Unit of Nephrology, Dialysis and Transplantation, Department of Experimental Diagnostic and Specialty Medicine, University of Bologna, S. Orsola Malpighi Hospital, Via G. Massarenti 9 (Pad. 15), 40138, Bologna, Italy.
Unit of Oncology and Transplant Pathology, Department of Experimental Diagnostic and Specialty Medicine, University of Bologna, S. Orsola Malpighi Hospital, Via G. Massarenti 9 (Pad. 18), 40138, Bologna, Italy.
BMC Nephrol. 2019 Nov 21;20(1):418. doi: 10.1186/s12882-019-1587-4.
IgG4-related disease, described around the years 2000 as a form of autoimmune pancreatitis, is now increasingly accepted as a systemic syndrome. The diagnosis is based on both comprehensive and organ-specific criteria. For the kidney, Mayo clinic classification and the guidelines of the Japanese Nephrology Society are used. Ultimately, together with parameters that characterize every organ or apparatus involved, the key element is the confirmation of growing levels of IgG4 in blood or in tissues.
We describe a male patient with chronic renal failure associated to hypertension without proteinuria. IgG4-related disease was diagnosed through renal biopsy. After an initial positive response to steroids, he presented tinnitus, and histological assessment showed cerebral and subsequently cardiac damage, both IgG4-related. This case appears unique for the type of histologically documented cardiac and neurological parenchymal involvement, and at the same time, exemplifies the subtle and pernicious course of the disease. Frequently, blurred and non-specific signs prevail. Here, kidney damage was associated with minimal urinary findings, slowly progressive renal dysfunction and other factors that can be equivocated in the differential diagnosis. Neurological involvement was represented by tinnitus alone, while cardiac alterations were completely asymptomatic.
This report is representative of the neurological and cardiac changes described in the literature for IgG4-related disease, which may be correlated or not with the renal form and highlights the need, in some cases, of targeted therapeutic approaches. In addition to glucocorticoids, as in this case, rituximab may be necessary.
IgG4 相关疾病于 2000 年前后被描述为一种自身免疫性胰腺炎形式，现在被越来越多地认为是一种系统性综合征。该诊断基于综合和器官特异性标准。对于肾脏，使用梅奥诊所分类和日本肾脏病学会指南。最终，与每个涉及的器官或装置的特征参数一起，关键要素是确认血液或组织中 IgG4 水平的升高。
我们描述了一名患有慢性肾衰竭伴高血压但无蛋白尿的男性患者。通过肾活检诊断为 IgG4 相关疾病。在最初对类固醇有阳性反应后，他出现了耳鸣，组织学评估显示脑损伤，随后出现心脏损伤，均与 IgG4 相关。这种情况在组织学记录的心脏和神经实质受累的类型上是独特的，同时也体现了疾病的微妙和有害的过程。通常，模糊和非特异性迹象占主导地位。在这里，肾脏损伤与最小的尿液发现、肾功能逐渐恶化以及其他在鉴别诊断中可能存在混淆的因素相关。神经系统受累仅表现为耳鸣，而心脏改变则完全无症状。
本报告代表了 IgG4 相关疾病文献中描述的神经和心脏变化，这些变化可能与肾脏形式相关或不相关，并强调了在某些情况下需要靶向治疗方法。除了糖皮质激素，如本例中，利妥昔单抗可能也是必要的。
