A 24 year old caucasian male with no significant past medical history was admitted with right flank pain and hematuria of 5 days duration. The pain was stabbing in chararcter, non-radiating with no significant aggrevating or allevaiting factors. The patient reported episodes of intense pain that lasts for three to four hours, with a notable basal level of pain in between exacerbations. Review of system negative for fever , chills , nausea , vomiting , dysuria and urinary frequency. He has no history of renal calculi and no family history of kidney diseases. Urinalysis revelaed large blood and was negative for leukocytes, bacteria and cytology. Urine culture was also negative. Blood urea Nitrogen was 13 and Serum creatinine was 1.44. His 24 hour urine protein was less than 300 mg /day and urine creatinine was normal. Nephritis workup revealed a normal serum complement and ANCA screen, negative antinuclear antibody, anti ds-DNA and antiglomerular basement antibody test. Hepatitis B and C antibody were not detected and HIV test was non-reactive. Computerized Tomography ( CT ) scan of abdomen with and without contrast did not demonstrate any renal stones, masses, or hydronephrosis. CT angiogram of the kidney done to evaluate for ArterioVenous malformation was also negative. Bilateral diagnostic ureteroscopy showed hematuria through right ureter. Patient underwent renal biopsy which showed normal glomeruli in light and electron microscopy with Red Blood Cells in Bowman’s space and tubular lumens whch is diagnostic of Loin Pain Hematuria Syndrome(LPHS).The patient received pain management consultation and was maintained on opiods for pain control and started on neurontin. Patient was eventually discharged and did well on follow up visit.
Discussion:
LPHS is a rare disease with a prevalence of ∼0.012%. The most prominent clinical features include periods of severe intermittent or persistent unilateral or bilateral loin pain accompanied by either microscopic or gross hematuria. Further, loin pain hematuria syndrome is not associated with loss of kidney function or urinary tract infections. But patient suffers from incapacitating colic and most of the initial investigations will be normal. The diagnosis of LPHS remains one of exclusions.The minimal criteria to reach a diagnosis of LPHS include recurrent or persistent flank pain sometimes requiring Norco, absence of obstruction confirmed by at least two kidney imaging procedures, glomerular hematuria and renal biopsy showing normal glomeruli on light and immunofluorescence microscopy. Analgesic medications, including narcotics, commonly are used to treat loin pain hematuria syndrome associated pain. Interventional treatments include renal denervation, kidney autotransplantation, and nephrectomy; however, these methods should be used only as a last resort when less invasive measures have been tried unsuccessfully.
Conclusions:
LPHS is a complex condition that is not well understood; diagnosis continues to be one of exclusion. Going forward, it would be very beneficial to have more basic science and controlled clinical studies conducted on LPHS to help determine the definitive pathophysiology of the syndrome and how to diagnose and treat the condition.