[High-dose immunosuppression and autologous peripheral blood stem cell transplantation for immunological reconstitution in two patients with primary Sjögren's syndrome].
OBJECTIVE
To assess the safety and efficacy of high-dose immunosuppression and autologous peripheral blood cell transplantation (APBSCT) in severe and refractory primary Sjögren's syndrome (pSS) and to analyze immune reconstitution in pSS.


METHODS
Two patients with severe and refractory primary pSS were included in this study. They suffered still with active pSS despite the use of prednisone and immunosuppression agents. A regimen of high-dose immunosuppression and APBSCT was carried out for them. Dynamic T cell subgroup was tested with flow cytometry before and after PBSCT and the diversity of T cell receptor repertoire and CDR3 spectrum with RT-PCR and genescan.


RESULTS
Both of the pSS cases underwent PBSCT smoothly. Clinical assessments showed improvement. Immune reconstruction lagged behind hematopoietic reconstitution. The skew of T cell receptor repertoire was somewhat corrected and CDR3 spectrum changed from oligoclonality to poly-clonality.


CONCLUSIONS
High dose chemotherapy (HDC) and APBSCT are feasible and safe and can result in short-term or middle-term improvement of disease in patients with severe pSS which is refractory to conventional treatment. It is observed in this study that immune reconstruction recovered 3 moths after the treatment. Long-term efficacy of HDC + PBSCT in pSS should be studied in large number of cases with follow up of longer time.
Zhang
Zhi-yi
ZY
Department of Rheumatology and Immunology, the First Affiliated Hospital of Haerbin Medical University, Haerbin 150001, China.
Li
Rong-bin
RB
Jiang
Ying
Y
Leng
Xiao-mei
XM
Mei
Yi-fang
YF
Zhao
Yan-ping
YP
Zhao
Yan
Y
D000328:Adult; D003520:Cyclophosphamide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D036102:Peripheral Blood Stem Cell Transplantation / Q000379:methods*; D011971:Receptors, Immunologic / Q000276:immunology; D012859:Sjogren's Syndrome / Q000276:immunology* / Q000628:therapy*; D016176:T-Lymphocyte Subsets / Q000276:immunology; D019172:Transplantation Conditioning; D014182:Transplantation, Autologous
D007166:Immunosuppressive Agents; D011971:Receptors, Immunologic; D003520:Cyclophosphamide