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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Which complement assays and typings are necessary for the diagnosis of complement deficiency in patients with lupus erythematosus? A study of 25 patients.
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Which complement assays and typings are necessary for the diagnosis of complement deficiency in patients with lupus erythematosus? A study of 25 patients.

机译：对于红斑狼疮患者，补体缺乏症的诊断需要哪些补体测定法和类型？一项针对25位患者的研究。

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INTRODUCTION: Deficiencies in components of the classical pathway of complement activation are strong risk factors for lupus erythematosus (LE).Yet, it has not been addressed whether the conventional measurements of the serum hemolytic CH50 activity and antigenic concentrations of C3 and C4 are sufficient to asses a deficiency in C4A, C4B or C2 components, the most common deficiencies associated with LE. PATIENTS AND METHODS: In a retrospective series, we performed complement analyses in 35 patients with LE who were systematically screened for a complement deficiency. The majority of patients had cutaneous LE with mild systemic involvement and no complement consumption. Of 25 patients (72%) with complement deficiency we found 13 with a partial C4A deficiency, 2 with a complete C4A deficiency, 6 with a partial C4B deficiency, 2 with a complete C4B deficiency and 2 with a combined partial C2 and C4A deficiency. RESULTS: The total complement activity (CH50) was decreased in only one out of two patients with complete C4B deficiency. CH50 level was found to be low-normal (35-38 U/ml(-1)) in one patient with partial C4B deficiency, one patient with complete C4B deficiency and both patients with combined partial C4A and C2 deficiency. Total C4 levels were normal in 9 out of 13 the patients with a partial C4A deficiency and in 2 out of 6 patients with a complete C4B deficiency. The antigenic concentration of C3 was low in only 1 patients with a complete C4B deficiency and within the normal range in all the others patients. Overall, 50% of the patients had normal or elevated C3, C4, and CH50 levels. DISCUSSION: This study emphasizes that the usual measurements of CH50, C3 and C4 levels are not adequate to detect a C4 and/or C2 deficiency in patients with LE. In epidemiologic or investigative studies addressing the prevalence of complement deficiency, more elaborated diagnostic tests, such as C4 protein allotyping, C2 level measurement and genetic screening for type I C2 deficiency should also be performed.
机译：简介：经典补体激活途径中的成分缺乏是红斑狼疮（LE）的强大危险因素，但尚未解决常规的血清溶血CH50活性以及C3和C4抗原浓度的常规测量是否足以评估C4A，C4B或C2组件的缺陷，这是与LE相关的最常见缺陷。病人和方法：在一项回顾性研究中，我们对35例经系统筛查补体缺乏症的LE患者进行了补体分析。大多数患者患有皮肤性LE，全身轻度受累，没有补充补体。在25名补体缺乏症患者中（72％），我们发现13名患有部分C4A缺乏症，2名患有完全C4A缺乏症，6名患有部分C4B缺乏症，2名患有完全C4B缺乏症以及2名合并了部分C2和C4A缺乏症。结果：在完全C4B缺乏的两名患者中，只有一名患者的总补体活性（CH50）降低。在一名部分C4B缺乏症患者，一名完全C4B缺乏症患者以及两名部分C4A和C2合并合并症患者中，CH50水平被发现为低正常水平（35-38 U / ml（-1））。在部分C4A缺乏的13位患者中，有9位的C4总水平正常，而在完全C4B缺乏的6位患者中，有2位的总C4水平正常。仅1名完全C4B缺乏的患者中C3的抗原浓度较低，其他所有患者均在正常范围内。总体而言，有50％的患者C3，C4和CH50水平正常或升高。讨论：本研究强调，通常的CH50，C3和C4水平测量不足以检测LE患者的C4和/或C2缺乏症。在针对补体缺乏症患病率的流行病学或调查研究中，还应进行更详尽的诊断测试，例如C4蛋白同种分型，C2水平测量和I型C2缺乏症的基因筛查。

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