Pompilian Valer Mihai, Tănăseanu Ştefăniţă, Badea Camelia, Zurac Sabina, Socoliuc Claudiu, Badelita Sorina, Botez Elena, Antohe Mihaela
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Rom J Intern Med. 2017 Sep 26;55(3):179-182. doi: 10.1515/rjim-2017-0019.
Monoclonal gammopathies complicated by AL amyloidosis can mimic giant cell arteritis (GCA). We hereby present the case of a 63 year old woman in whom symptoms consistent with GCA were the first manifestations of a monoclonal gammopathy of unknown significance (MGUS) associated with amyloidosis. A 63 year old woman was admitted for temporal headache, maseterine claudication, neck and shoulder stiffness. She was recently diagnosed with carpal tunnel syndrome. On physical examination she had prominent temporal arteries, macroglosia and orthostatic hypotension. Muscular strength was normal. She had high ESR and CRP; in this clinical context, GCA was suspected. A gamma spike on serum protein electrophoresis raised the suspicion of monoclonal gammopathy (MG). Immunoelectrophoresis revealed monoclonal bands for IgG and kappa chains. Massive deposits of amyloid and no inflammation were found on temporal artery biopsy. Multiple myeloma and lymphoma were ruled out. A diagnosis of AL amyloidosis complicating MGUS was formulated. She did well on therapy with bortezomib, cyclophosphamide and dexamethasone. Cases published in medical literature reveal amyloidosis mimicking GCA in the setting of established MGUS. As far as we know, this is the first case of MGUS with IgG and kappa chains in which a GCA-like picture induced by amyloidosis was present from the very onset.
合并 AL 淀粉样变性的单克隆丙种球蛋白病可酷似巨细胞动脉炎（GCA）。我们在此报告一例 63 岁女性病例，其与 GCA 相符的症状是与淀粉样变性相关的意义未明单克隆丙种球蛋白病（MGUS）的首发表现。一名 63 岁女性因颞部头痛、咀嚼肌跛行、颈部和肩部僵硬入院。她最近被诊断为腕管综合征。体格检查发现其颞动脉突出、巨舌和体位性低血压。肌力正常。她的血沉（ESR）和 C 反应蛋白（CRP）升高；在此临床背景下，怀疑为 GCA。血清蛋白电泳上的γ峰引起了对单克隆丙种球蛋白病（MG）的怀疑。免疫电泳显示 IgG 和κ链的单克隆条带。颞动脉活检发现大量淀粉样蛋白沉积且无炎症。排除了多发性骨髓瘤和淋巴瘤。诊断为合并 MGUS 的 AL 淀粉样变性。她接受硼替佐米、环磷酰胺和地塞米松治疗效果良好。医学文献中发表的病例显示，在已确诊的 MGUS 情况下，淀粉样变性可酷似 GCA。据我们所知，这是首例具有 IgG 和κ链的 MGUS 病例，其中淀粉样变性从一开始就呈现出类似 GCA 的表现。
