Clinical and Serologic Features in Patients With Incomplete Lupus Classification Versus Systemic Lupus Erythematosus Patients and Controls

Abstract

Objective Incomplete lupus erythematosus (ILE) involves clinical and/or serologic manifestations consistent with but insufficient for SLE classification. Because the nature of ILE is poorly understood and no treatment recommendations exist, we examined clinical manifestations, medication history, and immunologic features in a diverse collection of ILE and SLE patients. Methods Medical records of subjects enrolled to the Lupus Family Registry and Repository were reviewed for medication history and American College of Rheumatology (ACR) classification criteria to identify ILE patients (3 ACR criteria; n=440) and SLE patients (≥4 ACR criteria; n=3,397). Participants completed the connective tissue disease screening questionnaire (CSQ). Anti-cardiolipin and plasma BLyS were measured by ELISA, anti-nuclear antibodies by indirect immunofluorescence, and 13 autoantibodies by bead-based assays. Results ILE patients were older than SLE patients (46.2 vs. 42.0 y, P<0.0001), and fewer ILE patients were African American (23.9% vs. 32.2%, P<0.001). ILE patients exhibited fewer autoantibody specificities (1.3 vs. 2.6, P<0.0001) than SLE patients and were less likely to have ANA titers ≥1:1080 (10.5% vs. 19.5%,P<0.0001). BLyS levels were intermediate in ILE patients (controls<ILE, P=0.016; ILE<SLE, P=0.008). Pericarditis, renal, or neurologic manifestations occurred in 12.5% of ILE patients and associated with non-European American race/ethnicity (P=0.012). Hydroxychloroquine use increased over time, but was less frequent in ILE than SLE patients (65.2% vs. 83.1%, P<0.0001). Conclusion Although usually characterized by milder symptoms, ILE manifestations may require immunomodulatory treatments. Longitudinal studies are necessary to understand how ILE impacts organ damage and future SLE risk, and to delineate molecular pathways unique to ILE.

Complement Activation in Patients With Probable Systemic Lupus Erythematosus and Ability to Predict Progression to American College of Rheumatology–Classified Systemic Lupus Erythematosus

“…Consistent with findings from other studies (12,(22)(23)(24), the most common ACR clinical criteria fulfilled by patients with SLE and patients with probable SLE were arthritis (77% and 50%, respectively) and hematologic disorder (72% and 41%), while the most common SLICC clinical criterion was synovitis (75% and 47%). Patients with SLE had more mucosal ulceration, serositis, hematologic features, and immunologic features than patients with probable SLE.…”

Pathogenesis of Human Systemic Lupus Erythematosus: A Cellular Perspective

“…These studies support the concept of a gradual chronological disease progression, from a preclinical asymptomatic phase, to incomplete lupus erythematosus, and finally, to complete lupus erythematosus [149]. Clinical and serologic parameters distinguish patients with incomplete lupus from SLE patients, and identification of these parameters is crucial for early management of disease [150–152]. Consequently, tailoring management based on these early identifications has the potential to significantly improve patient outcomes and reduce healthcare costs.…”

How Do We Classify “Incomplete Lupus?”

“…The study by Aberle et al in the current issue emphasizes and extends these prior observations . The investigators utilized records of subjects enrolled in a large, geographically and racially/ethnically diverse lupus family registry to compare clinical and immunologic features of 440 ILE subjects (3 criteria) with 3,397 subjects classified as SLE (≥4 criteria).…”

“…In the study by Aberle et al, in this issue of Arthritis Care & Research , the authors studied a group of “incomplete” lupus erythematosus (ILE) patients, defined in the context of the 1997 updated ACR classification criteria for SLE . They compared patients with 3 of the current ACR criteria, thus not meeting the high threshold for definition of SLE for the purpose of clinical studies, with patients with ≥4 items from the ACR Classification Criteria.…”