Loin pain hematuria syndrome

  Loin pain hematuria syndrome, also LPHS, is the combination of unilateral or bilateral flank pain and microscopic or macroscopic amounts of blood in the urine that is otherwise unexplained.^[1]

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Diagnosis

LPHS is considered a diagnosis of exclusion as hematuria (blood in the urine) and flank pain can result from a number of causes.

Relation to chronic pelvic pain

LPHS has considerable overlap with chronic pelvic pain and often, like chronic pelvic pain, has a significant psychiatric component, with a high co-morbidity of somatoform disorder.^[2]

Relation to thin basement membrane disease

A thin glomerular basement membrane, as in thin basement membrane disease, is proposed to be the characteristic finding on renal biopsy,^[3] but not part of the syndrome definition.

Prevalence

The condition is very rare and predominately affects females. Worldwide, only several hundred cases have been reported.

Etiology

The cause of LPHS is currently not known.^[1] One theory proposes that it is caused by a thin glomerular basement membrane and red blood cell (RBC) renal tubular congestion that leads to swelling of the kidney and distension of the renal fascia resulting in pain.^[3]

Treatment

The treatment of LPHS varies considerably from centre to centre. As the condition is rare and poorly understood a widely adopted standard of care is not existent.

Conservative

Pain management with opiate and non-opiate analgesia is common. Angiotensin converting enzyme inhibitors are thought to be beneficial,^[3] as they reduce intraglomerular pressure and, presumably, reduce renal tubular congestion with RBCs.

Surgical

Surgery (autotransplantation) is thought by some to be of benefit in selected individuals^[4] and advocated in some centres, but usually considered the last resort.^[5]

Many physicians discourage surgery, as LPHS symptoms often re-occur after autotransplantation.^[5]

Differential diagnosis

• Kidney stones
• Nutcracker syndrome
• IgA nephropathy
• Cancer of the genitourinary tract - classically not associated with pain
• Chronic pelvic pain
• Endometriosis

See also

• Pelvic pain

References

1. ^ ^{a b} Pukenas BA, Zaslau S (2003). "Loin pain hematuria syndrome: case series". W V Med J 99 (5): 192–3. PMID 14959511.
2. ^ Bass CM, Parrott H, Jack T, Baranowski A, Neild GH (2007). "Severe unexplained loin pain (loin pain haematuria syndrome): management and long-term outcome". QJM 100 (6): 369–81. doi:10.1093/qjmed/hcm034. PMID 17525133.
3. ^ ^{a b c} Hebert LA, Betts JA, Sedmak DD, Cosio FG, Bay WH, Carlton S (1996). "Loin pain-hematuria syndrome associated with thin glomerular basement membrane disease and hemorrhage into renal tubules". Kidney Int. 49 (1): 168–73. PMID 8770964.
4. ^ Chin JL, Kloth D, Pautler SE, Mulligan M (1998). "Renal autotransplantation for the loin pain-hematuria syndrome: long-term followup of 26 cases". J. Urol. 160 (4): 1232–5; discussion 1235–6. PMID 9751325.
5. ^ ^{a b} Parnham AP, Low A, Finch P, Perlman D, Thomas MA (1996). "Recurrent graft pain following renal autotransplantation for loin pain haematuria syndrome". Br J Urol 78 (1): 25–8. PMID 8795395.