Manetti Mirko, Milia Anna Franca, Benelli Gemma, Messerini Luca, Matucci-Cerinic Marco, Ibba-Manneschi Lidia
Department of Anatomy, Histology and Forensic Medicine, University of Florence, Florence, Italy.
Ital J Anat Embryol. 2010;115(1-2):115-21.
Organ failure secondary to fibrosis is the main cause of morbidity and death in patients with systemic sclerosis. Gastrointestinal tract dysmotility is a major visceral manifestation, clinically ranging from an asymptomatic form to severe paresis. Although the oesophagus is the most frequently affected part of the gastrointestinal tract, all other segments can be involved. The present study was undertaken to evaluate the histopathological changes of the gastric wall in a series of full-thickness biopsies from systemic sclerosis patients who underwent gastric surgery due to severe gastroesophageal involvement. Gastric biopsies were processed for light microscopy and transmission electron microscopy. The histological and ultrastructural observations revealed a generalized fibrosis affecting all the gastric wall layers. The most severe changes were observed in the muscularis mucosae and muscle layers. Wide areas of marked focal fibrosis with dense collagen bundles and elastic fibre deposition surrounding smooth muscle cells were found. Myofilaments and thickened dense bodies were severely disarranged or absent in most smooth muscle cells. Nerve fibres showed ultrastructural alterations, such as oedematous axoplasm and scarce cytoskeletal elements. Abundant elastic and collagen fibres enveloped nerve fibres, nerve endings and interstitial cells of Cajal, thereby separating them from smooth muscle cells and blood microvessels. This study provides evidence for a prominent fibrosis and severe ultrastructural alterations of smooth muscle cells and nerve fibres as the main histopathological hallmarks in the gastric wall of systemic sclerosis patients.
继发于纤维化的器官衰竭是系统性硬化症患者发病和死亡的主要原因。胃肠道动力障碍是主要的内脏表现，临床上从无症状形式到严重麻痹不等。尽管食管是胃肠道最常受累的部位，但其他所有节段也可能受累。本研究旨在评估一系列因严重胃食管受累而接受胃手术的系统性硬化症患者全层活检标本中胃壁的组织病理学变化。对胃活检标本进行光镜和透射电镜检查。组织学和超微结构观察显示，全层胃壁均有广泛纤维化。黏膜肌层和肌层的变化最为严重。发现广泛区域有明显的局灶性纤维化，伴有密集的胶原束和围绕平滑肌细胞的弹性纤维沉积。大多数平滑肌细胞中的肌丝和致密小体严重紊乱或缺失。神经纤维显示超微结构改变，如轴浆水肿和细胞骨架成分稀少。丰富的弹性纤维和胶原纤维包裹着神经纤维、神经末梢和 Cajal 间质细胞，从而使它们与平滑肌细胞和血液微血管分离。本研究为系统性硬化症患者胃壁中明显的纤维化以及平滑肌细胞和神经纤维的严重超微结构改变提供了证据，这些是主要的组织病理学特征。
