近端肌病表现为全身性肌无力,通常累及上肢和/或下肢肌肉。毒素,长期使用他汀类药物,皮质类固醇,酒精,SGLT2抑制剂,COVID-19疫苗接种,抗疟药被归因于它的发展。在内分泌和代谢紊乱中,据报道,包括肾上腺激素过度分泌和不足在内的肾上腺功能障碍可导致肌病。此外,甲状旁腺和甲状腺疾病以及垂体疾病也可以直接或间接导致这种情况。在特发性炎性肌病,包括多发性肌炎,皮肌炎,包涵体肌炎(IBM),和系统性红斑狼疮(SLE),干燥综合征,和重叠综合征,已观察到中度至重度肌肉无力。据报道,IBM是50岁以上最普遍的获得性肌病。遗传性或先天性肌病包括四肢带肌营养不良,面肩肱肌营养不良,Duchenne和Becker肌营养不良症,和近端强直性肌病。除了这些,糖原贮积病如McArdle病也会导致快速耗尽,肌痛,工作肌肉抽筋。这里有必要提到一类遗传性代谢性肌病,称为“脂质沉积肌病”导致脂质在骨骼肌纤维中积聚,导致病变和变性。在病毒原因中,艾滋病毒,登革热病毒,流感病毒,乙型肝炎病毒,丙型肝炎病毒,SARS-CoV2也与肌肉无力有关。结节病,炎症性疾病,也可以表现为肌肉无力和肌痛。由于近端肌病的这种复杂的病理生理学,这篇综述旨在总结与这一现象相关的现有文献,以及其他有关导致广泛性肌无力发展的事件的最新进展。据作者所知,这是第一次详细讨论与近端肌病相关的原因和条件的叙述性综述。
Proximal myopathy presents as generalized muscle weakness commonly involving the muscles of upper and/or lower limbs. Toxins, long-term use of statins, corticosteroids, alcohol, SGLT2 inhibitors, COVID-19 vaccination, and antimalarials have been attributed to its development. In endocrine and metabolic disorders, adrenal dysfunction including both overproduction and insufficiency of the adrenal gland hormones has been reported to cause myopathy. Moreover, parathyroid and thyroid disorders along with pituitary gland disorders can also directly or indirectly contribute to this condition. In idiopathic inflammatory
myopathies including polymyositis, dermatomyositis, inclusion body myositis (IBM), and Systemic Lupus Erythematosus (SLE), Sjögren\'s Syndrome, and overlap syndromes, moderate to severe muscle weakness has been observed. IBM has been reported to be the most prevalent acquired myopathy above the age of 50. Hereditary or congenital
myopathies include limb girdle muscular dystrophies, facioscapulohumeral muscular dystrophy, Duchenne and Becker muscular dystrophy, and proximal myotonic myopathy. In addition to these, glycogen storage diseases such as the McArdle disease can also cause fast exhaustion, myalgia, and cramping in working muscles. It is pertinent to mention here that a class of hereditary metabolic
myopathies, referred to as \"lipid deposition myopathy\" causes lipids to accumulate in skeletal muscle fibers, leading to lesions and degeneration. Among viral causes, HIV, dengue virus, influenza virus, hepatitis B virus, hepatitis C virus, SARS-CoV2 are also associated with muscle weakness. Sarcoidosis, an inflammatory disease, can also manifest as muscle weakness and myalgia. Owing to this complicated pathophysiology of proximal myopathy, this review aims to summarize the existing literature on conditions associated with this phenomenon and other recent developments that have been made regarding events leading to development of generalized muscle weakness. To the authors\' knowledge this is the first narrative review that discusses causes and conditions associated with proximal myopathy in thorough detail.