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Hematopoietic Stem Cell Transplantation for Systemic Sclerosis: If You Are Confused, Remember: 'It Is a Matter of the Heart'
- Source :
- The Journal of Rheumatology. 39:206-209
- Publication Year :
- 2012
- Publisher :
- The Journal of Rheumatology, 2012.
-
Abstract
- For the past 20 years, the standard of care for systemic sclerosis (SSc) with lung involvement has been oral or intravenous (IV) cyclophosphamide (CYC). To date, there have been 2 randomized trials and 2 metaanalyses of prospective studies using oral or IV CYC in SSc-related interstitial pneumonitis (interstitial lung disease; ILD) and none have reported improvement in lung function1,2,3,4,5. The Scleroderma Lung Research Study Group’s study in The New England Journal of Medicine reported that oral CYC daily for 1 year is of “modest benefit” compared to placebo1. However, the term “modest benefit” does not mean that lung function improved. In fact, the forced vital capacity (FVC) and DLCO declined in both placebo and CYC-treated patients1. “Modest benefit” means that, after 1 year, the rate of decline in FVC was less in those receiving CYC compared to placebo, but the lung function still worsened on CYC1. Further, at 2-year followup there was no difference in loss of lung function between oral daily CYC and placebo2. Due to a lack of effective standard therapy, SSc — a lethal disease that involves vital organs — needs a new and effective approach. An approach that began in patients about 14 years ago, hematopoietic stem cell transplantation (HSCT), has been demonstrated to improve both skin and lung function as well as quality of life in patients with SSc6,7,8. Transplantation has been performed safely in some studies8, but results have been complicated by high treatment-related mortality in others9. Mortality of HSCT for SSc can be markedly reduced, however, if the reasons for mortality are properly recognized. The safety of HSCT is determined by 3 variables: (1) … Address correspondence to Dr. Burt; E-mail: rburt{at}northwestern.edu
- Subjects :
- Male
Vital capacity
medicine.medical_specialty
Scleroderma, Systemic
business.industry
medicine.medical_treatment
Immunology
Hematopoietic Stem Cell Transplantation
Interstitial lung disease
Hematopoietic stem cell transplantation
respiratory system
medicine.disease
Scleroderma
Surgery
Transplantation
FEV1/FVC ratio
Rheumatology
DLCO
Internal medicine
medicine
Humans
Immunology and Allergy
Female
business
Prospective cohort study
Subjects
Details
- ISSN :
- 14992752 and 0315162X
- Volume :
- 39
- Database :
- OpenAIRE
- Journal :
- The Journal of Rheumatology
- Accession number :
- edsair.doi.dedup.....01907cb3975905c32c4214e3a8651580
- Full Text :
- https://doi.org/10.3899/jrheum.111302