Shenhar Chen, Kass Alon, Yakimov Maxim, Tomashev Dinkovich Roni, Golan Shay, Baniel Jack, Gilon Gabriel, Aharony Shachar
Urology Division, Rabin Medical Center, Israel.
Faculty of Medicine, Hebrew University, Jerusalem, Israel.
Harefuah. 2021 Sep;160(9):586-593.
Interstitial cystitis/bladder pain syndrome (IC/BPS) is a chronic, debilitating syndrome involving bladder pain and lower urinary tract symptoms (LUTS), with a substantial effect on patients' quality of life. IC/BPS poses a diagnostic challenge, and its available treatment options remain only moderately effective. Bladder-wall biopsies from IC/BPS patients commonly uncover mastocytosis. While mast-cells are suspected as pivotal in disease pathogenesis, the clinical significance of their presence remains unclear. Clinical guidelines differ on whether or not bladder biopsies should be a part of routine IC/BPS workup.
To determine whether detrusor mastocytosis can serve as a prognostic marker for treatment response and improvement duration.
We retrospectively collected patient data for IC/BPS patients who underwent bladder hydrodistension under anesthesia. We used statistical modelling to determine the effect of mastocystosis and other possible predictive factors - age, comorbidity, Hunner lesions - on the presence and duration of symptom improvement.
A total of 35 patients (89% female, median age 63.5 [IQR 48.8-73.6] years) underwent hydrodistension, of whom 28 (89% female, median age 63.0 [44.8-73.1] years) had bladder biopsies; 11 (39%) of them had mastocystosis. Within a median follow-up of 8.8 [1.7-24.2] months, 11 (100%) of the patients with mastocytosis and 9 (53%) of the patients without it, experienced symptomatic improvement (p=0.007). Improvement duration was 8 months longer (p=0.006) in those with mastocystosis. Univariate logistic regression models were used to estimate odds ratios (OR). Mastocytosis (p=0.004) and Charlson Comorbidity score were the only variables with a statistically significant OR. Univariate survival models were fitted, and improvement duration was estimated to be longer in patients with mastocystosis (p=0.01). A multivariate Cox regression model found no variable to be statistically significant, though mastocystosis was borderline significant (p=0.055).
Mastocystosis defines a discernible phenotype of IC/BPS, which exhibits higher rates and longer duration of hydrodistention treatment response.
Notwithstanding limitations of sample size and retrospective study design, we were able to demonstrate that mastocystosis can serve as a prognostic factor for symptom improvement after hydrodistension in IC/BPS patients. Prospective studies are required to validate this finding and to investigate the mechanisms involved.
间质性膀胱炎/膀胱疼痛综合征（IC/BPS）是一种慢性、使人衰弱的综合征，涉及膀胱疼痛和下尿路症状（LUTS），对患者的生活质量有重大影响。IC/BPS带来了诊断挑战，其现有的治疗选择效果仍仅为中等。IC/BPS患者的膀胱壁活检通常发现肥大细胞增多症。虽然肥大细胞被怀疑在疾病发病机制中起关键作用，但其存在的临床意义仍不明确。关于膀胱活检是否应作为IC/BPS常规检查的一部分，临床指南存在分歧。
确定逼尿肌肥大细胞增多症是否可作为治疗反应和改善持续时间的预后标志物。
我们回顾性收集了在麻醉下接受膀胱水扩张术的IC/BPS患者的资料。我们使用统计模型来确定肥大细胞增多症和其他可能的预测因素——年龄、合并症、Hunner病变——对症状改善的存在和持续时间的影响。
共有35例患者（89%为女性，中位年龄63.5[四分位间距48.8 - 73.6]岁）接受了水扩张术，其中28例（89%为女性，中位年龄63.0[44.8 - 73.1]岁）进行了膀胱活检；其中11例（39%）有肥大细胞增多症。在中位随访8.8[1.7 - 24.2]个月期间，11例（100%）有肥大细胞增多症的患者和9例（53%）无肥大细胞增多症的患者症状得到改善（p = 0.007）。有肥大细胞增多症的患者改善持续时间长8个月（p = 0.006）。使用单因素逻辑回归模型估计比值比（OR）。肥大细胞增多症（p = 0.004）和Charlson合并症评分是仅有的具有统计学显著OR的变量。拟合单因素生存模型，估计有肥大细胞增多症的患者改善持续时间更长（p = 0.01）。多因素Cox回归模型未发现有统计学显著意义的变量，不过肥大细胞增多症接近显著（p = 0.055）。
肥大细胞增多症定义了一种可识别的IC/BPS表型，其水扩张治疗反应率更高且持续时间更长。
尽管存在样本量和回顾性研究设计的局限性，但我们能够证明肥大细胞增多症可作为IC/BPS患者水扩张术后症状改善的预后因素。需要进行前瞻性研究来验证这一发现并研究其中涉及的机制。
