@article{c6100525ed9a45e2b28b29020c99fd14,
title = "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 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.",
author = "Teresa Aberle and Bourn, {Rebecka L.} and Munroe, {Melissa E.} and Hua Chen and Roberts, {Virginia C.} and Guthridge, {Joel M.} and Krista Bean and Robertson, {Julie M.} and Sivils, {Kathy L.} and Astrid Rasmussen and Meghan Liles and Merrill, {Joan T.} and Harley, {John B.} and Olsen, {Nancy J.} and Karp, {David R.} and James, {Judith A.}",
note = "Funding Information: 1Teresa Aberle, PA, Rebecka L. Bourn, PhD, Melissa E. Munroe, MD, PhD, Hua Chen, PhD, Virginia C. Roberts, LPN, Joel M. Guthridge, PhD, Krista Bean, BS, Julie M. Robertson, PhD, Kathy L. Sivils, PhD, Astrid Rasmussen, MD, PhD, Meghan Liles, MS, Joan T. Merrill, MD: Oklahoma Medical Research Foundation, Oklahoma City; 2John B. Harley, MD, PhD: Center for Autoimmune Genomics and Etiology, Cincinnati Children{\textquoteright}s Hospital Medical Center, and US Department of Veterans Affairs Medical Center, Cincinnati, Ohio; 3Nancy J. Olsen, MD: Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania; Funding Information: of Diabetes and Digestive and Kidney Diseases (grant R01-DK-107502), and by the US Department of Veterans Affairs (grant I01-BX-001834). Funding Information: Supported by the NIH through the National Institute of Allergy and Infectious Disease (grants U19-AI-082714, U01-AI-101934, and R37-AI-24717), Institutional Development Awards from the National Institute of General Medical Sciences (grants P30-GM-103510 and U54-GM-104938), the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grants P30-AR-053483 and P30-AR-070549), the National Human Genome Research Institute (grant U01-HG-008666), the National Heart, Lung, and Blood Institute (grant R24-HL-105333), and the National Institute Funding Information: 4David R. Karp, MD, PhD: University of Texas Southwestern Medical Center, Dallas; 5Judith A. James, MD, PhD: Oklahoma Medical Research Foundation and University of Oklahoma Health Sciences Center, Oklahoma City. Funding Information: Address correspondence to Judith A. James, MD, PhD, Arthritis & Clinical Immunology, Oklahoma Medical Research Foundation, 825 NE Thirteenth Street, Oklahoma City, OK 73104. E-mail: judith-james@omrf.org. Publisher Copyright: {\textcopyright} 2017, American College of Rheumatology",
year = "2017",
month = dec,
doi = "10.1002/acr.23201",
language = "English (US)",
volume = "69",
pages = "1780--1788",
journal = "Arthritis Care and Research",
issn = "2151-464X",
number = "12",
}