Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, Tokyo, Japan.
Medicine (Baltimore). 2023 Oct 13;102(41):e35470. doi: 10.1097/MD.0000000000035470.
A predominant Th2 immune response is suggested in the pathogenesis of both minimal change disease (MCD) and membranous nephropathy (MN); however, consecutive development of the 2 diseases in a patient is extremely rare.
A Japanese man, who developed nephrotic syndrome in his 50s and was diagnosed with MCD by renal biopsy, experienced a relapse of proteinuria approximately 3 years later during long-term steroid treatment. Since the proteinuria was resistant to increase in steroid dosage, repeat renal biopsy was performed, which revealed a small amount of glomerular subepithelial immune deposits containing immunoglobulin (Ig)G (dominantly IgG4). Immunostaining for thrombospondin-type-1-domain-containing-7A (THSD7A) was positive on the glomerular capillary walls, whereas that for other causative antigens of MN, such as phospholipase A2 receptor or neural epidermal growth factor-like 1 protein, was negative. Detailed examination found no associated condition, including malignancies and allergic diseases.
The diagnosis of THSD7A-associated idiopathic MN was made.
He received further increased dose of steroids. Thereafter he maintained clinical improvement because his urinary protein level was decreased.
The present case suggested that histological transition from MCD to MN is possible and repeat biopsy would be crucial for accurate diagnosis.
微小病变病(MCD)和膜性肾病(MN)的发病机制中均存在 Th2 优势免疫反应;然而,患者先后罹患这两种疾病的情况极其罕见。
一名日本男性,50 多岁时出现肾病综合征,经肾活检诊断为 MCD,在长期接受类固醇治疗后大约 3 年后出现蛋白尿复发。由于蛋白尿对类固醇剂量增加无反应,因此再次进行肾活检,结果显示肾小球上皮下有少量免疫沉积物,其中含有免疫球蛋白(Ig)G(主要为 IgG4)。肾小球毛细血管壁的血栓反应蛋白样 1 型结构域包含 7A(THSD7A)免疫染色阳性,而其他 MN 的致病抗原,如磷脂酶 A2 受体或神经表皮生长因子样 1 蛋白的免疫染色则为阴性。详细检查未发现任何相关疾病,包括恶性肿瘤和过敏性疾病。
诊断为 THSD7A 相关特发性 MN。
他接受了进一步增加剂量的类固醇治疗。此后,他的临床状况得到改善,因为他的尿蛋白水平降低了。
本病例提示从 MCD 向 MN 的组织学转变是可能的,重复进行肾活检对于准确诊断至关重要。