Hunner-Type (Classic) Interstitial Cystitis: A Distinct Inflammatory Disorder Characterized by Pancystitis, with Frequent Expansion of Clonal B-Cells and Epithelial Denudation.
Hunner-Type (Classic) Interstitial Cystitis: A Distinct Inflammatory Disorder Characterized by Pancystitis, with Frequent Expansion of Clonal B-Cells and Epithelial Denudation.
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DOI：
10.1371/journal.pone.0143316
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发表时间：
2015
期刊：
影响因子：
3.7
通讯作者：
Homma Y
中科院分区：
文献类型：
--
作者：
Maeda D;Akiyama Y;Morikawa T;Kunita A;Ota Y;Katoh H;Niimi A;Nomiya A;Ishikawa S;Goto A;Igawa Y;Fukayama M;Homma Y
间质性膀胱炎 (IC) 是一种慢性膀胱疾病，伴有尿频、膀胱不适或病因不明的膀胱疼痛。根据膀胱镜检查结果，IC 患者分为 Hunner 型/经典 IC (HIC)（表现为特定的 Hunner 病变）或非 Hunner 型 IC (NHIC)（表现为无 Hunner 病变，但有水扩张后粘膜出血）。主要由淋巴细胞、浆细胞和上皮剥脱组成的炎症细胞浸润在过去被记录为主要的病理学发现。然而，近年来IC病理学评估的意义，尤其是HIC与NHIC之间的差异，已被淡化。在本研究中，我们对浸润性 T 淋巴细胞、B 淋巴细胞和浆细胞进行了免疫组织化学定量，并使用图像分析软件测量了 HIC 和 NHIC 患者以及无 IC 患者的膀胱活检标本中残留上皮的数量。此外，还进行了轻链原位杂交以检查克隆 B 细胞的扩增。 HIC 标本中的淋巴浆细胞浸润比 NHIC 标本中的淋巴浆细胞浸润明显更严重（P <0.0001）。 93% 的 HIC 标本中观察到明显的淋巴浆细胞炎症（≥200 个细胞/mm2），而只有 8% 的 NHIC 标本出现炎症。与 NHIC 和非 IC 膀胱炎标本相比，HIC 标本中浆细胞浸润更为突出（P <0.005）。此外，在 31% 的 HIC 病例中观察到轻链限制性 B 细胞的扩增。与NHIC标本和非IC膀胱炎标本相比，HIC标本中残留上皮的数量减少（P <0.0001）。这些结果表明，NHIC 和 HIC 是不同的病理实体，后者的特征是胰腺炎、频繁的克隆性 B 细胞扩增和上皮剥脱。 B 细胞群异常可能与 HIC 的发病机制有关。
Interstitial cystitis (IC) is a chronic bladder disease with urinary frequency, bladder discomfort or bladder pain of unknown etiology. Based on cystoscopic findings, patients with IC are classified as either Hunner-type/classic IC (HIC), presenting with a specific Hunner lesion, or non-Hunner-type IC (NHIC), presenting with no Hunner lesion, but post-hydrodistension mucosal bleeding. Inflammatory cell infiltration, composed predominantly of lymphocytes, plasma cells and epithelial denudation, has in the past been documented as a major pathological IC finding. However, the significance of the pathological evaluation of IC, especially with regard to the difference between HIC and NHIC, has been downplayed in recent years. In this study, we performed immunohistochemical quantification of infiltrating T-lymphocytes, B-lymphocytes and plasma cells, and measured the amount of residual epithelium in urinary bladder biopsy specimens taken from patients with HIC and NHIC, and those with no IC, using image analysis software. In addition, in situ hybridization of the light chains was performed to examine clonal B-cell expansion. Lymphoplasmacytic infiltration was significantly more severe in HIC specimens than in NHIC specimens (P <0.0001). Substantial lymphoplasmacytic inflammation (≥200 cells/mm2) was observed in 93% of HIC specimens, whereas only 8% of NHIC specimens were inflamed. Plasmacytic infiltration was more prominent in HIC specimens compared with NHIC and non-IC cystitis specimens (P <0.005). Furthermore, expansion of light-chain-restricted B-cells was observed in 31% of cases of HIC. The amount of residual epithelium was decreased in HIC specimens compared with NHIC specimens and non-IC cystitis specimens (P <0.0001). These results suggest that NHIC and HIC are distinct pathological entities, with the latter characterized by pancystitis, frequent clonal B-cell expansion and epithelial denudation. An abnormality in the B-cell population may be involved in the pathogenesis of HIC.