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狼疮患者皮肤中甘露糖结合凝集素的沉积:病例系列

Mannose binding lectin deposition in skin of lupus erythematosus patients: a case series.

作者信息

Wallim Liz Ribeiro, Nisihara Renato, Skare Thelma, Mocelin Valmir, Messias-Reason Iara J

机构信息

Medicine Department, Pontifical Catholic University of Paraná, Curitiba, Paraná, Brazil; Rheumatology Unit, Hospital Universitário Evangélico de Curitiba, Brazil.

Medicine Department, Positivo University, Curitiba, Paraná, Brazil.

出版信息

Hum Immunol. 2014 Jul;75(7):629-32. doi: 10.1016/j.humimm.2014.04.015. Epub 2014 May 4.

Abstract

INTRODUCTION

Mannose binding lectin (MBL) has been linked to predisposition to systemic lupus erythematosus (SLE) and to disease activity. Some studies found deposits of MBL in glomerular tissue of patients with lupus nephritis. There is no research about the deposition of MBL in skin.

MATERIALS AND METHODS

Skin biopsies from lesional and non lesional skin of 4 discoid lupus erythematosus (DLE) and 10 SLE patients were submitted to immunofluorescence staining for IgG, IgA, IgM, C3, C4, C1q, C5b-9 and MBL. Charts were reviewed for demographic, clinical and serological data. Patients with SLE had disease activity measured by SLEDAI.

RESULTS

MBL was found only in SLE lesional skin and its presence showed an association trend towards higher disease activity. Deposition of C5b-9 occurred in vessels only in patients with SLE (70%) and in the two patients with kidney involvement.

CONCLUSIONS

MBL deposition was found in the lesional skin of SLE patients but not in SLE non lesional skin nor in DLE patients, and it seems to be less frequent and less strong than observed in the kidneys biopsies, suggesting that the complement participation in the pathophysiology of SLE process may not be the same in these two clinical manifestations.

摘要

引言

甘露糖结合凝集素(MBL)与系统性红斑狼疮(SLE)的易感性及疾病活动度有关。一些研究发现狼疮性肾炎患者肾小球组织中有MBL沉积。目前尚无关于MBL在皮肤中沉积的研究。

材料与方法

对4例盘状红斑狼疮(DLE)患者和10例SLE患者的皮损及非皮损皮肤进行活检,进行IgG、IgA、IgM、C3、C4、C1q、C5b - 9和MBL的免疫荧光染色。查阅病历以获取人口统计学、临床和血清学数据。SLE患者的疾病活动度通过SLEDAI进行评估。

结果

仅在SLE皮损皮肤中发现MBL,其存在与较高的疾病活动度呈关联趋势。C5b - 9仅在SLE患者(70%)及2例有肾脏受累的患者的血管中沉积。

结论

在SLE患者的皮损皮肤中发现了MBL沉积,但在SLE非皮损皮肤及DLE患者中未发现,且其似乎比在肾脏活检中观察到的频率更低、强度更弱,这表明补体在SLE这两种临床表现的病理生理学中的参与情况可能不同。

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