Understanding Systemic Lupus Erythematosus Physiopathology in the Light of Primary Immunodeficiencies

Carneiro‐Sampaio, Magda; Liphaus, Bernadete L.; Jesús, Adriana Almeida de; Silva, Clóvis A.; Oliveira, João Bosco; Kiss, Maria Augusta P. D.

doi:10.1007/s10875-008-9187-2

Cited by 76 publications

(69 citation statements)

References 46 publications

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“…Correlation between SLE and periodontal disease SLE is characterized by hyperactivation of B and T cells, increased production of immunoglobulin G [49] and deposition of immune complexes, which are formed by autoantibodies to nuclear antigens into tissues, thus leading to their destruction [50]. Common symptoms in the oral cavity include the appearance of blazing, xerostomia, candidiasis [51], gingivitis and periodontal disease [52].…”

Section: Periodontal Diseasementioning

confidence: 99%

Possible evidence of systemic lupus erythematosus and periodontal disease association mediated by Toll-like receptors 2 and 4

Marques

Maor

Andrade

et al. 2015

Clinical and Experimental Immunology

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Summary Toll‐like receptors (TLRs) participate in the innate immune response and trigger the immune responses of the body. Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown aetiology, characterized by an excessive autoimmune response in the body affecting the connective tissues. The disease is possibly triggered by both environmental aetiological factors and pathological organic processes such as exposure to sunlight, chronic infectious processes and genetic factors. Conversely, periodontal disease is an infectious disease caused by microorganisms in the oral cavity, resulting in a chronic inflammatory process which continuously stimulates the immune response, thus causing damage to the periodontal tissues. The expression of both TLR‐2 and TLR‐4 receptors are increased in both SLE and periodontal disease. Periodontitis might trigger excessive activation of immune response occurring in SLE by maintaining a high expression of TLRs, leading in turn to the acceleration of the onset and progression of autoimmune reactions. In addition, periodontal treatment is able to reduce the expression of these receptors and therefore the symptoms of SLE. Here we discuss the possible interaction between SLE and periodontitis, and suggest further studies evaluating common features in both factors that could explored, due to morbidity and mortality of SLE and the high incidence of periodontal infections around the world.

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Section: Periodontal Diseasementioning

confidence: 99%

Possible evidence of systemic lupus erythematosus and periodontal disease association mediated by Toll-like receptors 2 and 4

Marques

Maor

Andrade

et al. 2015

Clinical and Experimental Immunology

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“…There is a hierarchy of prevalence and disease severity according to the position of the protein in the classical pathway. The most prevalent and most severe disease is associated with deficiency of the proteins of the C1 complex and with total C4 deficiency; almost every human with C1q deficiency (474), over 75% of all individuals with total C4 deficiency, and 57% of individuals with C1r/s deficiency have SLE (54,492), often with severe clinical manifestations. In contrast, C2 deficiency is associated with a much lower prevalence of disease, estimated at ϳ10%.…”

Section: Inherited Deficiencies Of Complement Activationmentioning

confidence: 99%

Infections of People with Complement Deficiencies and Patients Who Have Undergone Splenectomy

2010

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SUMMARY The complement system comprises several fluid-phase and membrane-associated proteins. Under physiological conditions, activation of the fluid-phase components of complement is maintained under tight control and complement activation occurs primarily on surfaces recognized as “nonself” in an attempt to minimize damage to bystander host cells. Membrane complement components act to limit complement activation on host cells or to facilitate uptake of antigens or microbes “tagged” with complement fragments. While this review focuses on the role of complement in infectious diseases, work over the past couple of decades has defined several important functions of complement distinct from that of combating infections. Activation of complement in the fluid phase can occur through the classical, lectin, or alternative pathway. Deficiencies of components of the classical pathway lead to the development of autoimmune disorders and predispose individuals to recurrent respiratory infections and infections caused by encapsulated organisms, including Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae. While no individual with complete mannan-binding lectin (MBL) deficiency has been identified, low MBL levels have been linked to predisposition to, or severity of, several diseases. It appears that MBL may play an important role in children, who have a relatively immature adaptive immune response. C3 is the point at which all complement pathways converge, and complete deficiency of C3 invariably leads to severe infections, including those caused by meningococci and pneumococci. Deficiencies of the alternative and terminal complement pathways result in an almost exclusive predisposition to invasive meningococcal disease. The spleen plays an important role in antigen processing and the production of antibodies. Splenic macrophages are critical in clearing opsonized encapsulated bacteria (such as pneumococci, meningococci, and Escherichia coli) and intraerythrocytic parasites such as those causing malaria and babesiosis, which explains the fulminant nature of these infections in persons with anatomic or functional asplenia. Paramount to the management of patients with complement deficiencies and asplenia is educating patients about their predisposition to infection and the importance of preventive immunizations and seeking prompt medical attention.

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“…However, a complex disease like SLE involves multiple, interacting genetic and environmental determinants, which makes identifying candidate genes challenging and with limited success to date [19][20][21][22]. The strongest risk is conferred by genetic defects of the early complement components namely C1q and C4 and to a lesser extent C2 [23][24][25][26][27].…”

Section: S Lementioning

confidence: 99%

Familial juvenile systemic lupus erythematosus in Arab children

Al‐Mayouf¹,

Abdwani²,

Al-Brawi³

2011

Rheumatol Int

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A ten-year retrospective analysis of the clinical features and survival of 60 Saudi children with systemic lupus erythematosus (SLE) was made. All the patients fulfilled the 1982 American College of Rheumatology's revised criteria for SLE and had had the disease at or before the age of 16 years. The female to male ratio was 5:1, the mean age of onset was 12.1 years (range 1.6-16 years), and the mean duration of follow-up was 4.7 years (range 2.2-11). Thirty-eight patients (63%) were diagnosed correctly before referral to KFSH&RC or KKUH. The mode of presentation was as follows: 55 patients had musculoskeletal involvement (91.6%), 49 patients had skin involvement (81.6%), 40 patients had hematological abnormalities (66.6%), 39 patients had renal disease (65%), 10 patients had pulmonary involvement (16%), 23 patients had cardiovascular disease (38%) and 18 patients had central nervous system involvement. During the study period four patients died (6.6%)-two of renal failure, one from meningitis and one from severe sepsis. This is the largest collection of childhood systemic lupus erythematosus from the Middle East and it shows that SLE is more common in Saudis than was hitherto believed, and that it has a high rate of organ involvement.

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Understanding Systemic Lupus Erythematosus Physiopathology in the Light of Primary Immunodeficiencies

Abstract: Therefore, 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.

Cited by 76 publications

References 46 publications

Possible evidence of systemic lupus erythematosus and periodontal disease association mediated by Toll-like receptors 2 and 4

Possible evidence of systemic lupus erythematosus and periodontal disease association mediated by Toll-like receptors 2 and 4

Infections of People with Complement Deficiencies and Patients Who Have Undergone Splenectomy

Familial juvenile systemic lupus erythematosus in Arab children

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