Richet G, Mougenot B, Roland J
Ann Med Interne (Paris). 1984;135(8):594-600.
Renal biopsies were carried out in 29 patients with scleroderma to study the early vascular lesions and their eventual clinical significance. Haemolytic acute renal failure was present in 9 patients. The biopsies showed early vascular lesions on interlobar arteries. The main biological change was proliferative or fibrous endarteritis. Mucoid infiltration was found in 3 biopsies. The arterioles were spared or only slightly affected without major fibrinoid necrosis. These lesions were therefore distinct from those of malignant hypertension. However, at autopsy of 2 of these cases, the vascular lesions were undistinguishable from those of malignant hypertension. The biopsies in 13 out of 14 patients with scleroderma without obvious renal involvement (9 cases) or with moderate proteinuria and/or hypertension without renal failure (5 cases) showed interlobar endarteritis with associated mucoid infiltration in 3 patients. This lesion was isolated but sometimes extensive even in young patients without hypertension. One patient died within one year, of disseminated colonic carcinoma, and 4 of cardio-respiratory failure due to scleroderma without hypertension, renal failure or proteinuria. Eight of the 9 remaining patients were traced 6 to 16 years after biopsy. Two were moderately hypertensive but none had renal failure. Cutaneous and internal organ scleroderma had regressed in the majority and proteinuria had disappeared in 3 cases.
对29例硬皮病患者进行了肾活检,以研究早期血管病变及其最终的临床意义。9例患者出现溶血性急性肾衰竭。活检显示叶间动脉有早期血管病变。主要的生物学变化是增殖性或纤维性动脉内膜炎。3例活检发现有黏液样浸润。小动脉未受累或仅轻微受累,无明显的纤维蛋白样坏死。因此,这些病变与恶性高血压的病变不同。然而,在其中2例患者的尸检中,血管病变与恶性高血压的病变无法区分。14例无明显肾脏受累(9例)或有中度蛋白尿和/或高血压但无肾衰竭(5例)的硬皮病患者中,13例的活检显示叶间动脉内膜炎,3例伴有黏液样浸润。即使在无高血压的年轻患者中,这种病变也是孤立的,但有时范围广泛。1例患者在1年内死于播散性结肠癌,4例死于硬皮病导致的心肺衰竭,无高血压、肾衰竭或蛋白尿。其余9例患者中有8例在活检后6至16年接受了随访。2例有中度高血压,但均无肾衰竭。大多数患者的皮肤和内脏硬皮病已消退,3例患者的蛋白尿消失。