Association of immunological cell profiles with specific clinical phenotypes of scleroderma disease.

José Manuel López-Cacho,Manuel Posada,Blanca Cárdaba,Soledad Gallardo,David Calzada,Antonio María Rabasco,Carlos Lahoz,María Luisa González-Rodríguez,Miriam Aguerri,Teodoro Mayayo

doi:10.1155/2014/148293

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.

Highlights

Scleroderma is a rare autoimmune disease of unknown etiology which affects thousands of people around the world
We have studied the relationship between clinical scleroderma phenotypes and the different cellular subpopulations in well-characterized groups of scleroderma patients, comparing their results with healthy controls and stratifying them according to scleroderma subtypes, time since the diagnosis of the disease, and presence of complications and by time with corticosteroids treatment
Significant differences were detected in scleroderma patients when we compare their results with the control group

Summary

Introduction

Scleroderma is a rare autoimmune disease of unknown etiology which affects thousands of people around the world. Its prevalence is estimated to be between 15 and 35 cases per 100000 inhabitants [1,2,3]. Its first symptoms can be seen around the third and fourth decade of the life but, in some cases, symptoms can exist for several years without a correct diagnosis. Scleroderma is three times more common in women than men. The disease is not linked in any consistent way to race, season, geography, occupation, or socioeconomic status. Environmental etiologies are possible [1]

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