干燥综合征中的小纤维神经病：与其他小纤维神经病的比较。

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

机构信息

Department of Rheumatology; National Reference Center for Sjögren Syndrome and Rare Autoimmune Diseases, Université Paris-Saclay; Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, AP-HP, Le Kremlin Bicêtre, France.

Department of Neurology; National Reference Center for Amyloidosis Neuropathies and Other Rare Peripheral Neuropathies, Université Paris-Saclay; Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin Bicêtre, France.

出版信息

Muscle Nerve. 2020 Apr;61(4):515-520. doi: 10.1002/mus.26824. Epub 2020 Feb 15.

DOI:10.1002/mus.26824

PMID:32012291

Abstract

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.

摘要

简介

我们比较了原发性干燥综合征（pSS）小纤维神经病（SFN；pSS-SFN）与特发性 SFN（i-SFN）和遗传性转甲状腺素蛋白淀粉样变性 SFN（hATTR-SFN）的组织学和临床特征，并描述了 pSS-SFN 的演变过程。

方法

所有经皮肤活检证实的减少表皮内神经纤维密度的 pSS-SFN、i-SFN 和 hATTR-SFN 患者均被回顾性纳入，并对其特征进行比较。为了分析 pSS-SFN 的预后，前瞻性对患者进行了第二次评估。

结果

共纳入 15 例 pSS-SFN、17 例 hATTR-SFN 和 11 例 i-SFN。pSS-SFN 和 i-SFN 患者的 SFN 诊断时间长于 hATTR-SFN。pSS-SFN 比 hATTR-SFN 更常出现疼痛和非长度依赖性模式。12 例（80%）pSS-SFN 患者为非长度依赖性模式。10 例 pSS 患者在 3.1 年后（1.7-4.7 年）再次评估，均未出现与 pSS 相关的大纤维神经病。