title: Association of immunological cell profiles with specific clinical phenotypes of scleroderma disease.
abstract: 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.
authors.affiliation: Department of Immunology, IIS-Jiménez Díaz Foundation, Reyes Católicos Avenue 2, 28040 Madrid, Spain.
authors.forename: José Manuel
authors.identifier: 0000-0002-9286-8469
authors.initials: JM
authors.lastname: López-Cacho
authors.affiliation: Department of Immunology, IIS-Jiménez Díaz Foundation, Reyes Católicos Avenue 2, 28040 Madrid, Spain.
authors.forename: Soledad
authors.identifier: 0000-0002-4046-1130
authors.initials: S
authors.lastname: Gallardo
authors.affiliation: Institute of Rare Diseases Research, Carlos III Institute of Health, EuroBioBank, and CIBERER, 28029 Madrid, Spain.
authors.forename: Manuel
authors.identifier: 0000-0002-8372-4180
authors.initials: M
authors.lastname: Posada
authors.affiliation: Department of Immunology, IIS-Jiménez Díaz Foundation, Reyes Católicos Avenue 2, 28040 Madrid, Spain.
authors.forename: Miriam
authors.identifier: 0000-0003-2996-2150
authors.initials: M
authors.lastname: Aguerri
authors.affiliation: Department of Immunology, IIS-Jiménez Díaz Foundation, Reyes Católicos Avenue 2, 28040 Madrid, Spain.
authors.forename: David
authors.initials: D
authors.lastname: Calzada
authors.affiliation: Sani-Red S.L, Barcelona Scientific Park, 08013 Barcelona, Spain.
authors.forename: Teodoro
authors.initials: T
authors.lastname: Mayayo
authors.affiliation: Department of Pharmaceutical Technology, Faculty of Pharmacy, University of Seville, 41012 Seville, Spain.
authors.forename: María Luisa
authors.initials: ML
authors.lastname: González-Rodríguez
authors.affiliation: Department of Pharmaceutical Technology, Faculty of Pharmacy, University of Seville, 41012 Seville, Spain.
authors.forename: Antonio María
authors.initials: AM
authors.lastname: Rabasco
authors.affiliation: Department of Immunology, IIS-Jiménez Díaz Foundation, Reyes Católicos Avenue 2, 28040 Madrid, Spain ; CIBER of Respiratory Diseases (CIBERES), 28029 Madrid, Spain.
authors.forename: Carlos
authors.initials: C
authors.lastname: Lahoz
authors.affiliation: Department of Immunology, IIS-Jiménez Díaz Foundation, Reyes Católicos Avenue 2, 28040 Madrid, Spain ; CIBER of Respiratory Diseases (CIBERES), 28029 Madrid, Spain.
authors.forename: Blanca
authors.identifier: 0000-0003-0142-9901
authors.initials: B
authors.lastname: Cárdaba
mesh_terms: D001402:B-Lymphocytes / Q000276:immunology; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000503:physiopathology; D007694:Killer Cells, Natural / Q000276:immunology; D008214:Lymphocytes / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D011658:Pulmonary Fibrosis / Q000150:complications / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology* / Q000473:pathology*