Kogan E A, Drozdova A S
Arkh Patol. 1989;51(10):63-7.
A case of necrotizing sarcoid-like granulomatous vasculitis (NSGV) in a female with liver cirrhosis (probably of virus etiology) complicated with systemic manifestations (skin vasculitis, syndromes of Raynaud and Sjögren). NSGV was diagnosed on the basis of the autopsy findings. Giant cell, epithelioid and macrophagal granulomas that differed from those in sarcoidosis by the presence of central necrosis, were found histologically in the walls of lung vessels and bronchi of various caliber. Keeping in mind a possible immuno-complexed pathogenesis of vasculitis in liver cirrhosis and NSGV as well as a probable viral etiology of liver cirrhosis it is likely that NSGV resulted from the vascular damage by immune complexes containing hepatitis virus antigens. Another peculiar feature of this observation was development of pulmonary hypertension.
一名患有肝硬化（可能由病毒引起）的女性发生坏死性结节样肉芽肿性血管炎（NSGV），并伴有全身表现（皮肤血管炎、雷诺综合征和干燥综合征）。NSGV根据尸检结果诊断。组织学检查发现，不同管径的肺血管壁和支气管壁存在巨细胞、上皮样和巨噬细胞肉芽肿，这些肉芽肿与结节病不同之处在于有中央坏死。考虑到肝硬化和NSGV中血管炎可能的免疫复合物发病机制以及肝硬化可能的病毒病因，NSGV可能是由含有肝炎病毒抗原的免疫复合物导致血管损伤引起的。该病例的另一个独特特征是发生了肺动脉高压。
