54 year old female presented with 5 month history of B/L flank pain constant with intermttent exacerbations and one UA in past positive for blood, dipstick in office also positive for blood. No evidence of UTI. NSAIDs use on and off. One differential is LPHS.
Attached is a good review article .
Imp points:
1.Majority of the cases are females but recent reports have higher male population.
2.Pain episodes may be associated with low-grade fevers and dysuria, although there is no evidence of concurrent urinary tract infection.
3. Lowgrade proteinuria has been reported in some patients, primarily in the setting of an attack of flank pain.
4. Biopsy may show mild tubular atrophy, patchy interstitial fibrosis, glomerulosclerosis, arteriolar hyalinosis, and subcapsular cortical ischemia. Electron microscopy in some patients reveals abnormally thin or thick glomerular basement membranes.
5.In LPHS associated with Thin Basement membrane ACEIs can decrease severity of pain as suggested in this study.
6.Most importantly, renal biopsy should be considered on all patients in whom the diagnosis of LPHS is considered as 21% patient were found to have immunoglobulin A nephropathy, especially if proteinuria (even tubular proteinuria) or renal dysfunction is present
Attached is a good review article .
Imp points:
1.Majority of the cases are females but recent reports have higher male population.
2.Pain episodes may be associated with low-grade fevers and dysuria, although there is no evidence of concurrent urinary tract infection.
3. Lowgrade proteinuria has been reported in some patients, primarily in the setting of an attack of flank pain.
4. Biopsy may show mild tubular atrophy, patchy interstitial fibrosis, glomerulosclerosis, arteriolar hyalinosis, and subcapsular cortical ischemia. Electron microscopy in some patients reveals abnormally thin or thick glomerular basement membranes.
5.In LPHS associated with Thin Basement membrane ACEIs can decrease severity of pain as suggested in this study.
6.Most importantly, renal biopsy should be considered on all patients in whom the diagnosis of LPHS is considered as 21% patient were found to have immunoglobulin A nephropathy, especially if proteinuria (even tubular proteinuria) or renal dysfunction is present
I wasn't aware of the association with IgA nephropathy. Interesting that the pathogenesis of the pain is thought to be glomerular hematuria itself, since other GNs causing glomerular hematuria are not classically associated with pain. I would argue that in the absence of proteinuria (and with normal renal function) a renal biopsy is likely not justified, since IgA, even if present, would to be treated in this setting. Unless treating the underlying IgA (ACEi? Steroids?) would improve pain symptoms?
ReplyDeleteAgree. May be give a trial of ACEIs along with analgesics (probably opioids) given that it helps in LPHS with TBM as rest of the treatment options are highly invasive with serious complications.
ReplyDeleteI saw a patient during my fellowship where she underwent autotransplantation of kidney because of intractable pain associated with LPHS. I remember my program director mentioning that the success rate of autotransplantion was 50%. The etiology of LPHS is unknown. Surprisingly this is the only situation where ACEIs were claimed to be helpful for pain control (purely observational).
ReplyDeletePradeep brought up the issue of tubular proteinuria. I am wondering how we are assessing tubular proteinuria in office patients? One of the test was beta 2 microglobulin but I never performed in my office patients.
Other DD for LPHS in young women with left sided flank pain is nutcracker syndrome.
Link to Kiran's Nutcracker Syndrome Article from Mayo Clinic Proc 2010.
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