Revisiting the complement system in systemic lupus erythematosus

Sharma, Manisha; Vignesh, Pandiarajan; Tiewsoh, Karalanglin; Rawat, Amit

doi:10.1080/1744666x.2020.1745063

Cited by 28 publications

(21 citation statements)

References 98 publications

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“…SLE has long been associated with monogenic complement defects (35). Interestingly, several additional genes that cause PID have recently been linked to monogenic SLE and other autoimmune/autoinflammatory disorders (1,(35)(36)(37)(38)(39)(40). We might not have excluded SLE patients if we had begun this study today.…”

Section: Discussionmentioning

confidence: 99%

Primary Immunodeficiency in Children With Autoimmune Cytopenias: Retrospective 154-Patient Cohort

et al. 2021

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BackgroundPrimary immunodeficiency is common among patients with autoimmune cytopenia.ObjectiveThe purpose of this study is to retrospectively identify key clinical features and biomarkers of primary immunodeficiency (PID) in pediatric patients with autoimmune cytopenias (AIC) so as to facilitate early diagnosis and targeted therapy.MethodsElectronic medical records at a pediatric tertiary care center were reviewed. We selected 154 patients with both AIC and PID (n=17), or AIC alone (n=137) for inclusion in two cohorts. Immunoglobulin levels, vaccine titers, lymphocyte subsets (T, B and NK cells), autoantibodies, clinical characteristics, and response to treatment were recorded.ResultsClinical features associated with AIC-PID included splenomegaly, short stature, and recurrent or chronic infections. PID patients were more likely to have autoimmune hemolytic anemia (AIHA) or Evans syndrome than AIC-only patients. The AIC-PID group was also distinguished by low T cells (CD3 and CD8), low immunoglobulins (IgG and IgA), and higher prevalence of autoantibodies to red blood cells, platelets or neutrophils. AIC diagnosis preceded PID diagnosis by 3 years on average, except among those with partial DiGeorge syndrome. AIC-PID patients were more likely to fail first-line treatment.ConclusionsAIC patients, especially those with Evans syndrome or AIHA, should be evaluated for PID. Lymphocyte subsets and immune globulins serve as a rapid screen for underlying PID. Early detection of patients with comorbid PID and AIC may improve treatment outcomes. Prospective studies are needed to confirm the diagnostic clues identified and to guide targeted therapy.

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Section: Discussionmentioning

confidence: 99%

Primary Immunodeficiency in Children With Autoimmune Cytopenias: Retrospective 154-Patient Cohort

et al. 2021

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“…SLE has been described as an immune complex disease, since it is often associated with decreased levels of complement components (140). Other characteristics are the presence of ANAs, notably DNA Abs, which are included in the SLEDAI, but in many cases AuAbs to a heterogenous panel of AuAgs are present and changes in the AuAb profile may reflect changes in disease activity (141)(142)(143)(144).…”

Section: Epstein-barr Virus and Systemic Lupus Erythematosusmentioning

confidence: 99%

Epstein-Barr Virus and Systemic Autoimmune Diseases

2021

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Epstein-Barr Virus (EBV) is an extremely successful human herpes virus, which infects essentially all human beings at some time during their life span. EBV infection and the associated immune response results in production of antibodies (seroconversion), which occurs mainly during the first years of life, but may also happen during adolescence or later in life. Infection of adolescents can result in infectious mononucleosis, an acute serious condition characterized by massive lymphocytosis. Transmission of EBV mainly occurs through saliva but can rarely be spread through semen or blood, e.g. through organ transplantations and blood transfusions. EBV transmission through oral secretions results in infection of epithelial cells of the oropharynx. From the epithelial cells EBV can infect B cells, which are the major reservoir for the virus, but other cell types may also become infected. As a result, EBV can shuttle between different cell types, mainly B cells and epithelial cells. Moreover, since the virus can switch between a latent and a lytic life cycle, EBV has the ability to cause chronic relapsing/reactivating infections. Chronic or recurrent EBV infection of epithelial cells has been linked to systemic lupus erythematosus and Sjögren’s syndrome, whereas chronic/recurrent infection of B cells has been associated with rheumatoid arthritis, multiple sclerosis and other diseases. Accordingly, since EBV can shuttle between epithelial cells and B cells, the systemic autoimmune diseases often occur as overlapping syndromes with symptoms and characteristic autoantibodies (e.g. antinuclear antibodies and rheumatoid factors) reflecting epithelial and/or B cell infection.

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“…Complement also has an important role in the pathogenesis of LN. On the one hand, a deficiency of complement components predisposes to lupus, while on the other hand, excess complement activation increases renal damage, and measuring it is done to assess disease activity (Sharma et al 2020). Although OS involvement in renal injury being driven by complement activation seems obvious, to the best of our knowledge, to date there are no available data linking complement activation in GNs to PRDXs.…”

Section: Discussionmentioning

confidence: 99%

Peroxiredoxins as Markers of Oxidative Stress in IgA Nephropathy, Membranous Nephropathy and Lupus Nephritis

Krata

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Zagożdżon

et al. 2021

Arch. Immunol. Ther. Exp.

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IgA nephropathy (IgAN), membranous nephropathy (MN), and lupus nephritis (LN) represent important causes of chronic kidney disease. They belong to the immune-mediated glomerulonephritis (GNs), and have distinct pathogenesis, distinct clinical courses, and variable responses to treatment. Therefore, specific diagnostic procedures are necessary for more effective patient management. Recently, a role for oxidative stress has been proposed in various renal disorders. Thus, molecules related to oxidative stress, such as 2-Cys-peroxiredoxins (PRDXs), may represent plausible candidates for biomarkers in renal pathologies. The aim of this study was to assess whether there are differences between individual GNs and healthy controls in the context of PRDXs serum concentration. We enrolled 108 patients with biopsy-proven IgAN (47), MN (26), LN (35) and 30 healthy age- and sex-matched controls. The serum concentrations of PRDX 1–5 were measured with ELISA assays and correlated with demographic and clinical data. The PRDXs’ concentration varied depending on the GN type. We also observed an association of PRDXs with lower estimated glomerular filtration rates, complement, hemoglobin, and body mass index. Our study indicates that individual PRDX can play roles in pathophysiology of selected GNs and that their serum concentrations may become useful as a new supplementary diagnostic markers in IgAN, MN as well as LN. The results of this study open a new avenue for prospective research on PRDXs in renal diseases.

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Revisiting the complement system in systemic lupus erythematosus

Cited by 28 publications

References 98 publications

Primary Immunodeficiency in Children With Autoimmune Cytopenias: Retrospective 154-Patient Cohort

Primary Immunodeficiency in Children With Autoimmune Cytopenias: Retrospective 154-Patient Cohort

Epstein-Barr Virus and Systemic Autoimmune Diseases

Peroxiredoxins as Markers of Oxidative Stress in IgA Nephropathy, Membranous Nephropathy and Lupus Nephritis

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