Affiliation:
1. From the Arthritis and Immunology Program, Oklahoma Medical Research Foundation, Oklahoma City; W. K. Warren Medical Research Institute, Oklahoma City; Department of Veterans Affairs Medical Center and the Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City.
Abstract
Abstract
Systemic lupus erythematosus (SLE) is a complicated autoimmune disease with a definite genetic predisposition. Thrombocytopenia predicts severe disease and death in SLE, making the identification of the related genetic risk factors especially important. We selected the 38 pedigrees that had an SLE patient with thrombocytopenia (platelets, < 10 × 109/L [< 100 000/μL]) from a collection of 184 pedigrees multiplex for SLE. Linkages were established at 1q22-23 (maximum logarithm of odds [lodmax] = 3.71) in the 38 pedigrees and at 11p13 (lodmax = 5.72) in the 13 African American pedigrees. Nephritis, serositis, neuropsychiatric involvement, autoimmune hemolytic anemia, anti–double-stranded DNA, and antiphospholipid antibody were associated with thrombocytopenia. Other results show that SLE is more severe in the families with a thrombocytopenic SLE patient, whether or not thrombocytopenia in an individual patient is considered. These results are consistent with thrombocytopenia being a component of a severe familial form of SLE and with genes at 1q22-23 and 11p13 contributing to this severe phenotype and to the subsequent high mortality associated with thrombocytopenia in SLE.
Publisher
American Society of Hematology
Subject
Cell Biology,Hematology,Immunology,Biochemistry
Reference41 articles.
1. The 1982 revised criteria for the classification of systemic lupus erythematosus.;Tan;Arthritis Rheum.,1982
2. Familial aggregation of lupus and autoimmunity in an unusual multiplex pedigree.;Sestak;J Rheumatol.,1999
3. The application of genetic epidemiology to systemic lupus erythematosus.;Hochberg;J Rheumatol.,1987
4. A revised estimate of twin concordance in systemic lupus erythematosus.;Deapen;Arthritis Rheum.,1992
5. Systemic lupus erythematosus: considerations for a genetic approach.;Harley;J Invest Dermatol.,1994
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