely the only abnormality at diagnosis. Case Summary: We report the case of a 40-year-old woman who was admitted for recurrent anaemia with a 2-month history of constitutional symptoms and no other complaints. Her Full Blood Count (FBC) showed bi-cytopenia, a Haemoglobin level of 4g/dl and a platelet level of 77x103/μl. An Autoimmune serology revealed a positive Antinuclear antibody (ANA) test at 1:2560 and positive SLE antibodies. The diagnosis of SLE was established using the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 classification criteria. She was commenced on steroids and Disease Modifying Anti-Rheumatic Drugs (DMARD), and there was a sustained increase in her Haemoglobin and platelet count. She subsequently required no further blood transfusions. Conclusion: SLE can present in a myriad of ways. It is, however, essential to note that haematological features may be the sole manifestation at the time of diagnosis. Early recognition and commencement of appropriate treatment often improve prognosis.

Anti-DFS70 antibodies in systemic lupus erythematosus: Prevalence in a large Chinese cohort and an unexpected association with anti-dsDNA antibodies by a long-term follow-up.

ObjectiveMonospecific autoantibodies to dense fine speckles 70 (DFS70) antigen are purported to aid in excluding systemic autoimmune rheumatic diseases (SARD) such as systemic lupus erythematosus (SLE). However, the non-isolated anti-DFS70 still has a certain prevalence in SLE patients, and the clinical significance remains unclear. We aimed to investigate the prevalence, clinical relevance, and value of long-term monitoring of anti-DFS70 antibodies in SLE patients.MethodsAnti-DFS70 antibodies were measured by enzyme-linked immunosorbent assay (ELISA) in 851 SLE patients, 211 healthy individuals, and 194 patients with other SARD (except SLE). Demographic, serological, and clinical associations of anti-DFS70 antibodies were analyzed by a stepwise multivariable logistic regression model. The correlation of anti-DFS70 with anti-dsDNA, anti-C1q, and SLE Disease Activity Index 2000 (SLEDAI-2K) was analyzed. Sixty-one SLE patients with follow-up time ranging from 2 to 57 months were measured anti-DFS70 antibodies using both ELISA and line immunoassay. The dynamic variations of anti-DFS70 antibodies were evaluated with anti-dsDNA, anti-C1q, and SLEDAI-2K during the follow-up.ResultsThe prevalence of anti-DFS70 was significantly higher in SLE (20.7% (176/851)) than in healthy individuals (9.5% (20/211), p = 0.0002) and other SARD (10.8% (21/194), p = 0.002). Multivariable analysis revealed that anti-DFS70-positive SLE patients were associated with younger age (odds ratio (OR) = 0.982; 95% confidence interval (CI) = 0.969, 0.995), higher frequencies of anti-dsDNA (OR 1.598; 95% CI 1.107, 2.306) and anti-PCNA (OR 6.101; 95% CI 2.534, 14.688), and higher levels of serum IgG (OR 1.097; 95% CI 1.067, 1.129) and were more likely to be accompanied by mucosal ulcers (OR 5.921; 95% CI 1.652, 21.215). The O.D. value of anti-DFS70 positively correlated with levels of anti-dsDNA (r = 0.183, p < 0.0001) and anti-C1q (r = 0.181, p < 0.0001), respectively, but not with SLEDAI-2K (p = 0.920). During the follow-up, 49 (42 negative and 7 positive) patients remained stable with anti-DFS70 levels. The other 12 patients experienced significant changes in anti-DFS70, and 83.3% (10/12) of them showed similar trends between anti-DFS70 and anti-dsDNA by evaluation of dynamic variations.ConclusionAnti-DFS70 antibodies seem to be prevalent in Chinese SLE patients. The positive association of anti-DFS70 with anti-dsDNA and consistent dynamic variation between anti-DFS70 and anti-dsDNA during the follow-up suggested a potential relationship between anti-DFS70 and anti-dsDNA in patients with SLE.

Lower disease activity but higher risk of severe COVID-19 and herpes zoster in patients with systemic lupus erythematosus with pre-existing autoantibodies neutralising IFN-α

ObjectivesType-I interferons (IFNs-I) have potent antiviral effects. IFNs-I are also overproduced in patients with systemic lupus erythematosus (SLE). Autoantibodies (AAbs) neutralising IFN-α, IFN-β and/or IFN-ω subtypes are strong determinants of...