Schrager M A, Rothfield N F
J Clin Invest. 1976 Jan;57(1):212-21. doi: 10.1172/JCI108262.
61 biopsies of normal skin from the deltoid area and lesional skin from various sites from 48 patients with systemic lupus erythematosus (SLE) were studied for the presence of properdin, C3, C4, and immunoglobulins (IgG, IgM, and IgA) in the dermal-epidermal junction (DEJ) using direct and indirect immunofluorescence. Properdin was present in 50% of normal and 40% of lesional skins. Properdin was present without C4 in only 2 of 38 nonlesional skin biopsies and in only 2 of 20 lesions. There was no significant difference in incidence of deposition of any of the six proteins studied between nonlesional and lesional skin. The frequency of deposition of each of the proteins correlated with clinical disease activity. The presence of proteins in the DEJ did not correlate with the presence of active renal disease at the time of biopsy nor with previously documented active nephritis. In addition, no other single clinical manifestation correlated with the presence of DEJ deposition of any protein studied. IgA was not demonstrated in the DEJ of nonlesional skin of 16 patients in remission and was present in 7 of 23 patients with active disease (P less than 0.05). Deposition of properdin in lesional skin correlated with the presence of extracutaneous disease activity (P less than 0.05). Analysis of serologic studies on serum obtained at the time of biopsy revealed a statistically significant correlation between C4 and C3 (r = 0.67). This correlation was stronger than that between properdin and C4 (r = 0.37). Titer of antinuclear antibody and percent of DNA binding correlated better with C4 levels than with properdin levels. Serum properdin levels were significantly lower in patients with active disease than in those in remission (P less than 0.05). Serum properdin levels were significantly lower in patients with properdin deposits in lesional skin than in those without properdin deposits. The data suggest that both alternative and classical pathways are activated in patients with clinically active SLE.
对48例系统性红斑狼疮（SLE）患者三角肌区的61份正常皮肤活检标本以及来自不同部位的病损皮肤进行了研究，采用直接和间接免疫荧光法检测真皮-表皮交界处（DEJ）备解素、C3、C4及免疫球蛋白（IgG、IgM和IgA）的存在情况。备解素存在于50%的正常皮肤和40%的病损皮肤中。在38份非病损皮肤活检标本中，仅有2份备解素单独存在而无C4；在20份病损标本中也仅有2份如此。所研究的六种蛋白质中，非病损皮肤和病损皮肤中任何一种蛋白质沉积的发生率均无显著差异。每种蛋白质的沉积频率与临床疾病活动度相关。活检时DEJ处蛋白质的存在与活动性肾脏疾病的存在无关，也与既往记录的活动性肾炎无关。此外，没有其他单一临床表现与所研究的任何蛋白质在DEJ处的沉积相关。在16例缓解期患者的非病损皮肤DEJ中未检测到IgA，而在23例活动期患者中有7例存在IgA（P<0.05）。病损皮肤中备解素的沉积与皮肤外疾病活动度相关（P<0.05）。对活检时采集的血清进行血清学研究分析发现，C4与C3之间存在统计学显著相关性（r = 0.67）。这种相关性强于备解素与C4之间的相关性（r = 0.37）。抗核抗体滴度和DNA结合百分比与C4水平的相关性优于与备解素水平的相关性。活动期患者的血清备解素水平显著低于缓解期患者（P<0.05）。病损皮肤中有备解素沉积的患者血清备解素水平显著低于无备解素沉积的患者。数据表明，在临床活动期SLE患者中，替代途径和经典途径均被激活。
