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Loin Pain/Hematuria Syndrome

Kantima Phisitkul, MD Reviewed 06/2017
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Subject: Loin Pain/Hematuria Syndrome

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BASICS

DESCRIPTION

  • A rare and poorly understood syndrome characterized by unexplained chronic, severe, intermittent or persistent, unilateral or bilateral loin/flank pain with microscopic or gross hematuria

  • It is a diagnosis of exclusion.

  • Treatment requires reassurance, careful management of analgesia, and psychological support.

EPIDEMIOLOGY

INCIDENCE

Unknown 

PREVALENCE

  • 0.01%

  • Range: 10 to 70 years

  • 70% of cases are in females.

  • More common in White female in 20s to 30s

ETIOLOGY

  • Uncertain

  • Several hypotheses have been proposed such as microvascular abnormalities, complement activation on arterioles, renal vasospasm, veno-caliceal fistula, abnormal ureteral peristalsis, intratubular crystal formation, abnormal platelet function, or psychogenic nature.

  • Glomerular basement membrane abnormalities (too thick or thin) leading to glomerular wall rupture and passage of red blood cell (RBC) into the urinary space, causing tubular obstruction and back leak of glomerular filtrate with resultant parenchymal edema and stretching of renal capsule have been proposed.

GENETICS

No report of genetic involvement. 

RISK FACTORS

Unknown 

GENERAL PREVENTION

N/A 

ASSOCIATED CONDITIONS

  • Somatoform disorder

  • 50% of patients have a history of nephrolithiasis.

DIAGNOSIS

HISTORY

  • Chronic flank pain (>6 months) that is severe, unilateral or bilateral, persistent or recurrent. The duration is variable and may persist for months. Pain may or may not radiate to groin area and may be associated with a low-grade fever, nausea and vomiting, or dysuria.

  • Hematuria can be intermittent, microscopic, or gross.

PHYSICAL EXAM

  • Unremarkable exam

  • Low-grade fever, CVA tenderness variably present

DIFFERENTIAL DIAGNOSIS

  • A diagnosis should be made after a thorough evaluation and exclusion of alternative etiologies:

    • Obstructive or nonobstructive nephrolithiasis

    • Urinary tract infection

    • Adult polycystic kidney disease

    • Renal cell cancer

    • Recurrent papillary necrosis

    • Recurrent renal thromboembolism

    • Recurrent renal vein thrombosis

    • Nutcracker syndrome (left renal vein entrapment)

    • Factitious hematuria

TESTS

INITIAL TESTS

  • Normal renal function tests

  • Urinalysis (UA) shows >5 RBC/HPF but may be absent during pain-free period.

  • The presence of dysmorphic RBC or RBC casts is possible but not necessary.

  • Spot urine protein to creatinine ratio is normal (<0.3).

  • CT of abdomen with/without contrast is normal.

  • Urine culture is negative.

  • Coagulation studies are normal.

TESTS CONSIDERATIONS
Psychiatric/psychological evaluation for depression, anxiety, drug addiction, psychosocial stressors, and underlying psychopathology. It is unclear if psychiatric conditions are the cause or the result of pain. 

DIAGNOSTIC PROCEDURES/SURGERY

  • Consider nephrology consultation for renal biopsy in the presence of dysmorphic RBC/RBC cast and proteinuria or abnormal kidney function to exclude underlying glomerular disease.

  • Renal angiography to exclude arteriovenous (AV) malformation

  • Cystoscopy/ureteroscopy to evaluate stone, tumor, other pathology of bladder and ureter

INTERPRETATION

  • No consensus on validated diagnostic criteria.

  • All standard hematologic and chemistry evaluation should be normal.

TREATMENT

GENERAL MEASURES

  • Controversial. Most recommendations are based on observational studies, small case series, and anecdotal reports.

  • A multidisciplinary approach partnering medical management with cognitive behavioral therapy should be exhausted prior to invasive or surgical interventions (1,2)[C].

MEDICATION

FIRST LINE

  • Nonopioid analgesic and adjuvant analgesics

  • Antidepressant

  • An angiotensin-converting enzyme inhibitor (ACEI). It was believed to reduce the frequency and severity of the episodes of gross hematuria and flank pain by reducing glomerular hydrostatic pressure (1,2,3)[C].

