一名可能由寻常型银屑病诱发的系统性AA淀粉样变性患者的肌病：一例尸检病例。
Myopathy in a patient with systemic AA amyloidosis possibly induced by psoriasis vulgaris: An autopsy case.
作者信息
Tanabe Hajime, Maki Yoshimitsu, Urabe Shogo, Higuchi Itsuro, Obayashi Konen, Hokezu Youichi
机构信息
Department of Neurology, Oita Prefectural Hospital, 476 Bunyo, Oita, 870-0855, Japan.
Department of Clinical Laboratory, Oita Prefectural Hospital, Oita, Japan.
出版信息
Muscle Nerve. 2015 Dec;52(6):1113-7. doi: 10.1002/mus.24771. Epub 2015 Jul 28.
INTRODUCTION
Amyloid myopathy is a rare manifestation of primary systemic amyloid light-chain (AL) amyloidosis, but it has not been reported to occur in secondary amyloid A (AA) amyloidosis.
METHODS
We describe a 46-year-old man with psoriasis vulgaris who presented with idiopathic upper and lower limb weakness and was eventually diagnosed with hypertrophic cardiomyopathy. Muscle biopsy findings were compatible with mild inflammatory myopathy. He died of cardiopulmonary arrest, and an autopsy was performed.
RESULTS
The autopsy revealed amyloid plaques immunopositive for AA (but not AL or transthyretin) in the perimysial, perivascular, and endomysial regions of the iliopsoas muscle. The final diagnosis was systemic AA amyloidosis with muscle amyloid angiopathy, possibly induced by psoriasis vulgaris.
CONCLUSION
This is an extremely rare autopsy case of myopathy in a patient with systemic AA amyloidosis. The reason for the unusually large amount of amyloid deposition in muscle blood vessel walls remains unclear.
引言
淀粉样变肌病是原发性系统性轻链（AL）淀粉样变性的一种罕见表现，但尚未见其在继发性淀粉样蛋白A（AA）淀粉样变性中发生的报道。
方法
我们描述了一名46岁寻常型银屑病男性患者，该患者出现特发性上下肢无力，最终被诊断为肥厚型心肌病。肌肉活检结果符合轻度炎症性肌病。他死于心肺骤停，并进行了尸检。
结果
尸检显示在髂腰肌的肌束膜、血管周围和肌内膜区域有对AA免疫阳性（而非AL或转甲状腺素蛋白）的淀粉样斑块。最终诊断为系统性AA淀粉样变性伴肌肉淀粉样血管病，可能由寻常型银屑病诱发。
结论
这是一例系统性AA淀粉样变性患者发生肌病的极其罕见的尸检病例。肌肉血管壁中淀粉样蛋白沉积异常大量的原因尚不清楚。
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