Primary Sjogren's syndrome and autoimmune hemolytic anemia in sisters. A family study.

Familial primary Sjogren's syndrome is infrequently reported despite a strong association with a genetically determined factor, HLA-DR3. Although autoimmune hemolytic anemia occurring with secondary Sjogren's syndrome has been well-documented, its association with primary Sjogren's syndrome has not previously been well-recognized. Thus, the unique occurrence of primary Sjogren's syndrome and autoimmune hemolytic anemia in two sisters prompted investigation of other family members for autoimmune diseases and serologic phenomena and their relationships to HLA genotypes. Serologic analysis (antinuclear antibodies, anti-single-stranded DNA, biologic false-positive result for syphilis, and rheumatoid factor) and HLA-A, B, C, and DR typing were performed in 19 relatives in the kindred. Although HLA-DR3 occurred in those affected with Sjogren's syndrome and thyroid disease, there was no consistent segregation of HLA haplotype with serologic abnormalities in other relatives. These data suggest that there are other, non-HLA-linked, factors contributing to the autoimmune diseases in this family.