Incidence and ClinicaI Significance of Anti-ENA Antibodies in Systemic Lupus Erythematosus:<i>Estimation by Counterimmunoelectrophoresis</i>

Clotet, B; Guàrdia, J; Pigrau, Carles; Lience, E; Murcia, Clara Manso; Pujol, Roser; Bacardí, R

doi:10.3109/03009748409102662

Cited by 26 publications

(6 citation statements)

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“…In the context of other research regarding associated features of myositis overlap, our findings of the tendency of myositis overlap patients to have anti-RNP positive, renal sparing SLE is consistent with the current literature [18]. Additionally, the rate of anti-Sm antibodies in our cohort was 69%, which is quite high compared to the reported prevalence of 30% in North America [24]. This could be secondary to the fact that our population is largely African-American, and the literature demonstrates that anti-Sm antibodies are known to be more prevalent in African-Americans [24-26].…”

Section: Discussionsupporting

confidence: 92%

“…Additionally, the rate of anti-Sm antibodies in our cohort was 69%, which is quite high compared to the reported prevalence of 30% in North America [24]. This could be secondary to the fact that our population is largely African-American, and the literature demonstrates that anti-Sm antibodies are known to be more prevalent in African-Americans [24-26]. …”

Section: Discussionmentioning

confidence: 52%

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High prevalence of myositis in a southeastern United States pediatric systemic lupus erythematosus cohort

2011

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Inflammatory myositis is reported in 4-16% of adult systemic lupus erythematosus (SLE) patients. The aim of this study was to determine the prevalence of myositis in a cohort of pediatric SLE patients in the southeastern United States. A retrospective chart review was performed of 55 SLE patients evaluated by Pediatric Rheumatologists in Alabama since January 1, 2008. Patients were defined as having myositis if they satisfied one of the following categories: 1) Proximal muscle weakness on exam with lower extremity muscle edema on MRI; 2) Proximal muscle weakness with elevation in CK, AST, aldolase, or LDH muscle enzymes; or 3) Patient reported weakness or muscle pain and an elevated CK. Inflammatory myositis was present as a feature of SLE in 31% (n = 17) with a 95% confidence interval of 19-45%, statistically different from the reported rates of 4-16% (p < 0.0001). Myositis was positively associated with the presence of anti-ribonucleoprotein antibodies (p = 0.009). Negative associations with myositis were the presence of anti-double stranded DNA antibodies (p = 0.02) and hematologic disorders (p = 0.02). Thus, in the state of Alabama, pediatric SLE myositis is present at a statistically higher rate than previously published values of adult SLE myositis, possibly reflecting geographic (genetic or environmental) and/or age-of-onset related influence(s).

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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 52%

High prevalence of myositis in a southeastern United States pediatric systemic lupus erythematosus cohort

2011

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“…Approximately 25% of SLE patients produce antibodies against the Sm proteins of the spliceosome, particularly autoantibodies to the B/B' proteins [10], [11]. The related anti-nRNP antibodies, directed against nRNP 70 K, nRNP A, and nRNP C, are more prevalent but less specific for SLE [12]. Antibodies against the Ro autoantigen are present in approximately 50% sera from SLE patients [13], though even less specific, and generally bind a 60 kD Ro protein with many also binding a 52 kD Ro moiety.…”

Section: Introductionmentioning

confidence: 99%

60 kD Ro and nRNP A Frequently Initiate Human Lupus Autoimmunity

et al. 2010

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Systemic lupus erythematosus (SLE) is a clinically heterogeneous, humoral autoimmune disorder. The unifying feature among SLE patients is the production of large quantities of autoantibodies. Serum samples from 129 patients collected before the onset of SLE and while in the United States military were evaluated for early pre-clinical serologic events. The first available positive serum sample frequently already contained multiple autoantibody specificities (65%). However, in 34 SLE patients the earliest pre-clinical serum sample positive for any detectable common autoantibody bound only a single autoantigen, most commonly 60 kD Ro (29%), nRNP A (24%), anti-phospholipids (18%) or rheumatoid factor (15%). We identified several recurrent patterns of autoantibody onset using these pre-diagnostic samples. In the serum samples available, anti-nRNP A appeared before or simultaneously with anti-nRNP 70 K in 96% of the patients who had both autoantibodies at diagnosis. Anti-60 kD Ro antibodies appeared before or simultaneously with anti-La (98%) or anti-52 kD Ro (95%). The autoantibody response in SLE patients begins simply, often binding a single specific autoantigen years before disease onset, followed by epitope spreading to additional autoantigenic specificities that are accrued in recurring patterns.

