Plumley Spencer G, Rubio Ronald, Alasfar Said, Jasin Hugo E
Division of Rheumatology and Clinical Immunology, Department of Internal Medicine, University of Arkansas for Medical Sciences and Veterans Administration Hospital, Little Rock, AR 72205, USA.
Semin Arthritis Rheum. 2002 Jun;31(6):377-83. doi: 10.1053/sarh.2002.32549.
Skeletal muscle involvement has been well documented in patients with polyarteritis nodosa (PAN), and symptoms referable to skeletal muscle are not uncommon. However, polymyositis as a mode of presentation of PAN is uncommon. This unusual presentation of PAN has been reported only once previously in the English literature.
This study describes a patient who had diffuse weakness, myalgias, and markedly elevated serum creatinine phosphokinase, mimicking polymyositis. The literature dealing with the clinical aspects of muscle involvement in PAN is reviewed.
A 24-year-old man was admitted to the hospital with a 1-month history of fever, myalgia, and muscle weakness. Necrotizing vasculitis was shown on subsequent muscle biopsy, consistent with PAN. Literature review indicated that muscle involvement is common in PAN, as has been shown by the frequency of muscular symptoms and by histologic evidence obtained from both clinical and autopsy studies. Nineteen percent of patients with PAN had documented myopathy, and autopsy series have shown skeletal muscle involvement in 30% to 48% of cases. However, polymyositis as a mode of presentation of PAN is rare. We found only 1 other patient with PAN who had elevated creatinine phosphokinase and diffuse myopathy suggestive of polymyositis.
PAN should be suspected in cases of focal or diffuse myopathy, especially in the context of a systemic disease. Biopsy of symptomatic muscles or EMG-directed biopsies can be helpful in establishing a diagnosis of PAN to allow the physician to provide early treatment.
结节性多动脉炎(PAN)患者出现骨骼肌受累已有充分记录,骨骼肌相关症状并不少见。然而,以多发性肌炎作为PAN的一种表现形式并不常见。此前英文文献中仅报道过一次PAN的这种不寻常表现。
本研究描述了一名出现弥漫性肌无力、肌痛且血清肌酸磷酸激酶显著升高,类似多发性肌炎的患者。对有关PAN患者肌肉受累临床方面的文献进行综述。
一名24岁男性因发热、肌痛和肌无力1个月病史入院。随后的肌肉活检显示为坏死性血管炎,与PAN相符。文献综述表明,PAN患者出现肌肉受累很常见,这已通过肌肉症状的发生频率以及临床和尸检研究获得的组织学证据得到证实。19%的PAN患者有记录在案的肌病,尸检系列研究显示30%至48%的病例存在骨骼肌受累。然而,以多发性肌炎作为PAN的一种表现形式较为罕见。我们仅发现另外1例PAN患者肌酸磷酸激酶升高且存在提示多发性肌炎的弥漫性肌病。
对于局灶性或弥漫性肌病患者,尤其是在存在全身性疾病的情况下,应怀疑PAN。对有症状的肌肉进行活检或在肌电图引导下进行活检有助于确诊PAN,以便医生尽早进行治疗。