SECOND LINE

Opioid-type analgesics 

ISSUES FOR REFERRAL

  • Pain management specialist

  • Psychiatrist/psychologist

  • Urologist

  • Nephrology if renal biopsy considered

ADDITIONAL THERAPIES

Hypnotherapy (4)[C

SURGERY

  • Intrathecal morphine pump (1,2)[C]

  • Transcutaneous electrical nerve stimulation (1,2)[C]

  • Percutaneous regional nerve blocks of splanchnic, intercostal, or celiac nerve (1,2)[C]

  • Surgical sympathectomy with renal capsulotomy (1,2)[C]

  • Catheter-based bilateral renal denervation (3,5)[C]

  • Laparoscopic nephrectomy with renal autotransplantation of the involved kidney is used to preserve renal function and provide sustained relief of intractable pain. Pain might recur within 2 years or occur in transplanted or contralateral kidney due to renal reinnervation or psychosocial problem (1,2,3)[C].

COMPLEMENTARY & ALTERNATIVE MEDICINE

Nephrectomy should be considered as a last resort. 

INPATIENT CONSIDERATIONS

  • Admission criteria/initial stabilization: pain exacerbation with intractable pain/nausea or vomiting

  • Intravenous (IV) fluids in case of poor oral intake

  • IV or patient-controlled analgesia may be used to control pain.

  • Discharge when pain is under control.

ONGOING CARE

DIET

No restrictions 

PATIENT TEACHING

  • It is a chronic pain syndrome with medical and psychological aspects.

  • Nephrectomy should be considered as a last resort.

PROGNOSIS

  • 1/3 of patients have a spontaneous resolution after a mean of 3.5 years conservative treatment.

  • Invasive procedures are a last resort.

  • Kidney function is preserved.

  • Hematuria typically persists.

COMPLICATIONS

  • High rate of opiate addiction and total disability

  • Postprocedure/surgery complications

  • Dialysis if bilateral nephrectomy is performed.

REFERENCES

1
Taba Taba Vakili S, Alam T, Sollinger H. Loin pain hematuria syndrome. Am J Kidney Dis.  2014;64(3):460–472.  [View Abstract]
2
Smith HS, Bajwa ZH. Loin pain hematuria syndrome—visceral or neuropathic pain syndrome? Clin J Pain.  2012;28(7):646–651.  [View Abstract]
3
Zubair AS, Salameh H, Erickson S et al. Loin pain hematuria syndrome. Clin Kid J.  2016;9(1):128–134.  [View Abstract]
4
Elkins GR, Koep LL, Kendrick CE. Hypnotherapy intervention for loin pain hematuria: a case study. Int J Clin Exp Hypn.  2012;60(1):111–120.  [View Abstract]
5
Prasad B, Giebel S, Garcia F et al. Renal denervation in patients with loin pain hematuria syndrome. Am J Kidney Dis.  2017;69(1):156–159.  [View Abstract]

ADDITIONAL READING

Dube GK, Hamilton SE, Ratner LE et al. Loin pain hematuria syndrome. Kidney Int.  2006;70(12):2152–2155.  [View Abstract] 

CODES

ICD10

  • R10.9 Unspecified abdominal pain

  • R31.9 Hematuria, unspecified

  • R31.2 Other microscopic hematuria

  • R31.0 Gross hematuria

ICD9

  • 789.09 Abdominal pain, other specified site

  • 599.70 Hematuria, unspecified

  • 599.72 Microscopic hematuria

  • 599.71 Gross hematuria

SNOMED

  • 86208007 Loin pain-hematuria syndrome (disorder)

PEARLS

  • Loin pain/hematuria syndrome is a controversial disease with uncertain cause and pathophysiology.

  • All standard labs and renal imaging are normal.

  • The diagnosis is made after a thorough evaluation and exclusion of alternative etiologies.

  • A multidisciplinary approach combining medical management and cognitive behavioral therapy should be exhausted prior to invasive or surgical interventions.

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