Department of Urology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Department of Urology, University of Iowa, Iowa City, Iowa, USA.
Int J Urol. 2020 Jun;27(6):491-503. doi: 10.1111/iju.14229. Epub 2020 Apr 4.
Interstitial cystitis/bladder pain syndrome is a debilitating condition of unknown etiology characterized by persistent pelvic pain with lower urinary tract symptoms and comprises a wide variety of potentially clinically useful phenotypes with different possible etiologies. Current clinicopathological and genomic evidence suggests that interstitial cystitis/bladder pain syndrome should be categorized by the presence or absence of Hunner lesions, rather than by clinical phenotyping based on symptomatology. The Hunner lesion subtype is a distinct inflammatory disease with proven bladder etiology characterized by epithelial denudation and enhanced immune responses frequently accompanied by clonal expansion of infiltrating B cells, with potential engagement of infection. Meanwhile, the non-Hunner lesion subtype is a non-inflammatory disorder with little evidence of bladder etiology. It is potentially associated with urothelial malfunction and neurophysiological dysfunction, and frequently presents with somatic and/or psychological symptoms, that commonly result in central nervous sensitization. Animal models of autoimmune cystitis and neurogenic sensitization might serve as disease models for the Hunner lesion and non-Hunner lesion subtypes, respectively. Here, we revisit the taxonomy of interstitial cystitis/bladder pain syndrome according to current research, and discuss its potential pathophysiology and representative animal models. Categorization of interstitial cystitis/bladder pain syndrome based on cystoscopy is mandatory to design optimized treatment and research strategies for each subtype. A tailored approach that specifically targets the characteristic inflammation and epithelial denudation for the Hunner lesion subtype, or the urothelial malfunction, sensitized/altered nervous system and psychosocial problems for the non-Hunner lesion subtype, is essential for better clinical management and research progress in this complex condition.
间质性膀胱炎/膀胱疼痛综合征是一种病因不明的使人虚弱的疾病,其特征为持续性骨盆疼痛伴下尿路症状,并包含广泛的具有不同潜在病因的潜在临床有用表型。目前的临床病理和基因组证据表明,间质性膀胱炎/膀胱疼痛综合征应根据是否存在 Hunner 病变进行分类,而不是根据基于症状的临床表型进行分类。Hunner 病变亚型是一种明确的炎症性疾病,具有明确的膀胱病因,其特征为上皮脱落和增强的免疫反应,常伴有浸润 B 细胞的克隆扩增,可能涉及感染。同时,非 Hunner 病变亚型是非炎症性疾病,几乎没有膀胱病因的证据。它可能与尿路上皮功能障碍和神经生理功能障碍有关,常伴有躯体和/或心理症状,通常导致中枢敏化。自身免疫性膀胱炎和神经源性致敏的动物模型可能分别作为 Hunner 病变和非 Hunner 病变亚型的疾病模型。在这里,我们根据当前的研究重新审视间质性膀胱炎/膀胱疼痛综合征的分类,并讨论其潜在的病理生理学和代表性的动物模型。根据膀胱镜检查对间质性膀胱炎/膀胱疼痛综合征进行分类是为每个亚型设计优化治疗和研究策略的必要条件。针对 Hunner 病变亚型的特征性炎症和上皮脱落,或针对非 Hunner 病变亚型的尿路上皮功能障碍、致敏/改变的神经系统和社会心理问题的针对性方法对于这种复杂疾病的更好的临床管理和研究进展至关重要。