Abstract
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.
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Key Summary Points
1. The most prominent features of LPHS include recurrent flank pain and hematuria that is usually seen in young women
2. The diagnosis of LPHS is considered a diagnosis of exclusion
3. Proposed pathophysiologies range from vasospasm, coagulopathy, hypersensitivity, complement activation on arterioles, glomerular basement membrane (GBM) abnormalities, abnormal ureteral peristalsis, psychopathology, and intratubular deposition of calcium or uric acid
4. Treatment of LPHS would include multiple conservative therapy measures, such as non-opioid analgesics, opioid analgesics, cognitive behavioral therapy, physical medicine, and ACE inhibitors
5. More aggressive and invasive measures include intra-ureteric infusions, denervation, radiofrequency ablation, neuromodulation, and transplantation with variable success rates
This article is part of the Topical Collection on Chronic Pain Medicine
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Urits, I., Li, N., Berger, A.A. et al. Treatment and Management of Loin Pain Hematuria Syndrome. Curr Pain Headache Rep 25, 6 (2021). https://doi.org/10.1007/s11916-020-00925-0
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DOI: https://doi.org/10.1007/s11916-020-00925-0