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

• Published in: Arthritis care & research (2010) Vol. 69; no. 12; pp. 1780 - 1788
• Main Authors: Aberle, Teresa, Bourn, Rebecka L., Munroe, Melissa E., Chen, Hua, Roberts, Virginia C., Guthridge, Joel M., Bean, Krista, Robertson, Julie M., Sivils, Kathy L., Rasmussen, Astrid, Liles, Meghan, Merrill, Joan T., Harley, John B., Olsen, Nancy J., Karp, David R., James, Judith A.
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.12.2017
• Subjects: Adult; Aged; Aged, 80 and over; Antibodies, Anticardiolipin - blood; Antinuclear antibodies; Antirheumatic Agents - therapeutic use; Autoantibodies; B-Cell Activating Factor - immunology; Biomarkers - blood; BLyS protein; British Virgin Islands; Cardiolipin; Case-Control Studies; Classification; Continental Population Groups; Enzyme-Linked Immunosorbent Assay; Ethnic Groups; Female; Fluorescent Antibody Technique, Indirect; Humans; Hydroxychloroquine; Hydroxychloroquine - therapeutic use; Immunofluorescence; Immunomodulation; Kidneys; Lupus; Lupus Erythematosus, Systemic - blood; Lupus Erythematosus, Systemic - classification; Lupus Erythematosus, Systemic - diagnosis; Lupus Erythematosus, Systemic - immunology; Lymphocytes B; Male; Medical records; Middle Aged; Minority & ethnic groups; Pericarditis; Predictive Value of Tests; Puerto Rico; Registries; Serologic Tests; Severity of Illness Index; Surveys and Questionnaires; Systemic lupus erythematosus; Terminology as Topic; United States; United States Virgin Islands
• ISSN: 2151-464X; 2151-4658
• DOI: 10.1002/acr.23201

Objective Incomplete lupus erythematosus (ILE) involves clinical and/or serologic manifestations consistent with but insufficient for systemic lupus erythematosus (SLE) classification. Because the nature of ILE is poorly understood and no treatment recommendations exist, we examined the clinical manifestations, medication history, and immunologic features in a diverse collection of ILE and SLE patients. Methods Medical records of subjects enrolled in 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. Anticardiolipin and plasma B lymphocyte stimulator (BLyS) were measured by enzyme‐linked immunosorbent assay, antinuclear antibodies (ANAs) by indirect immunofluorescence, and 13 autoantibodies by bead‐based assays. Results On average, ILE patients were older than SLE patients (46.2 years versus 42.0 years; P < 0.0001), and fewer ILE patients were African American (23.9% versus 32.2%; P < 0.001). ILE patients exhibited fewer autoantibody specificities than SLE patients (1.3 versus 2.6; P < 0.0001) and were less likely to have ANA titers ≥1:1,080 (10.5% versus 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 were 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% versus 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 affects organ damage and future SLE risk, and to delineate molecular pathways unique to ILE.