Small-fiber neuropathy and celiac disease: A narrative review

Abstract

Background: Neurological manifestations of celiac disease (CD) have a prevalence of 10% to 22% among patients. Of this group, neuropathy is present in up to 23%, with small fiber neuropathy (SFN) being the most described, with a predominance of painful symptoms and appendicular paresthesia. Objectives: Review literature to describe the clinical management of SFN in CD. Design and setting: Narrative review. Methods: Non-systematic review on Pubmed and Scielo database. Results: CD is a chronic inflammatory autoimmune disease that can generate extraintestinal manifestations as SFN. Small fiber neuropathy is a painful focal sensory neuropathy of slow progression, with distal predominance, symmetrical or not, beginning in adulthood and, sometimes, followed by autonomic dysfunction. Electroneuromyography studies (ENMG) suggest greater involvement of myelinated Adelta and C myelinated thin fibers, which is a precursor of sensory ganglionopathy in the dorsal root and can progress to large fiber neuropathy. The ENMG of SFN is usually normal because it is generally not demyelinating. To confirm the diagnosis, a skin biopsy that evaluates the fiber’s intra-epidermal density is indicated. Another exam is the quantitative test of the sudomotor reflex, capable of evaluating autonomic function. Finally, treatment should be directed to the underlying cause, optimization of the treatment of CD, and the management of symptoms, such as pain. Conclusions: The SFN, despite being an uncommon manifestation of CD, is possibly underdiagnosed due to the lack of studies evaluating this manifestation in celiac patients. Therefore, further studies are needed in order to instigate early diagnosis and adequate clinical management.