疾病的概念主要源于肥大细胞(MC)的慢性异常组成型和反应性激活,没有肥大细胞增生症的MC瘤形成,首次出现在1980年代,但仅在过去十年中,对“肥大细胞活化综合征”(MCAS)的认识才显著增长。关于诊断标准的两个主要建议已经出现。一,最初发表于2012年,被其作者标记为“共识”(此处重新称为“共识-1”)。另一个相当大的调查人员和从业人员赞成不同的方法(最初发表于2011年,这里新称为“共识-2”),在某些方面类似于“共识-1”,但在其他方面不同,导致这些建议在符合诊断条件(以及治疗条件)的患者数量上存在实质性差异。“共识-2”标准的过度诊断可能会有问题,但是根据“共识-1”标准进行的诊断不足似乎是一个更大的问题,因为(1)越来越认识到MCAS是普遍存在的(占总人口的17%),和(2)大多数MCAS患者,无论诊断前的疾病持续时间如何,最终可以确定产生持续改善的治疗方法。我们分析这些建议(和其他建议),并建议,直到仔细的研究提供更明确的答案,任何一个建议的诊断都是有效的,合理,和有益的。
The concept that disease rooted principally in chronic aberrant constitutive and reactive activation of mast cells (MCs), without the gross MC neoplasia in mastocytosis, first emerged in the 1980s, but only in the last decade has recognition of \"mast cell activation syndrome\" (MCAS) grown significantly. Two principal proposals for diagnostic criteria have emerged. One, originally published in 2012, is labeled by its authors as a \"
consensus\" (re-termed here as \"
consensus-1\"). Another sizable contingent of investigators and practitioners favor a different approach (originally published in 2011, newly termed here as \"
consensus-2\"), resembling \"
consensus-1\" in some respects but differing in others, leading to substantial differences between these proposals in the numbers of patients qualifying for diagnosis (and thus treatment). Overdiagnosis by \"
consensus-2\" criteria has potential to be problematic, but underdiagnosis by \"
consensus-1\" criteria seems the far larger problem given (1) increasing appreciation that MCAS is prevalent (up to 17% of the general population), and (2) most MCAS patients, regardless of illness duration prior to diagnosis, can eventually identify treatment yielding sustained improvement. We analyze these proposals (and others) and suggest that, until careful research provides more definitive answers, diagnosis by either proposal is valid, reasonable, and helpful.