Lim Beom Jin, Kim Min Ju, Hong Soon Won, Jeong Hyeon Joo
Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
Severance Institute for Vascular and Metabolic Research, Yonsei University College of Medicine, Seoul, Korea.
J Pathol Transl Med. 2016 May;50(3):211-6. doi: 10.4132/jptm.2016.02.01. Epub 2016 Apr 11.
Segmental glomerulosclerosis without significant mesangial or endocapillary proliferation is rarely seen in IgA nephropathy (IgAN), which simulates idiopathic focal segmental glomerulosclerosis (FSGS). We recently recognized aberrant blood vessels running through the adhesion sites of sclerosed tufts and Bowman's capsule in IgAN cases with mild glomerular histologic change.
To characterize aberrant blood vessels in relation to segmental sclerosis, we retrospectively reviewed the clinical and histologic features of 51 cases of FSGS-like IgAN and compared them with 51 age and gender-matched idiopathic FSGS cases.
In FSGS-like IgAN, aberrant blood vessel formation was observed in 15.7% of cases, 1.0% of the total glomeruli, and 7.3% of the segmentally sclerosed glomeruli, significantly more frequently than in the idiopathic FSGS cases (p = .009). Aberrant blood vessels occasionally accompanied mild cellular proliferation surrounding penetrating neovessels. Clinically, all FSGS-like IgAN cases had hematuria; however, nephrotic range proteinuria was significantly less frequent than idiopathic FSGS.
Aberrant blood vessels in IgAN are related to glomerular capillary injury and may indicate abnormal repair processes in IgAN.
在IgA肾病（IgAN）中很少见到无明显系膜或毛细血管内增生的节段性肾小球硬化，这种情况类似于特发性局灶节段性肾小球硬化（FSGS）。我们最近在肾小球组织学改变轻微的IgAN病例中发现，异常血管穿过硬化的肾小球节段与鲍曼囊的粘连部位。
为了描述与节段性硬化相关的异常血管，我们回顾性分析了51例FSGS样IgAN的临床和组织学特征，并将其与51例年龄和性别匹配的特发性FSGS病例进行比较。
在FSGS样IgAN中，15.7%的病例观察到异常血管形成，占所有肾小球的1.0%，节段性硬化肾小球的7.3%，明显高于特发性FSGS病例（p = 0.009）。异常血管偶尔伴有围绕穿透性新生血管的轻度细胞增殖。临床上，所有FSGS样IgAN病例均有血尿；然而，肾病范围蛋白尿的发生率明显低于特发性FSGS。
IgAN中的异常血管与肾小球毛细血管损伤有关，可能提示IgAN中的异常修复过程。
