ReviewJournal of clinical immunology2008
Understanding systemic lupus erythematosus physiopathology in the light of primary immunodeficiencies.
Review in Journal of clinical immunology, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
22 citing papers in PubMed, 1 synthesis or guideline pooled it, 97 citations in OpenAlex.
- Association among Complement Factor H Autoantibodies, Deletions of CFHR, and the Risk of Atypical Hemolytic Uremic Syndrome.International journal of environmental research and public health · 2016Pooled it
- Editorial: Autoimmune diseases in childhood.Frontiers in immunology · 2024Article
- Reduced expressions of apoptosis-related proteins TRAIL, Bcl-2, and TNFR1 in NK cells of juvenile-onset systemic lupus erythematosus patients: relations with disease activity, nephritis, and neuropsychiatric involvement.Frontiers in immunology · 2024Article
- The complement system and human autoimmune diseases.Journal of autoimmunity · 2023Review
- Vasculitis and vasculopathy associated with inborn errors of immunity: an overview.Frontiers in pediatrics · 2023Review
- [Systemic lupus erythematosus-are children small adults?]Zeitschrift fur Rheumatologie · 2022Review
- A rare association between factor H deficiency and lupus: Case report and experimental treatment with curcumin.Frontiers in pediatrics · 2022Article
- Phenotypic characterization of patients with activated PI3Kδ syndrome 1 presenting with features of systemic lupus erythematosus.Genes & diseases · 2021Article
- Disease presentation of 1312 childhood-onset systemic lupus erythematosus: influence of ethnicity.Clinical rheumatology · 2019Article
- Complement C4 Prevents Viral Infection through Capsid Inactivation.Cell host & microbe · 2019Article
- Complementing the Cancer-Immunity Cycle.Frontiers in immunology · 2019Review
- Reply.Arthritis & rheumatology (Hoboken, N.J.) · 2018Article
- Increased serum sFas, sTRAIL, and reduced sFasL in juvenile-onset systemic lupus erythematosus.Clinical rheumatology · 2017Article
- Possible evidence of systemic lupus erythematosus and periodontal disease association mediated by Toll-like receptors 2 and 4.Clinical and experimental immunology · 2016Review
- Molecular characterization of the complement C1q, C2 and C4 genes in Brazilian patients with juvenile systemic lupus erythematosus.Clinics (Sao Paulo, Brazil) · 2015Article
- Early-onset autoimmune disease as a manifestation of primary immunodeficiency.Frontiers in immunology · 2015Review
- Increased IgE serum levels are unrelated to allergic and parasitic diseases in patients with juvenile systemic lupus erythematosus.Clinics (Sao Paulo, Brazil) · 2012Article
- Familial juvenile systemic lupus erythematosus in Arab children.Rheumatology international · 2012Article
- Systemic lupus erythematosus as a first presentation of common variable immunodeficiency associated with infrequent mannose-binding lectin gene polymorphisms.Rheumatology international · 2011Article
- Common variable immunodeficiency associated with hepatosplenic T-cell lymphoma mimicking juvenile systemic lupus erythematosus.Clinical & developmental immunology · 2011Article
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionAssociations between systemic lupus erythematosus (SLE) and primary immunodeficiencies (PIDs) were analyzed to gain insight into the physiopathology of SLE. Some PIDs have been consistently associated with SLE or lupus-like manifestations: (a) homozygous deficiencies of the early components of the classical complement pathway in the following decreasing order: in C1q, 93% of affected patients developed SLE; in C4, 75%; in C1r/s, 57%; and in C2, up to 25%; (b) female carriers of X-linked chronic granulomatous disease allele; and (c) IgA deficiency, present in around 5% of juvenile SLE. DISCUSSION: In the first two groups, disturbances of cellular waste-disposal have been proposed as the main mechanisms of pathogenesis. On the other hand and very interestingly, there are PIDs systematically associated with several autoimmune manifestations in which SLE has not been described, such as autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED), immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX), and autoimmune lymphoproliferative syndrome (ALPS), suggesting that mechanisms considered as critical players for induction and maintenance of tolerance to autoantigens, such as (1) AIRE-mediated thymic negative selection of lymphocytes, (2) Foxp3+ regulatory T cell-mediated peripheral tolerance, and (3) deletion of auto-reactive lymphocytes by Fas-mediated apoptosis, could not be relevant in SLE physiopathology. The non-description of SLE and neither the most characteristic SLE clinical features among patients with agammaglobulinemia are also interesting observations, which reinforce the essential role of B lymphocytes and antibodies for SLE pathogenesis.
conclusionTherefore, monogenic PIDs represent unique and not fully explored human models for unraveling components of the conundrum represented by the physiopathology of SLE, a prototypical polygenic disease.
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