Diagnostic criteria for idiopathic small fiber neuropathy: A systematic review

Simon Haroutounian, Marko S. Todorovic, Mathias Leinders, Marta Campagnolo, Jennifer S. Gewandter, Robert H. Dworkin, Roy Freeman

Research output: Contribution to journalReview articlepeer-review

16 Scopus citations


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.

Original languageEnglish
Pages (from-to)170-177
Number of pages8
JournalMuscle and Nerve
Issue number2
StatePublished - Feb 2021


  • SFN
  • diagnostic criteria
  • idiopathic small fiber neuropathy
  • neuropathic pain
  • peripheral neuropathy
  • small fiber neuropathy


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