Malandrini A, Selvi E, Villanova M, Berti G, Sabadini L, Salvadori C, Gambelli S, De Stefano R, Vernillo R, Marcolongo R, Guazzi G
Institute of Neurological Sciences, University of Siena, Italy.
J Rheumatol. 2000 May;27(5):1203-6.
To investigate morphological abnormalities in nerve and smooth muscle structures of the anorectal wall underlying gastrointestinal dysfunction in patients with systemic sclerosis (SSc).
We performed deep rectal biopsy in 3 patients with limited scleroderma of relatively recent onset and intestinal symptoms.
We found ultrastructural signs of axonal degeneration and cytoskeletal abnormalities in the bundles of unmyelinated fibers. There was also focal degeneration of smooth muscle cells, often in association with the presence of partially degranulated mast cells. Many mast cells were also observed in close relation to nerve fibers and vessels. The enteric vessels often showed basal lamina reduplication and hypertrophied endothelial cells with obliterated lumen. No significant fibrosis was found.
Our findings indicate early involvement of the autonomic nervous system and to a lesser extent of smooth muscle cells. We confirmed the presence of early vascular lesions and involvement of mast cells in the pathological process.
研究系统性硬化症（SSc）患者胃肠道功能障碍时肛门直肠壁神经和平滑肌结构的形态学异常。
我们对3例近期发病且有肠道症状的局限性硬皮病患者进行了直肠深层活检。
我们在无髓纤维束中发现了轴突变性和细胞骨架异常的超微结构迹象。平滑肌细胞也有局灶性变性，常伴有部分脱颗粒肥大细胞的存在。还观察到许多肥大细胞与神经纤维和血管关系密切。肠血管常显示基底膜重复和内皮细胞肥大，管腔闭塞。未发现明显纤维化。
我们的研究结果表明自主神经系统早期受累，平滑肌细胞受累程度较轻。我们证实了早期血管病变的存在以及肥大细胞参与了病理过程。
