title: Clinical and Serologic Features in Patients With Incomplete Lupus Classification Versus Systemic Lupus Erythematosus Patients and Controls.
abstract: 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.
  
  
  
  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.
  
  
  
  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).
  
  
  
  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.
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Teresa
authors.initials: T
authors.lastname: Aberle
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Rebecka L
authors.initials: RL
authors.lastname: Bourn
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Melissa E
authors.initials: ME
authors.lastname: Munroe
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Hua
authors.initials: H
authors.lastname: Chen
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Virginia C
authors.initials: VC
authors.lastname: Roberts
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Joel M
authors.initials: JM
authors.lastname: Guthridge
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Krista
authors.initials: K
authors.lastname: Bean
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Julie M
authors.initials: JM
authors.lastname: Robertson
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Kathy L
authors.initials: KL
authors.lastname: Sivils
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Astrid
authors.initials: A
authors.lastname: Rasmussen
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Meghan
authors.initials: M
authors.lastname: Liles
authors.affiliation: Oklahoma Medical Research Foundation, Oklahoma City.
authors.forename: Joan T
authors.initials: JT
authors.lastname: Merrill
authors.affiliation: Cincinnati Children's Hospital Medical Center and US Department of Veterans Affairs Medical Center, Cincinnati, Ohio.
authors.forename: John B
authors.initials: JB
authors.lastname: Harley
authors.affiliation: Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania.
authors.forename: Nancy J
authors.initials: NJ
authors.lastname: Olsen
authors.affiliation: University of Texas Southwestern Medical Center, Dallas.
authors.forename: David R
authors.initials: DR
authors.lastname: Karp
authors.affiliation: Oklahoma Medical Research Foundation and University of Oklahoma Health Sciences Center, Oklahoma City.
authors.forename: Judith A
authors.initials: JA
authors.lastname: James
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017153:Antibodies, Anticardiolipin / Q000097:blood*; D018501:Antirheumatic Agents / Q000627:therapeutic use; D053264:B-Cell Activating Factor / Q000276:immunology*; D015415:Biomarkers / Q000097:blood; D064966:British Virgin Islands; D016022:Case-Control Studies; D004797:Enzyme-Linked Immunosorbent Assay; D005006:Ethnicity; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000145:classification* / Q000175:diagnosis* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011647:Puerto Rico; D044469:Racial Groups; D012042:Registries; D012698:Serologic Tests; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D009626:Terminology as Topic; D014481:United States; D014767:United States Virgin Islands
chemical_list: D017153:Antibodies, Anticardiolipin; D018501:Antirheumatic Agents; D053264:B-Cell Activating Factor; D015415:Biomarkers; C505719:TNFSF13B protein, human; D006886:Hydroxychloroquine