Longy M, Beylot J, Beylot C, Veyret V, Moretti G
Sem Hop. 1979;55(15-16):762-8.
Renal lesions are one of the must serious visceral complications of progressive systemic sclerosis: three of the five cases reported died in anuria within a few weeks. A part from this very severe fulminating form, however, cases in which lesions can be demonstrated by pathological examination may produce much milder clinical and biological signs, though still having the same prognostic significance. They can become worse at any moment, either spontaneously, or more often because of other factors such as corticotherapy or pregnancy. A review of the published literature has shown the exact frequency of these lesions and their two principal modes of progression, in both of which the same basic histological changes of proliferation of the intima of the interlobular and preglomerular arteries are found. Kidney function tests can be used to guage the severity and extent of these vascular lesions. It is much more difficult, however, to determine the nature of the initial mechanism causing these lesions. The vascular lesion could be a primary one or may be secondary to an immunological disorder, the exact nature of which has still to be determined.
所报道的5例中有3例在几周内死于无尿。然而，除了这种非常严重的暴发型之外，通过病理检查可证实有病变的病例可能产生更轻微的临床和生物学体征，尽管仍具有相同的预后意义。它们随时可能恶化，要么自发恶化，要么更常见的是由于其他因素，如皮质激素治疗或妊娠。对已发表文献的回顾表明了这些病变的确切发生率及其两种主要进展方式，在这两种方式中都发现了小叶间动脉和入球小动脉内膜增生相同的基本组织学变化。肾功能检查可用于评估这些血管病变的严重程度和范围。然而，要确定引起这些病变的初始机制的性质则困难得多。血管病变可能是原发性的，也可能继发于免疫紊乱，其确切性质仍有待确定。
