Golovach I Y, Yehudina Y D
Feofaniya Clinical hospital State Management of Affairs.
Clinic of modern rheumatology.
Ter Arkh. 2019 Dec 15;91(12):63-69. doi: 10.26442/00403660.2019.12.000206.
Vasculitis is a clinically diverse group of diseases with histopathological signs of blood vessel inflammation, which contributes to vascular damage and ischemic damage to the affected tissues. Vasculitic neuropathy is a common complication of the primary systemic vasculitides, such as polyartertis nodosa and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, systemic diseases of the connective tissue - systemic lupus erythematosus and Sjogren syndrome, vasculitis associated with infection, most often viral hepatitis C and B and non - systemic vasculitis neuropathy. Vessels of medium and small caliber are involved in the pathological process in these diseases. With all vasculitis, except for those caused by the direct effect of the infectious trigger on the blood vessel walls, the main pathogenetic mechanism is an autoimmune process with the development of vasa nervorum vasculitis - small arteries and vessels that supply peripheral nerves, and the outcome - nerve ischemia. The classic clinical presentation is an acute or subacute painful multifocal neuropathy that has a predilection for the lower extremities, affects two or more named nerves, and progresses in a step wise manner. However, vasculitic neuropathy can manifest in a variety of ways, including asymmetric polyneuropathies and distal symmetric sensory neuropathies, and it also can be slowly progressive, particularly in cases of nonsystemic vasculitic neuropathy (NSVN), a form of vasculitis that clinically remains restricted to peripheral nerves. Nerve biopsy can help establish the diagnosis of a systemic vasculitis, particularly when other organ involvement is not clinically apparent, and is required for diagnosis of NSVN. Neuropathy due to systemic vasculitis should be treated in accordance with the recommendations for the treatment of the underlying disease. In NSVH, the main medicine of choice are glucocrticoids, and in severe/progressive cases, pulse therapy with cyclophosphamide.
血管炎是一组临床症状多样的疾病，具有血管炎症的组织病理学特征，可导致血管损伤以及对受影响组织的缺血性损伤。血管炎性神经病是原发性系统性血管炎的常见并发症，如结节性多动脉炎和抗中性粒细胞胞浆抗体（ANCA）相关血管炎、结缔组织系统性疾病——系统性红斑狼疮和干燥综合征、与感染相关的血管炎（最常见的是丙型和乙型病毒性肝炎）以及非系统性血管炎性神经病。这些疾病的病理过程累及中小口径血管。除了由感染性触发因素直接作用于血管壁引起的血管炎外，所有血管炎的主要发病机制都是自身免疫过程，伴有营养血管炎（供应周围神经的小动脉和血管）的发展，其结果是神经缺血。典型的临床表现是急性或亚急性疼痛性多灶性神经病，以下肢为好发部位，累及两条或更多条特定神经，并呈阶梯式进展。然而，血管炎性神经病可以有多种表现形式，包括不对称性多发性神经病和远端对称性感觉神经病，并且也可以缓慢进展，特别是在非系统性血管炎性神经病（NSVN）的情况下，这是一种临床上仅局限于周围神经的血管炎形式。神经活检有助于确立系统性血管炎的诊断，特别是当其他器官受累在临床上不明显时，并且是诊断NSVN所必需的。系统性血管炎所致的神经病应根据基础疾病的治疗建议进行治疗。在NSVH中，主要的治疗药物是糖皮质激素，在严重/进展性病例中，采用环磷酰胺冲击治疗。
