Ploier R, Emhofer J, Dorninger L, Kranzl G, Feichtinger J, Müller K M, Brandtzaeg P
Kinderabteilung, Landeskrankenhaus Steyr.
Klin Padiatr. 1998 Nov-Dec;210(6):409-12. doi: 10.1055/s-2008-1043913.
A case report of a girl aged 3.5 years affected by the extremely rare combination of idiopathic pulmonary haemosiderosis (IPH) and coeliac disease (CD) is presented. It is the 13th such case that has been published over the last 25 years and only the 7th to be reported in a child. We believe that the concurrence of these two diseases is not coincidental, because a gluten-free diet had beneficial effects on the pulmonary symptoms not only in our case but also in other such patients. However, the pathogenetic relation between IPH and CD remains unclear. Although circulating immune complexes were detected in our patient's serum, there was no evidence of their putative damaging effect on the basement membrane of the alveolar capillaries. Furthermore, no IgA deposits could be demonstrated in alveolar basement membranes. Therefore the hypothesis that there is a reaction between IgA reticulin or endomysial antibodies and an alveolar basement membrane antigen with consecutive structural damage is unlikely.
本文报告了一名3.5岁女童的病例,该女童患有极为罕见的特发性肺含铁血黄素沉着症(IPH)和乳糜泻(CD)。这是过去25年中已发表的第13例此类病例,且仅第7例报告发生在儿童身上。我们认为这两种疾病的并发并非偶然,因为无麸质饮食不仅对我们的病例,而且对其他此类患者的肺部症状都有有益影响。然而,IPH和CD之间的发病机制关系仍不清楚。尽管在我们患者的血清中检测到循环免疫复合物,但没有证据表明它们对肺泡毛细血管基底膜有假定的破坏作用。此外,在肺泡基底膜中未发现IgA沉积。因此,IgA网状蛋白或肌内膜抗体与肺泡基底膜抗原之间发生反应并导致连续结构损伤的假设不太可能成立。