Gabrielli A, Sbarbati A, Marchegiani G, Rupoli S, Montroni M, Cinti S, Danieli G
Arthritis Rheum. 1987 Aug;30(8):884-93. doi: 10.1002/art.1780300807.
To gain insight into the pathogenic mechanisms of immune complex-mediated vasculitis, clinically normal skin specimens from the forearms of 12 patients with essential mixed cryoglobulinemia were investigated by light microscopy, immunofluorescence microscopy, and electron microscopy. Basement membrane alterations were documented in 9 patients. Eleven patients had deposits in vessel walls, but only in 1 was there evidence of inflammation. The same immunoglobulins of the cryoproteins could be demonstrated in the tissue deposits by immunofluorescence analysis and by immunoelectron microscopy. These findings suggest that, in essential mixed cryoglobulinemia, immune reactants in dermal vessels of normal skin are more common than is evidenced by clinical examination. Moreover, it seems that cryoglobulins, as such, are not sufficient to trigger an inflammatory process: Additional local or plasma factors are required. Deposits were absent in 7 patients with cryoglobulinemia that was associated with a primary disorder. This could be ascribed to the lower cryocrit levels documented in this group of patients, or to the shorter duration of their disease.
为深入了解免疫复合物介导的血管炎的致病机制,我们通过光学显微镜、免疫荧光显微镜和电子显微镜对12例原发性混合性冷球蛋白血症患者前臂临床上正常的皮肤标本进行了研究。9例患者有基底膜改变。11例患者血管壁有沉积物,但只有1例有炎症迹象。通过免疫荧光分析和免疫电子显微镜可在组织沉积物中证实冷球蛋白的相同免疫球蛋白。这些发现表明,在原发性混合性冷球蛋白血症中,正常皮肤真皮血管中的免疫反应物比临床检查所显示的更为常见。此外,似乎冷球蛋白本身不足以引发炎症过程:还需要其他局部或血浆因子。7例与原发性疾病相关的冷球蛋白血症患者没有沉积物。这可能归因于该组患者记录的较低冷沉淀比容水平,或其疾病持续时间较短。