Idiopathic small fiber neuropathy (iSFN) lacks broadly accepted diagnostic criteria, which hinders its timely diagnosis and treatment. A systematic literature review was performed to assess the published screening and diagnostic criteria for iSFN, excluding studies where SFN was of well‐established etiology. Eighty‐four clinical studies and seven guideline/review publications were included in this systematic review. Substantial heterogeneity existed in iSFN diagnostic criteria. The most common set of criteria to diagnose iSFN [presence of any symptoms of iSFN, absence of large fiber involvement, and reduced intraepidermal nerve fiber density (IENFD)] was used in only 14% of studies. Mandatory individual criteria to confirm iSFN included any sensory symptoms (60% of studies), pain (19% of studies), small fiber signs (20% of studies), absence of large fiber signs (62% of studies), reduced IENFD (38% of studies), and autonomic symptoms (1% of studies). This review highlights a clear need for standardized, evidence‐based guidelines for diagnosing iSFN.

中文翻译：

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特发性小纤维神经病的诊断标准：系统评价

特发性小纤维神经病（iSFN）缺乏广泛接受的诊断标准，这阻碍了其及时的诊断和治疗。进行了系统的文献综述，以评估已发表的iSFN筛查和诊断标准，但SFN病因明确的研究除外。该系统评价包括84项临床研究和7项指南/综述出版物。iSFN诊断标准中存在大量异质性。仅14％的研究使用了诊断iSFN的最常见标准[iSFN的任何症状，无大纤维受累以及表皮内神经纤维密度降低（IENFD）]。确认iSFN的强制性个人标准包括任何感官症状（占研究的60％），疼痛（占研究的19％），小纤维征象（占研究的20％），没有大纤维征象（占研究的62％），IENFD减少（占研究的38％）和自主神经症状（占研究的1％）。这篇评论强调了对诊断iSFN的标准化，循证指南的明确需求。