Purpose of Review

Loin pain hematuria syndrome (LPHS) is rare and seldom diagnosed, yet it has a particularly significant impact on those affected. This is a review of the latest and seminal evidence of the pathophysiology and diagnosis of LPHS and presents the typical clinical presentation and treatment options available.

Recent Findings

LPHS is typically found in young women with characteristic symptoms, including severe recurrent flank pain and gross or microscopic hematuria. The majority of patients will experience crippling pain for many years without effective therapy, often requiring frequent use of narcotic medication. However, the lack of conclusive pathophysiology, in conjunction with the rarity of LPHS, has prohibited the development and trial of definitive treatment options. Nevertheless, in order to combat this rare but severe disease, management strategies have continued to evolve, ranging from conservative measures to invasive procedures. This review presents an overview of the current hypotheses on the pathophysiology of LPHS in addition to summarizing the management strategies that have been utilized.

Summary

Only 30% of LPHS patients will experience spontaneous resolution, whereas the majority will continue to face chronic, crippling pain. Several methods of treatment, including invasive and non-invasive, may provide an improved outcome to these patients. Treatment should be individually tailored and multi-disciplinary in nature. Further research is required to further elucidate the pathophysiology and develop new, specific, treatment options.

中文翻译：

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腰痛血尿综合征的治疗与管理

审查目的

腰痛血尿综合征 (LPHS) 很少见，也很少被诊断出来，但它对受影响的人有特别重要的影响。这是对 LPHS 病理生理学和诊断的最新和开创性证据的回顾，并介绍了典型的临床表现和可用的治疗方案。

最近的发现

LPHS 通常见于具有特征性症状的年轻女性，包括严重的复发性腰痛和肉眼或镜下血尿。大多数患者在没有有效治疗的情况下会经历多年的严重疼痛，通常需要频繁使用麻醉药物。然而，由于缺乏决定性的病理生理学，加上 LPHS 的罕见性，已经禁止了确定性治疗方案的开发和试验。尽管如此，为了对抗这种罕见但严重的疾病，管理策略不断发展，从保守措施到侵入性程序。除了总结已使用的管理策略外，本综述还概述了当前关于 LPHS 病理生理学的假设。

概括

只有 30% 的 LPHS 患者会经历自发消退，而大多数将继续面临慢性、严重的疼痛。包括侵入性和非侵入性在内的几种治疗方法可以为这些患者提供改善的结果。治疗应该是个性化的和多学科的。需要进一步研究以进一步阐明病理生理学并开发新的、特定的治疗方案。