Abstract

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.

Highlights

  • A significant number of individuals seeking care in rheumatology clinics have some findings suggestive of SLE but do not have 4 of the defined classification criteria

  • The results suggest that this treatment has benefits in incomplete lupus erythematosus (ILE) and potential to modulate long-term outcome in these patients

  • Patients with lupus syndromes were identified during routine care in the clinics of the rheumatology division

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Summary

Introduction

A significant number of individuals seeking care in rheumatology clinics have some findings suggestive of SLE but do not have 4 of the defined classification criteria. A patient with significant ANA positivity and a photosensitive malar rash, for example, does not fit classification criteria for SLE, but the findings may be of concern and warrant further evaluations or treatment. This is especially true for young females, who are in the highest risk group for development of SLE. 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]. 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

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