University of Cambridge School of Clinical Medicine, Cambridge, UK.
Arthritis Rheumatol. 2014 Apr;66(4):1000-9. doi: 10.1002/art.38302.
To characterize peripheral neuropathy subtypes, ancillary studies, and immunologic profiles associated with peripheral neuropathies in patients with systemic lupus erythematosus (SLE).
In this 25-year study of 2,097 SLE patients, we characterized peripheral neuropathies due to SLE and compared clinical and SLE-related features in patients with versus those without neuropathy.
The prevalence of peripheral neuropathies was 5.9% (123 of 2,097 patients), and 66.7% of these patients (82 of 123) had peripheral neuropathies attributable to SLE. We noted that 17.1% of the patients with peripheral neuropathies due to SLE (14 of 82 patients) had small-fiber neuropathy, which is a painful neuropathy not included in the American College of Rheumatology (ACR) neuropsychiatric SLE (NPSLE) case definitions. SLE patients with small-fiber neuropathies could present with unorthodox neuropathic pain patterns not consistent with a stocking-glove distribution and had associated skin biopsy results suggestive of dorsal root ganglion neuronal cell loss. Compared to SLE patients without peripheral neuropathies, those with peripheral neuropathies had lower mean disease activity (P = 0.01) and higher disease damage (P < 0.01) and were more likely to have a history of herpes zoster virus infection (P < 0.01), osteoporotic fractures (P < 0.01), and opportunistic infections (P < 0.01).
Our findings indicate that small-fiber neuropathy is a frequently occurring peripheral neuropathy. The skin biopsy findings in small-fiber neuropathy patients support the notion that distinct mechanisms target the dorsal root ganglia as well as distal axons. SLE patients with peripheral neuropathy have lower mean disease activity scores and higher disease damage. Our findings suggest that revision of the ACR NPSLE case definitions, which currently do not include small-fiber neuropathies, is warranted.
描述与系统性红斑狼疮（SLE）相关的周围神经病的亚型、辅助检查和免疫特征。
在这项对 2097 例 SLE 患者进行的 25 年研究中，我们对 SLE 引起的周围神经病进行了特征描述，并比较了有神经病变和无神经病变患者的临床和 SLE 相关特征。
周围神经病的患病率为 5.9%（2097 例患者中有 123 例），其中 66.7%（123 例中的 82 例）的周围神经病归因于 SLE。我们注意到，17.1%（82 例中的 14 例）SLE 引起的周围神经病患者患有小纤维神经病，这是一种疼痛性神经病，不包括在风湿性疾病学会（ACR）神经精神性 SLE（NPSLE）的病例定义中。患有小纤维神经病的 SLE 患者可能出现不符合袜套分布的非典型神经痛模式，并且相关皮肤活检结果提示背根神经节神经元细胞丢失。与无周围神经病的 SLE 患者相比，有周围神经病的 SLE 患者的平均疾病活动度较低（P = 0.01），疾病损害程度较高（P < 0.01），并且更有可能有带状疱疹病毒感染史（P < 0.01）、骨质疏松性骨折（P < 0.01）和机会性感染（P < 0.01）。
我们的发现表明，小纤维神经病是一种常见的周围神经病。小纤维神经病患者的皮肤活检结果支持这样一种观点，即不同的机制靶向背根神经节和远端轴突。有周围神经病的 SLE 患者的平均疾病活动评分较低，疾病损害程度较高。我们的发现表明，有必要对目前不包括小纤维神经病的 ACR NPSLE 病例定义进行修订。
