Waheed Waqar, Nickerson Joshua, Ambaye Abiy B, Babi Mohamed-Ali, Tandan Rup
Departments of *Neurological Sciences, †Radiology, and ‡Pathology, University of Vermont College of Medicine and Fletcher Allen Health Care, Burlington, VT.
J Clin Neuromuscul Dis. 2015 Jun;16(4):210-9. doi: 10.1097/CND.0000000000000066.
Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized idiopathic systemic disorder associated with elevated serum IgG4 level and tissue infiltration by IgG4-positive plasma cells. Multiple neurological manifestations, including peripheral neuropathy, have been described in IgG4-RD. Our objective is to discuss a case report and review of the literature, which would expand the spectrum of IgG4-RD.
We describe the clinical features and biopsy findings in a patient with IgG4-RD who presented with features suggestive of neuromyopathy in the setting of recurrent pleural effusion and weight loss.
Electrodiagnostic findings were suggestive of an irritable myopathy and polyradiculoneuropathy with primary demyelination and secondary axonal degeneration. Pleural biopsy and laboratory studies confirmed the diagnosis. Improvement was sustained with steroid therapy.
We describe the first case, to our knowledge, of IgG4-related neuromyopathy associated with recurrent pleural effusion. Our case expands the clinical spectrum of IgG4-RD. Neurologists should be aware of this treatable disorder and in the appropriate clinical context consider it in the differential diagnosis of neuromyopathy.
免疫球蛋白G4相关疾病（IgG4-RD）是一种日益被认识的特发性全身性疾病，与血清IgG4水平升高及IgG4阳性浆细胞的组织浸润有关。IgG4-RD中已描述了多种神经学表现，包括周围神经病变。我们的目的是讨论一例病例报告并进行文献复习，以拓展IgG4-RD的疾病谱。
我们描述了一名IgG4-RD患者的临床特征和活检结果，该患者在反复胸腔积液和体重减轻的情况下出现提示神经肌肉病的特征。
电诊断结果提示为易激惹性肌病和多神经根神经病，伴有原发性脱髓鞘和继发性轴索变性。胸腔活检和实验室检查确诊了该病。类固醇治疗后病情持续改善。
据我们所知，我们描述了首例与反复胸腔积液相关的IgG4相关性神经肌肉病。我们的病例拓展了IgG4-RD的临床谱。神经科医生应了解这种可治疗的疾病，并在适当的临床背景下将其纳入神经肌肉病的鉴别诊断中。
