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Stem cell transplant in systemic sclerosis: An Indian experience
Author(s) -
Nair Velu,
Vasdev Vivek,
Kumar Abhishek,
Shankar Subramanian,
Nair Vivek,
Sharma Ajay
Publication year - 2018
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/1756-185x.13262
Subject(s) - medicine , fludarabine , cyclophosphamide , hematopoietic stem cell transplantation , scleroderma (fungus) , cohort , surgery , interstitial lung disease , lung , transplantation , pathology , chemotherapy , inoculation
Aim To prospectively evaluate long term outcomes in a cohort of patients with Systemic sclerosis treated with Hematopoietic stem cell transplant (HSCT). Method This is a prospective observational study of four SS c patients who underwent HSCT at a tertiary care center in India between 2008–2012. The selection criteria included young individuals with rapidly progressive disease and at least one major organ involvement. We used granulocyte colony‐stimulating factor for peripheral blood stem cell mobilization, pre‐transplant conditioning with fludarabine, cyclophosphamide and rabbit anti‐thymocyte globulin followed by re‐infusion of autologous stem cells as per standard institute protocol. Results A total of four patients (one male and three females) underwent autologous HSCT for SS c. Patients had heterogeneous disease manifestations including severe Raynaud's phenomenon with vasculopathic ulcers, gastrointestinal problems and mild interstitial lung disease ( ILD ). Patients were followed up for a mean duration of 7 years. There was significant sustained improvement in skin score, vasculopathy and gastrointestinal manifestations. Interstitial lung disease did not show any deterioration. The quality of life indices showed remarkable improvement in all subjects. No complications related to transplant were noted. Conclusion In absence of an effective pharmacotherapy for SS c, autologous HSCT has a huge potential in management of cutaneous and internal organ manifestations.