Details

Autor(en) / Beteiligte

• Descamps, Elise
• Henry, Julien
• Labeyrie, Céline
• Adams, David
• Ghaidaa, Adebs Nasser
• Vandendries, Christophe
• Adam, Clovis
• Aiello, David
• Mariette, Xavier
• Seror, Raphaèle

Titel

Small fiber neuropathy in Sjögren syndrome: Comparison with other small fiber neuropathies

Ist Teil von

• Muscle & nerve, 2020-04, Vol.61 (4), p.515-520

Ort / Verlag

Hoboken, USA: John Wiley & Sons, Inc

Erscheinungsjahr

2020

Quelle

Wiley

Beschreibungen/Notizen

• Introduction We compared histological and clinical profiles of primary Sjögren syndrome (pSS) small fiber neuropathy (SFN; pSS‐SFN) with idiopathic SFN (i‐SFN) and hereditary transthyretin amyloidosis SFN (hATTR‐SFN) and described the evolution of pSS‐SFN. Methods All patients with pSS‐SFN, i‐SFN, and hATTR‐SFN confirmed by reduced intraepidermal nerve fiber density on skin biopsy were retrospectively included, and their characteristics were compared. To analyze prognosis of pSS‐SFN, patients prospectively underwent a second evaluation. Results Fifteen pSS‐SFN, 17 hATTR‐SFN, and 11 i‐SFN were included. Time to diagnosis SFN was longer in pSS‐SFN and i‐SFN than in hATTR‐SFN. Painful and non–length‐dependent patterns were more frequent in pSS‐SFN than in hATTR‐SFN. Twelve (80%) patients with pSS‐SFN had a non–length‐dependent pattern. Ten patients with pSS were reassessed after 3.1 years (1.7–4.7); none developed large fiber neuropathy linked to pSS. Discussion Primary Sjögren syndrome SFN is characterized by a more frequent non–length‐dependent pattern compared with i‐SFN and hATTR‐SFN. Primary Sjögren syndrome SFN did not evolve through large fiber neuropathy.