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[连续性结缔组织病(硬皮病,继而是狼疮)。4例临床及生物学研究]

[Successive connective tissue diseases (scleroderma, then lupus). Clinical and biological study of 4 cases].

作者信息

Kahn M F, Peltier A P, Degraeve B, Mery J P, Morel-Maroger L, de Seze S

出版信息

Ann Med Interne (Paris). 1977 Jan;128(1):1-8.

PMID:301001
Abstract

The authors present 4 patients who had suffered for several years from isolated systemic scleroderma and in whom, after 3, 10, 19 and 22 years, clinical, biological and histological signs of DLE appeared. There was no current further progression of the scleroderma in two cases, at the time of onset of DLE. In the oter two cases, it had disappeared. All four patients had a serum cryoglobulin. In all cases the kidneys were involved histologically : extra membranous glomerulitis in one, segmental glomerulitis in a second, and diffuse progressive glomerulitis in the last two. The authors analyse the biological changes which accompanied the clinical transformation. One of the patients had anti-ribonucleoprotein antibodies. She died from severe renal involvement which would be against the good prognosis usually associated with the presence of this type of antibody.

摘要

作者报告了4例患有局限性系统性硬皮病数年的患者,在3年、10年、19年和22年后,出现了盘状红斑狼疮的临床、生物学和组织学特征。在两例患者中,盘状红斑狼疮发病时,硬皮病无进一步进展。另外两例中,硬皮病已消失。所有4例患者均有血清冷球蛋白。所有病例肾脏均有组织学受累:1例为膜性肾小球肾炎,第2例为节段性肾小球肾炎,最后两例为弥漫性进行性肾小球肾炎。作者分析了伴随临床转变的生物学变化。其中1例患者有抗核糖核蛋白抗体。她死于严重的肾脏受累,而通常认为这种抗体的存在预后良好。

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