We Need Better Classification and Terminology for “People at High Risk of or in the Process of Developing Lupus”

Costenbader, Karen H.; Schur, Peter H.

doi:10.1002/acr.22484

Cited by 36 publications

(33 citation statements)

References 23 publications

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“…This evaluation requires a trained rheumatologist and may miss more subtle signs and symptoms that result in a clinician identifying a patient as having “potential SLE” (41). Screening families of lupus patients with the SLE-CSQ and serology may substantially facilitate identifying relatives who are at increased risk of disease compared to relatives who do not require enhanced monitoring or treatment with potentially toxic medications.…”

Section: Discussionmentioning

confidence: 99%

Discerning Risk of Disease Transition in Relatives of Systemic Lupus Erythematosus Patients Utilizing Soluble Mediators and Clinical Features

Munroe

Young

Kamen

et al. 2017

Arthritis & Rheumatology

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Objective Systemic lupus erythematosus (SLE) and other autoimmune diseases cause significant morbidity. Identifying populations at risk of developing SLE is essential to curtail irreversible inflammatory damage. The objective of this study was to identify factors associated with transition to classified disease that inform SLE risk. Methods Previously identified lupus patient blood relatives with < 4 American College of Rheumatology SLE classification criteria at baseline (n=409) were enrolled in this follow-up study. Participants provided detailed family, demographic, and clinical information, including the SLE-specific portion of the Connective Tissue Disease Screening Questionnaire (SLE-CSQ). Plasma samples were tested for the presence of lupus-associated autoantibodies and 52 soluble mediators. Generalized estimating equations (GEE) were applied to identify factors anticipating disease transition. Results Forty-five relatives (11%) transitioned to classified SLE during follow-up (mean time=6.4 years). Relatives who transitioned displayed more lupus-associated autoantibody specificities and higher SLE-CSQ scores (p<0.0001) at baseline than non-transitioned relatives. Importantly, they also had elevated baseline plasma levels of inflammatory mediators, including B-lymphocyte stimulator (BLyS), stem cell factor (SCF), and interferon-associated chemokines (p≤0.02), with concurrent decreases in levels of regulatory mediators, tumor growth factor (TGF)-β and interleukin (IL)-10 (p≤0.03). GEE revealed that baseline SLE-CSQ or ACR scores and plasma levels of SCF and TGF-β (p≤0.03), but not autoantibodies, were significant and independent predictors of SLE transition. Conclusions Altered levels of soluble mediators anticipate transition to classified disease in lupus relatives. Thus, immune perturbations precede SLE classification and can help identify high-risk relatives for rheumatology referral and potential enrollment in prevention trials.

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Section: Discussionmentioning

confidence: 99%

Discerning Risk of Disease Transition in Relatives of Systemic Lupus Erythematosus Patients Utilizing Soluble Mediators and Clinical Features

Munroe

Young

Kamen

et al. 2017

Arthritis & Rheumatology

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“…The aim is to develop classification criteria with specificity comparable with the ACR 1997 criteria, but with improved sensitivity, in early disease in particular. 30 In addition, we would like to arrive at a more intuitive approach that is easier to learn. It is important that the criteria approach rigorously keeps to scientific methods, and that circular reasoning is avoided.…”

Section: A Process Towards New Classification Criteriamentioning

confidence: 99%

Toward new criteria for systemic lupus erythematosus—a standpoint

Aringer

Dörner

Leuchten

et al. 2016

Lupus

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While clearly different in their aims and means, classification and diagnosis both try to accurately label the disease patients are suffering from. For systemic lupus erythematosus (SLE), this is complicated by the multi-organ nature of the disease and by our incomplete understanding of its pathophysiology. Hallmarks of SLE are the presence of antinuclear antibodies (ANA), and multiple immune-mediated organ symptoms that are largely independent. In an attempt to overcome limitations of the current sets of SLE classification criteria, a new fourphase approach is being developed, which is jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). This review attempts to delineate the performance of the current sets of criteria, the reasons for the decision for classification, and not diagnostic, criteria, and to provide a background of the current approach taken. Lupus (2016) 25, 805-811.

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“…At least one study from a university-based dermatology clinic suggests that some patients who present with an incomplete and largely cutaneous symptom complex do progress to SLE but have a low risk of developing organ-damaging manifestations [3]. But concerns also have been raised about whether overuse of the ILE terminology is inappropriate in view of the fact that many will never develop SLE or another significant illness [4, 5]. Determining which ILE patients are at greatest risk has some urgency, as it will open possibilities for interventional trials to modify disease and reduce progression to SLE.…”

Section: Introductionmentioning

confidence: 99%

Clinical and Immunologic Profiles in Incomplete Lupus Erythematosus and Improvement with Hydroxychloroquine Treatment

Olsen

McAloose

Carter

et al. 2016

Autoimmune Diseases

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Objective. The study goals were to evaluate performance of SLE classification criteria, to define patients with incomplete lupus erythematosus (ILE), and to probe for features in these patients that might be useful as indicators of disease status and hydroxychloroquine response. Methods. Patients with ILE (N = 70) and SLE (N = 32) defined by the 1997 American College of Rheumatology criteria were reclassified using the 2012 Systemic Lupus International Collaborating Clinics criteria. Disease activity, patient reported outcomes, and levels of Type I interferon- (IFN-) inducible genes, autoantibodies, and cytokines were measured. Subgroups treated with hydroxychloroquine (HCQ) were compared to patients not on this drug. Results. The classification sets were correlated (R2 = 0.87). ILE patients were older (P = 0.0043) with lower disease activity scores (P < 0.001) and greater dissatisfaction with health status (P = 0.034) than SLE patients. ILE was associated with lower levels of macrophage-derived cytokines and levels of expressed Type I IFN-inducible genes. Treatment of ILE with HCQ was associated with better self-reported health status scores and lower expression levels of Type I IFN-inducible genes than ILE patients not on HCQ. Conclusion. The 2012 SLICC SLE classification criteria will be useful to define ILE in trials. Patients with ILE have better health status and immune profiles when treated with HCQ.

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We Need Better Classification and Terminology for “People at High Risk of or in the Process of Developing Lupus”

Cited by 36 publications

References 23 publications

Discerning Risk of Disease Transition in Relatives of Systemic Lupus Erythematosus Patients Utilizing Soluble Mediators and Clinical Features

Discerning Risk of Disease Transition in Relatives of Systemic Lupus Erythematosus Patients Utilizing Soluble Mediators and Clinical Features

Toward new criteria for systemic lupus erythematosus—a standpoint

Clinical and Immunologic Profiles in Incomplete Lupus Erythematosus and Improvement with Hydroxychloroquine Treatment

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