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T lymphocyte abnormalities in juvenile systemic sclerosis patients |
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| Authors: | Andreas Reiff Kenneth I. Weinberg Timothy Triche Bernadette Masinsin Kris M. Mahadeo Chuan-Hao Lin Diane Brown Robertson Parkman |
| Affiliation: | 1. Division of Rheumatology, Children''s Hospital Los Angeles, 4650 Sunset Blvd., Mail Stop 60, Los Angeles, CA 90027, USA;2. Department of Pediatrics, Keck School of Medicine, University of Southern California, USA;3. Pediatric Stem Cell Transplant Program, Department of Pediatrics, Stanford University School of Medicine, 1291 Welch Drive, Stanford, CA 94305, USA;4. Department of Pathology, Children''s Hospital Los Angeles, 4650 Sunset Blvd., Mail Stop 43, Los Angeles, CA 90027, USA;5. Keck School of Medicine, University of Southern California, USA;6. Division of Research Immunology/Bone Marrow Transplantation, Blood and Marrow Transplant Program, Children''s Hospital Los Angeles, 4650 Sunset Blvd., Mail Stop 62, Los Angeles, CA 90027, USA;g Division of Gastroenterology, Children''s Hospital Los Angeles, 4650 Sunset Blvd., Mail Stop 78, Los Angeles, CA 90027, USA |
| Abstract: | Multi-center evaluations of pediatric patients with juvenile systemic sclerosis (jSSc) have suggested that the pathogenesis of jSSc may differ from that of systemic sclerosis (SSc) in adult patients. Therefore, we undertook to identify abnormalities in the T lymphocytes of jSSc patients and to determine if they differed from the abnormalities reported in the T lymphocytes of adult SSc patients. We identified decreases in the frequency of resting regulatory T lymphocytes and an increased frequency of CD45RA expressing effector memory (EMRA) CD4 T lymphocytes, which were characterized by an increased frequency of CCR7 protein expressing cells. Neither the increases in the EMRA subpopulation nor the increased CCR7 protein expression have been reported in adult SSc patients. The decrease in resting regulatory T lymphocytes in jSSc patients may permit the expansion of the disease initiating CD4 T lymphocytes present in the CCR7 expressing EMRA CD4 T lymphocyte subpopulation. |
| Keywords: | Regulatory T lymphocytes Juvenile systemic sclerosis CCR7 Autoreactive T lymphocytes |
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