Iida Harunobu, Hanaoka Hironari, Asari Yusa, Ishimori Kana, Kiyokawa Tomofumi, Takakuwa Yukiko, Yamasaki Yoshioki, Yamada Hidehiro, Okazaki Takahiro, Doi Masatomo, Ozaki Shoichi
Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Japan.
Division of Pathology, St. Marianna University School of Medicine, Japan.
Intern Med. 2018 Jan 1;57(1):101-106. doi: 10.2169/internalmedicine.8913-17. Epub 2017 Oct 11.
Polyarteritis nodosa (PAN) is a medium vessel vasculitis affecting systemic organs. Muscle involvement of PAN usually lacks elevation of creatinine kinase (CK). We herein report a case of PAN with rhabdomyolysis. A 71-year-old man was hospitalized because of muscle weakness of the lower limbs that persisted for 1 month. On a physical examination, rapidly progressive lower proximal muscle weakness and bilateral drop foot were observed. His blood test showed an elevation in the C-reactive protein (19.5 mg/dL) and CK (13,435 IU/L) levels and negativity for anti-neutrophilic cytoplasmic antibody. Computed tomographic angiography showed stenosis of the left renal artery. Electromyogram indicated mono-neuritis multiplex pattern, and enhanced magnetic resonance imaging demonstrated discretely granular hyperintensities on T2 and slow tau inversion recovery in his femoral muscles. A femoral muscle-biopsy specimen showed fibrinoid necrosis of medium-sized vessels and disruption of the elastic lamina of the vessel wall in fascia. Furthermore, muscle necrosis was localized depending on the arterial distribution, suggesting ischemic changes in the muscles. Given these findings, he was diagnosed with PAN with rhabdomyolysis and treated with methyl-prednisolone pulse therapy followed by oral prednisolone at 50 mg/day. He was additionally treated with monthly intravenous cyclophosphamide at 500 mg. Sustained remission has been obtained for two months since the treatment. Although rhabdomyolysis rarely manifests with PAN, it should be included in a differential diagnosis of febrile patients presenting with acute myalgia and weakness with CK elevation.
结节性多动脉炎（PAN）是一种影响全身器官的中等血管血管炎。PAN的肌肉受累通常缺乏肌酸激酶（CK）升高。我们在此报告一例伴有横纹肌溶解的PAN病例。一名71岁男性因下肢肌肉无力持续1个月而住院。体格检查发现，患者存在快速进展的近端下肢肌肉无力和双侧足下垂。他的血液检查显示C反应蛋白水平升高（19.5mg/dL），CK水平升高（13435IU/L），抗中性粒细胞胞浆抗体阴性。计算机断层血管造影显示左肾动脉狭窄。肌电图显示多灶性单神经炎模式，增强磁共振成像显示其股四头肌在T2加权像上有散在的颗粒状高信号，在液体衰减反转恢复序列上信号缓慢。股四头肌活检标本显示中等大小血管的纤维蛋白样坏死以及筋膜中血管壁弹性膜的破坏。此外，肌肉坏死根据动脉分布而定，提示肌肉存在缺血性改变。基于这些发现，他被诊断为伴有横纹肌溶解的PAN，并接受了甲泼尼龙冲击治疗，随后口服泼尼松龙，剂量为50mg/天。他还每月接受500mg静脉注射环磷酰胺治疗。自治疗以来已持续缓解两个月。尽管横纹肌溶解很少与PAN同时出现，但对于出现急性肌痛、肌无力且CK升高的发热患者，横纹肌溶解应列入鉴别诊断范围。
