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Fixed digital contractures revealing light-chain amyloidosis.

机译:固定的数字挛缩揭示轻链淀粉样变性。

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摘要

Among the many clinical manifestations of light-chain (AL) amyloidosis, musculoskeletal involvement is rarely reported. We describe the case of a 72-year-old woman who was referred to our rheumatology department for fixed flexion contractures of the fingers that developed concomitantly with a decline in general health. Macroglossia and recent-onset dyspnea were noted. Investigations, which included a tongue biopsy, established the diagnosis of kappa light-chain amyloidosis with soft-tissue, bone and cardiac deposits. Melphalan and dexamethasone therapy was successful in stabilizing the clinical and laboratory abnormalities within 6 months. This case is remarkable in that the musculoskeletal manifestations were at the forefront of the clinical picture and led to the diagnosis.
机译:在轻链(AL)淀粉样变性的许多临床表现中,鲜有肌肉骨骼受累的报道。我们描述了一个72岁的女人的情况,该女人因固定的手指屈曲挛缩症而转诊至我们的风湿病科,而手指的屈曲挛缩伴随着整体健康状况的下降而发展。注意到巨眼症和最近发作的呼吸困难。包括舌头活检在内的调查确定了具有软组织,骨骼和心脏沉积物的κ轻链淀粉样变性病的诊断。美法仑和地塞米松疗法在6个月内成功稳定了临床和实验室异常。该病例的突出之处在于肌肉骨骼的表现处于临床表现的最前沿,并导致了诊断。

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