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BioMed Research International
Volume 2014, Article ID 148293, 8 pages
Research Article

Association of Immunological Cell Profiles with Specific Clinical Phenotypes of Scleroderma Disease

1Department of Immunology, IIS-Jiménez Díaz Foundation, Reyes Católicos Avenue 2, 28040 Madrid, Spain
2Institute of Rare Diseases Research, Carlos III Institute of Health, EuroBioBank, and CIBERER, 28029 Madrid, Spain
3Sani-Red S.L, Barcelona Scientific Park, 08013 Barcelona, Spain
4Department of Pharmaceutical Technology, Faculty of Pharmacy, University of Seville, 41012 Seville, Spain
5CIBER of Respiratory Diseases (CIBERES), 28029 Madrid, Spain

Received 7 January 2014; Revised 17 February 2014; Accepted 16 March 2014; Published 10 April 2014

Academic Editor: Richard Gomer

Copyright © 2014 José Manuel López-Cacho et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


This study aimed to search the correlation among immunological profiles and clinical phenotypes of scleroderma in well-characterized groups of scleroderma patients, comparing forty-nine scleroderma patients stratified according to specific clinical phenotypes with forty-nine healthy controls. Five immunological cell subpopulations (B, CD4+ and CD8+ T-cells, NK, and monocytes) and their respective stages of apoptosis and activation were analyzed by flow cytometry, in samples of peripheral blood mononuclear cells (PBMCs). Analyses of results were stratified according to disease stage, time since the diagnosis, and visceral damage (pulmonary fibrosis, pulmonary hypertension, and cardiac affliction) and by time of treatment with corticosteroids. An increase in the percentages of monocytes and a decrease in the B cells were mainly related to the disease progression. A general apoptosis decrease was found in all phenotypes studied, except in localized scleroderma. An increase of B and NK cells activation was found in patients diagnosed more than 10 years ago. Specific cell populations like monocytes, NK, and B cells were associated with the type of affected organ. This study shows how, in a heterogeneous disease, proper patient’s stratification according to clinical phenotypes allows finding specific cellular profiles. Our data may lead to improvements in the knowledge of prognosis factors and to aid in the analysis of future specific therapies.