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Diagnosis: Loin pain-hematuria syndrome

The loin pain-hematuria syndrome (LPHS) is an ill-defined clinical syndrome characterized by recurrent episodes of loin pain accompanied by hematuria. It was first described in 1967 in a report of three young women who had recurrent episodes of severe unilateral or bilateral loin (flank) pain that were accompanied by gross or microscopic hematuria (Little, PJ, et al. A syndrome of loin pain and haematuria associated with disease of peripheral renal arteries. Q J Med 1967; 36:253-9 [PubMed link]). The major causes of flank pain and hematuria were not present. Renal arteriography suggested focally impaired cortical perfusion, while renal biopsy showed interstitial fibrosis and arterial sclerosis. Since this original report, several hundred cases have been reported in the medical literature. In LPHS appears to be glomerular bleeding. Most patients present with both manifestations, but some present with loin pain or hematuria alone. Pain episodes are rarely associated with low-grade fever and dysuria, but urinary tract infection is not present (Hebert LA, et al. Loin pain-hematuria syndrome. In: UpToDate [www.uptodate.com], consulted December 27, 2010).

Renal colic associated with hematuria can be seen in patients with ureteric stones, urinary tract infections, and known causes of glomerular hematuria, such as acute glomerulonephritis or IgA nephropathy. Loin pain with hematuria is also occasionally seen in patients with thin basement membrane disease. This syndrome of loin pain-hematuria should therefore be invoked only when the other causes of hematuria have been excluded. The clinical definition of a patient with this syndrome is a person with intermittent or persistent loin pain and intermittent or persistent hematuria (either gross but more usually microscopic) in the context of a normal collecting system, as confirmed by radiography. If it is performed, a renal biopsy should not show any form of primary or secondary glomerular lesion (Heptinstall's Pathology of the Kidney, 6th edition, p.1182).

The etiology of LPHS is unknown, but some authors have hypothesized to be due to glomerular capillary hemorrhage resulting in tubular obstruction and interstitial edema; over time, chronic edema is thought to result in capsular distention, pain, and further tubular obstruction and hemorrhage (Spetie DN, et al. Proposed pathogenesis of idiopathic loin pain-hematuria syndrome. Am J Kidney Dis. 2006;47:419-27. [PubMed link]). Canales BK, et al. found ring-like clots consistent with recent hemorrhage in each fornix surrounding all calyces of the collecting system, strongly suggesting the presence of a physiological disorder in this poorly understood disease (Canales BK, et al. Endoscopic findings in loin pain hematuria syndrome: concentric clot in calyceal fornices. Diagn Ther Endosc. 2008;2008:721850. [PubMed link] [Free full text])

The majority of patients are women (70 to 80 percent in most series) between the ages of 20 and 40, but the syndrome has also been reported in older children. As many as one-half of patients with LPHS have nephrolithiasis, as defined by either a history of passing stones or renal calcifications typical of stones on imaging studies. Clinical evaluation shows nothing unusual, except for the presence of pain and hematuria. Blood pressure and renal function are normal, and evidence of proteinuria is generally absent. Radiologic findings have been variable, but several investigators have described tortuosity, beading, and obliteration of medium-size intrarenal vessels. Commonly arteriography is completely normal.

When biopsy is performed, renal histology is normal or shows only minor abnormalities: mild mesangial hypertrophy, patchy interstitial fibrosis with tubular atrophy and/or arterial and arteriolar hyalinosis. On immunofluorescence some studies have reported deposition of C3 in affected vessels (a nonspecific finding). The radiologic and histologic findings suggest that the syndrome may result from diseases affecting the intrarenal vessels, but this theory has not been confirmed (Heptinstall's Pathology of the Kidney, 6th edition, p.1182).

Despite the dramatic nature of the clinical features, the prognosis from a renal point of view is excellent. There have been no reports of progressive renal injury or evolution to chronic disease.

Many conditions causing loin pain had being described:
•Urinary tract infection
•Kidney abscess
•Kidney stones
•Kidney cancer
•Renal vein thrombosis
•Polycystic kidney disease
•Prostate cancer
•Benign prostatic hypertrophy
•Acute glomerulonephritis
•Acute pyelonephritis
•Perinephric abscess
•Hydronephrosis
•Renal cell carcinoma
•Horseshoe kidney
•Kidney infection
•Kidney stone
•Ulcerative colitis
•Enlarged spleen
•Spleen inflammation
•Shingles
•Lumbar hernia
•Retroperitoneal bleed
•Renal infarction
•Nerve root irritation
•Muscular
(www.wrongdiagnosis.com/sym/loin_pain.htm#intro)

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Bibliography

• Coffman KL. Loin pain hematuria syndrome: a psychiatric and surgical conundrum. Curr Opin Organ Transplant. 2009;14(2):186-90. [PubMed link]
• Canales BK, Windsperger A, Lukasewycz S, Monga M. Endoscopic findings in loin pain hematuria syndrome: concentric clot in calyceal fornices. Diagn Ther Endosc. 2008;2008:721850. [PubMed link] [Free full text]
• Dube GK, Hamilton SE, Ratner LE, Nasr SH, Radhakrishnan J. Loin pain hematuria syndrome. Kidney Int. 2006;70(12):2152-5. [PubMed link]
• Spetie DN, Nadasdy T, Nadasdy G, Agarwal G, Mauer M, Agarwal AK, Khabiri H, Nagaraja HN, Nahman NS Jr, Hartman JA, Hebert LA. Proposed pathogenesis of idiopathic loin pain-hematuria syndrome. Am J Kidney Dis. 2006;47(3):419-27. [PubMed link]
• Little, PJ, Sloper, JS, de Wardener, HE. A syndrome of loin pain and haematuria associated with disease of peripheral renal arteries. Q J Med 1967; 36:253-9 [PubMed link]

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