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“…In 1966, Sm was identified as a unique autoantigen, the first non-histone target of autoantibodies in systemic lupus erythematosus (SLE) patients [47]. Named for 15-year-old Stephanie Smith, antibodies to Sm became one of the diagnostic criteria for SLE [48], detectable in 5-30% of SLE patients [48,49]. Later studies correlated anti-Sm titers with kidney disease [50][51][52][53].…”

Section: A Short History Of Smmentioning

confidence: 99%

The regulation of autoreactive B cells during innate immune responses

Vilen

Rutan

2008

Immunol Res

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Systemic lupus erythematosus (SLE) highlights the dangers of dysregulated B cells and the importance of initiating and maintaining tolerance. In addition to central deletion, receptor editing, peripheral deletion, receptor revision, anergy, and indifference, we have described a new mechanism of B cell tolerance wherein dendritic cells (DCs) and macrophages (MΦs) regulate autoreactive B cells during innate immune responses. In part, DCs and MΦs repress autoreactive B cells by releasing IL-6 and soluble CD40L (sCD40L). This mechanism is selective in that IL-6 and sCD40L do not affect Ig secretion by naïve cells during innate immune responses, allowing immunity in the absence of autoimmunity. In lupus-prone mice, DCs and MΦs are defective in secretion of IL-6 and sCD40L and cannot effectively repress autoantibody secretion suggesting that defects in DC/MΦ-mediated tolerance may contribute to the autoimmune phenotype. Further, these studies suggest that reconstituting DCs and MΦs in SLE patients might restore regulation of autoreactive B cells and provide an alternative to immunosuppressive therapies. KeywordsSystemic lupus erythematosus; B cell tolerance; Autoimmunity; Dendritic cell; Macrophage; Smith antigen Learning tolerance: a B cell's storyA diverse B cell repertoire is critical in combating pathogens, but inherent in generating diversity is the threat of autoimmunity. In the bone marrow, central tolerance mechanisms such as deletion or receptor editing remove high-affinity autoreactive B cells before they exit to the periphery [1][2][3][4][5][6][7][8][9][10]. Those that escape are subject to receptor revision [8,[11][12][13][14], peripheral deletion [15,16], or a shortened lifespan because they fail to enter B cell follicles [17][18][19][20][21][22]. In rare cases, autoreactive B cells are fully functional but indifferent to their specific antigen [23][24][25]. Finally, many low-affinity autoreactive B cells are maintained in an unresponsive state known as anergy. Anergic B cells do not receive sufficient activation signals to differentiate into plasma cells or secrete immunoglobulin (Ig) in response to antigenic or mitogenic stimulation [26][27][28][29]. Their proliferative responses to B cell receptor (BCR) or toll-like receptor (TLR) signaling as well as their lifespans vary in different models [18,[30][31][32][33][34][35]. Some anergic B cells transduce BCR-derived signals [30, 32,[36][37][38][39][40], while others exhibit desensitized BCRs [30, 33,41]. Quiescence is dependent on chronic exposure to self-antigen and occupancy of the BCR [42,43] The affinity and avidity of the antigen-BCR interaction determines whether developing B cells will be deleted, edited, anergized, or ignored [46]. Self-reactive B cells that survive these developmental checkpoints tend to bind self-antigens with low affinity and they remain anergic in the absence of costimulation by cognate T cells. Many of the early transgenic (Tg) models expressed BCRs with high affinities. However, concerns were raised that these models...

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Incidence and ClinicaI Significance of Anti-ENA Antibodies in Systemic Lupus Erythematosus:Estimation by Counterimmunoelectrophoresis

Cited by 26 publications

References 13 publications

High prevalence of myositis in a southeastern United States pediatric systemic lupus erythematosus cohort

High prevalence of myositis in a southeastern United States pediatric systemic lupus erythematosus cohort

60 kD Ro and nRNP A Frequently Initiate Human Lupus Autoimmunity

The regulation of autoreactive B cells during innate immune responses

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