Mastocytosis and systemic sclerosis: A clinical association

被引:0
作者
Bagnato G. [1 ]
Roberts W.N. [2 ]
Sciortino D. [1 ]
Sangari D. [1 ]
Cirmi S. [3 ]
Ravenell R.L. [2 ]
Navarra M. [3 ]
Bagnato G. [1 ]
Gangemi S. [4 ,5 ]
机构
[1] University of Messina, Division of Rheumatology, Department of Clinical and Experimental Medicine, Messina
[2] University of Louisville, Division of Rheumatology, Department of Medicine, Louisville, KY
[3] University of Messina, Department of Chemical, Biological, Pharmaceutical and Environmental Sciences, Messina
[4] University of Messina, School and Division of Allergy and Clinical Immunology, Department of Clinical and Experimental Medicine, Messina
[5] Institute of Applied Sciences and Intelligent Systems (ISASI), Pozzuoli NA
关键词
Fibrosis; Mastcells; Mastocytosis; Systemic sclerosis;
D O I
10.1186/s12948-016-0050-3
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学科分类号
摘要
Background: Systemic sclerosis (SSc) is a complex autoimmune disease characterized by vascular alterations and autoimmune activation leading to widespread organ fibrosis. At the early stage of disease when organ involvement and extent of disease are emerging, mast cells may have some role, as implied by both symptoms and histologic evidence. Case presentation: A female patient diagnosed with cutaneous mastocytosis experienced the onset of systemic sclerosis after 15 years followed by the switch of mastocytosis to the systemic phenotype. A literature review on the evidences related to mast-cells activation in systemic sclerosis is presented below. Conclusions: For clinicians, more attention must be paid to the potential association between systemic sclerosis and cancer. This case suggests that a proliferative disease in the mast cell compartment-though representing a rare association-may not be completely unexpected in SSc and perhaps excess mast cell activity can serve a pathogenic role in promoting fibrotic disease. © 2016 The Author(s).
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