Abraham Hrudya, Kuzhively Jose, Rizvi Syed W
Division of Rheumatology, Department of Internal Medicine, MacNeal Hospital, Berwyn, IL, USA.
Division of Endocrinology and Metabolism, Rush University Medical Center, Chicago, IL, USA.
Am J Case Rep. 2017 Sep 12;18:980-983. doi: 10.12659/ajcr.903541.
BACKGROUND Chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon manifestation of systemic lupus erythematosus (SLE). We report a case of SLE presenting as CIDP and discuss the diagnosis, management, and prognosis of CIDP. CASE REPORT A 40-year-old woman with a past medical history of SLE treated with hydroxychloroquine presented with bilateral, progressive, ascending, sensory and motor neuropathy. Physical examination showed weakness and reduced temperature of all extremities, reduced pinprick and vibration sense of the distal extremities, loss of reflexes, and walking with a wide-based unsteady gait. Laboratory investigations showed positive antinuclear antibodies (ANA), anti-(smooth muscle (SM) antibody, anti-RNP antibody, anti-SSA antibody, anti-ds-DNA antibody, and an erythrocyte sedimentation rate (ESR) of 75 mm/hr, low C4, leukopenia, and anemia. Electromyography (EMG) confirmed the diagnosis of CIDP. The patient's neuropathy and muscle weakness improved on treatment with intravenous immunoglobulin (IVIG) and high-dose steroids. CONCLUSIONS The early clinical diagnosis of CIDP, supported by serological autoantibody profiles associated with SLE, can predict a good response to steroids. Most patients with CIDP are treated successfully with steroids if the diagnosis is made early. IVIG, plasmapheresis, or immunosuppressive therapy should be considered if there is no response to steroids.
背景 慢性炎症性脱髓鞘性多发性神经病（CIDP）是系统性红斑狼疮（SLE）的一种罕见表现。我们报告一例以CIDP形式出现的SLE病例，并讨论CIDP的诊断、管理及预后。病例报告 一名40岁女性，有SLE病史，曾接受羟氯喹治疗，出现双侧进行性上升性感觉和运动性神经病。体格检查显示四肢无力、温度降低，远端肢体针刺觉和振动觉减退，反射消失，行走时呈宽基底不稳步态。实验室检查显示抗核抗体（ANA）、抗平滑肌（SM）抗体、抗核糖核蛋白（RNP）抗体、抗干燥综合征A（SSA）抗体、抗双链DNA（ds-DNA）抗体阳性，红细胞沉降率（ESR）为75mm/h，补体C4降低，白细胞减少和贫血。肌电图（EMG）确诊为CIDP。患者经静脉注射免疫球蛋白（IVIG）和大剂量类固醇治疗后，神经病和肌无力症状改善。结论 由与SLE相关的血清学自身抗体谱支持的CIDP早期临床诊断可预测对类固醇治疗的良好反应。如果早期诊断，大多数CIDP患者用类固醇治疗可成功。如果对类固醇无反应，应考虑IVIG、血浆置换或免疫抑制治疗。
