Pediatric Rheumatology Online Journal July 2003 Childhood Lupus→ Case Reports → Abstract #108


CLINICAL AND LABORATORY FINDINGS OF IDENTICAL TRIPLETS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

P. H. Pepmueller,1 S. Kamat,1 J. M. Low,1 A. K. Chauhan,1 T. L. Moore.1

1Pediatric Rheumatology, Saint Louis University, St. Louis, MO

Objective: To analyze clinical and laboratory studies in identical triplets with systemic lupus erythematosus (SLE).
Methods: We studied identical triplets who presented with manifestations of SLE in reverse birth order at the age of 8. All 3 had skin rash, fatigue, and Type II-III glomerulonephritis by biopsy. Laboratory findings showed all 3 with ANA titers 1:640, markedly elevated anti-ds DNA antibody levels 1000 IU, and low C3, C4, and CH5O levels. We isolated immune complexes (IC) from their sera by the FcΓ II immunoabsorbent (IA) column to determine if there were differences in the constituents of their isolated IC. All 3 patients sera were added to an equal volume of PBS, centrifuged, and the supernatant passed over the FcΓ II receptor bound Sepharose 4BFF IA column 3 times. The resin was washed 3 times with PBS and the IC eluted with 0.1M glycine-HCl (pH 3.5) and neutralized with 1M Tris-HC1 (pH 7.5). The samples were concentrated to a final volume using a Millipore Filter. The IC were then subjected to SDS-PAGE. The gel was then stained and analyzed.
Results: All 3 patients showed IC detected by the FcΓ II IA column on 2 simultaneous testing draws. They all demonstrated bands compatible with IgM and IgG breakdown components and C3 and C4. However, 2 triplets showed 12 other bands while the third triplet showed 14 bands, the differing bands being quite prominent indicating a different make-up of her IC.
Conclusions: We describe the first set of identical triplets with SLE. We demonstrate that IC can be isolated by the FcΓ II IA column from the sera of all the triplets. The isolated IC contained IgM, IgG, C3, and C4. They also contained other unidentified constituents, which differed among the triplets. This indicates that though they are genetically identical with SLE, they can produce different immunological responses.