Habib R, Beziau A, Goulet O, Blanche S, Niaudet P
INSERM U192, Hôpital Necker-Enfants Malades, Paris.
Ann Pediatr (Paris). 1993 Feb;40(2):103-7.
The authors report on a infant who presented with an auto-immune enteropathy characterized by the association of a protracted diarrhea, a neonatal insulin-dependent diabetes, and a dermatitis and who developed a nephrotic syndrome at 4 months of age. A renal biopsy showed a membranous glomerulonephritis (MGN) with IgG linear deposits along the tubular basement membranes (TMB). By indirect immunofluorescence anti-enterocyte antibodies together with anti-TMB antibodies and anti-renal brush border (BB) antibodies were found in the serum of the patient. The patient received various immunosuppressive drugs that failed to improve the disease. In the course of the disease the anti-TBM antibodies disappeared progressively but the BB antibodies persisted. A review of the literature indicates that renal involvement is not uncommon in auto-immune enteropathy and in 5 cases it has been reported as being characterized by a nephrotic syndrome related to the presence of a MGN. In 4 of these cases MGN was associated with the presence of anti-TBM antibodies and in the remaining one with anti-BB antibodies. This case report shows that in human pathology, auto-antibodies to BB proteins may, as well as in experimental models, be responsible for the development of a MGN. It suggests a close relationship (probably a common epitope) between the renal BB proteins and the proteins of the gut epithelium.
作者报告了一名患有自身免疫性肠病的婴儿，其特征为持续性腹泻、新生儿胰岛素依赖型糖尿病和皮炎，并在4个月大时发展为肾病综合征。肾活检显示为膜性肾小球肾炎（MGN），沿肾小管基底膜（TMB）有IgG线性沉积。通过间接免疫荧光法，在患者血清中发现了抗肠上皮细胞抗体以及抗TMB抗体和抗肾刷状缘（BB）抗体。患者接受了多种免疫抑制药物治疗，但病情未改善。在疾病过程中，抗TBM抗体逐渐消失，但BB抗体持续存在。文献综述表明，肾脏受累在自身免疫性肠病中并不少见，已有5例报告其特征为与MGN相关的肾病综合征。在其中4例中，MGN与抗TBM抗体的存在有关，在其余1例中与抗BB抗体有关。本病例报告表明，在人类病理学中，针对BB蛋白的自身抗体可能与实验模型一样，是MGN发生的原因。这表明肾BB蛋白与肠上皮蛋白之间存在密切关系（可能是共同表位）。
