肥大细胞增多症与系统性硬化症：一种临床关联。
Mastocytosis and systemic sclerosis: a clinical association.
作者信息
Bagnato Gianluca, Roberts William Neal, Sciortino Davide, Sangari Donatella, Cirmi Santa, Ravenell Roneka L, Navarra Michele, Bagnato Gianfilippo, Gangemi Sebastiano
机构信息
Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.
Division of Rheumatology, Department of Medicine, University of Louisville, Louisville, KY USA.
出版信息
Clin Mol Allergy. 2016 Oct 11;14:13. doi: 10.1186/s12948-016-0050-3. eCollection 2016.
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.
背景
系统性硬化症（SSc）是一种复杂的自身免疫性疾病，其特征为血管改变和自身免疫激活，进而导致广泛的器官纤维化。在疾病早期，当器官受累情况和疾病程度逐渐显现时，肥大细胞可能发挥了一定作用，这在症状和组织学证据中均有暗示。
病例报告
一名诊断为皮肤肥大细胞增多症的女性患者，在15年后出现了系统性硬化症，随后肥大细胞增多症转变为全身型。以下是关于系统性硬化症中肥大细胞激活相关证据的文献综述。
结论
对于临床医生而言，必须更加关注系统性硬化症与癌症之间的潜在关联。该病例表明，肥大细胞区室的增殖性疾病——尽管这种关联较为罕见——在系统性硬化症中可能并非完全出乎意料，也许肥大细胞活性过高在促进纤维化疾病方面可起到致病作用。
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