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Lupus
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research-article

Patients with familial and sporadic onset SLE have similar clinical profiles but vary profoundly by race

AL Sestak

Department of Arthritis and Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA Andrea-Sestak{at}omrf.ouhsc.edu

SK Nath

Department of Arthritis and Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA; Department of Pediatrics at the University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA

JA Kelly

Department of Arthritis and Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA

GR Bruner

Department of Arthritis and Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA

JA James

Department of Arthritis and Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA; Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA

JB Harley

Department of Arthritis and Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA; Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA; United States Department of Veterans Affairs Medical Center, Oklahoma City, Oklahoma, USA

Few large, multi-ethnic studies have examined the clinical and serologic differences between familial and sporadic SLE patients. Understanding these similarities and differences is critical for interpreting genetic studies and developing therapeutic strategies. We compiled information on 1915 patients with SLE in a large multi-racial cohort, including general demographics, pedigree structure and the specific American College of Rheumatology (ACR) criteria met. One patient was randomly selected from each multiplex family for analysis, yielding 554 European-Americans (EA), 373 African-Americans (AA), 193 Hispanics (HI) and 237 patients of other of mixed races. When comparing familial and sporadic patients stratified by race, lupus erythematosus (LE) cells and arthritis were increased in white familial cases (P = 5.5 x 10–6 and P = 0.028, respectively), but no other significant differences between familial and sporadic patients were found. We found that there were profound differences in clinical profiles between races. For example, photosensitivity and malar rash were decreased in AA (P = 1.3 x 10–13 and 1.4 x 10–7, respectively), whereas discoid rash was increased in AA (P = 5.5x10–6). EA had significantly less renal disease (P = 5.4x10–13), proteinuria (P = 4 x 10–12) and anti-Sm (P = 1.7 x 10–12) than AA or HI. We, therefore, conclude that familial and sporadic onset patients may be treated similarly with respect to clinical and genetic studies.

Key Words: SLE • familial • sporadic • gender • race • clinical profile

Lupus, Vol. 17, No. 11, 1004-1009 (2008)
DOI: 10.1177/0961203308091969


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