Ferrer Marta
Department of Allergy and Clinical Immunology, Clinica Universidad de Navarra, Pio XII, 36, 31008 Pamplona, Spain.
Clin Transl Allergy. 2015 Aug 25;5:30. doi: 10.1186/s13601-015-0074-7. eCollection 2015.
Chronic spontaneous urticaria (CSU) is a highly debilitating skin disease associated with systemic features. We have made significant progress in several aspects relating to this condition. However, the exact physiopathology remains unknown. There is mounting evidence for an autoimmune basis, demonstrated by the CSU serum ability to activate healthy donors skin mast cells and blood basophils. However, it is only seen among 35-40% of patients. Mast cells and basophils play an important role in this skin condition. Both cells in CSU patients have unique features that differentiate them from basophils and mast cells from healthy donors. In the case of basophils, basopenia is typically found in CSU patients. Basophils from CSU patients also tend to be hyporesponsive to stimuli that act through the IgE receptor, responsive to other stimuli as MCP-1 or C5a, and hyperesponsive when incubated with sera. Eosinophils are also present in CSU skin biopsies, yet their exact role has not yet been defined. Likewise, endothelial cells also play a function, as indirectly demonstrated by an increase of vasoactive peptides in skin and plasma of CSU patients' samples. All these facts orchestrate a systemic inflammation response producing a significant increase of several inflammatory markers. Unfortunately, we lack a unitary model that could explain the exact role of each of these players. In this review, we will describe the history and discover the pathway to the present knowledge on the immunological facts of this disease.
慢性自发性荨麻疹(CSU)是一种与全身症状相关的极具致残性的皮肤病。我们在与这种病症相关的几个方面已经取得了重大进展。然而,确切的生理病理学仍然未知。越来越多的证据表明其存在自身免疫基础,这通过CSU血清激活健康供体皮肤肥大细胞和血液嗜碱性粒细胞的能力得以证明。然而,这仅在35% - 40%的患者中出现。肥大细胞和嗜碱性粒细胞在这种皮肤病中起重要作用。CSU患者的这两种细胞都具有独特特征,使其与健康供体的嗜碱性粒细胞和肥大细胞有所不同。就嗜碱性粒细胞而言,CSU患者通常存在嗜碱性粒细胞减少。CSU患者的嗜碱性粒细胞对通过IgE受体起作用的刺激往往反应低下,对其他刺激如MCP - 1或C5a有反应,并且与血清一起孵育时反应过度。嗜酸性粒细胞也存在于CSU皮肤活检组织中,但其确切作用尚未明确。同样,内皮细胞也发挥作用,这在CSU患者样本的皮肤和血浆中血管活性肽增加中得到间接证明。所有这些因素共同促成了全身炎症反应,导致几种炎症标志物显著增加。不幸的是,我们缺乏一个能够解释这些因素各自确切作用的统一模型。在这篇综述中,我们将描述该病的历史,并探寻通向当前关于这种疾病免疫学事实知识的途径。