Tag Archives: LPHS

Loin Pain Hematuria Syndrome (LPHS)

Loin Pain Hematuria Syndrome (LPHS) is an extremely rare disease, where there is unexplained flank pain and Haematuria. Often misdiagnosed as a hypermobile kidney has the same symptoms. This is poorly understood condition for which specific diagnostic criteria are lacking.

Here are some facts about LPHS:

  • LPHS was first identified in England in 1967 among women who were taking birth control pills.
  • Approximately 90% of the reported cases of LPHS are female.
  • LPHS was first noted in men in the 1980’s.
  • LPHS was first noted in the USA in the 1980’s.
  • The first renal autotransplatation for LPHS was done in 1982.
  • Cases of LPHS have been reported from England, Canada, USA, India, Australia, Ireland, Scotland and Germany.
  • The age range for LPHS is from 6 to 50 years.
  • There are approximately 200 cases of LPHS in the world; no doubt, there are more undocumentated cases.
  • Pain attacks due to LPHS can occur several times per week, every few weeks, or every few months. These pain attacks can last from hours to weeks.

    Here are some guidelines to help you decide if you think you need to investigate this problem with your doctor.

  • The condition is mainly characterized by intractable pain involving the kidney and abdominal areas.
  • Microscopic blood or blatantly obvious amounts of it can be seen in the urine. Clot retention can also occur.
  • It is extremely important that physicians recognize the need for adequate pain medication for this disorder.
  • Forcing fluids by mouth or I.V. are very helpful in decreasing the hematuria and in reducing the risk of clot retention.
  • Be prepared to see several doctors and yield to psychiatric exams before you get a correct diagnosis. This is common with any disease that is not common, such as diabetes. LPHS has only been diagnosed in a few hundred people around the entire world, so this is one disease that is not likely to pop into a physician’s head.

    related articles

    Department of Surgery, Royal Prince Alfred Hospital, Sydney, NSW, Australia. agrs@liver.rpa.cs.nsw.gov.au

    Some patients with the loin pain/hematuria syndrome suffer incapacitating flank pain. No effective therapy has been reported. Uncertainty persists concerning the authenticity of the pain and the role of surgery in treatment.

    Forty-six patients with loin pain/hematuria syndrome and intractable pain were evaluated following treatment either by renal autotransplantation (30 patients, 10 bilaterally) or by renal denervation (20 patients, four bilaterally) over a 13-year period.

    All patients had concomitant renal nerve excision and ligation and capsulotomy. There were 37 (80%) women and nine men aged 18 to 61 years (mean age, 33 years).

    Excretion urography and angiography were normal in all patients. Nineteen of 25 (76%) patients in whom renal autotransplantation was successfully accomplished and who completed a follow-up questionnaire were free of pain, including eight of 10 with bilateral procedures.

    The follow-up periods ranged from 1 to 13 years (mean, 8.4 years). Six patients have been free of pain for 10 to 13 years.

    Of 18 patients treated with renal neurectomy who were available for follow-up examination, 12 (67%) developed recurrent renal pain, including four who had pain relief on the other side following previous renal autotransplantation.

    The follow-up period for these patients ranged from 6 to 9.9 years (mean, 8.0 years). Three of four patients with recurrent renal pain following neurectomy were treated successfully by renal autotransplantation.

    The loin pain/hematuria syndrome is a rare cause of incapacitation, predominantly of relatively young females. The pain of the syndrome is organic.

    Renal autotransplantation achieves pain relief in three quarters of patients, but the procedure is often (30%) required bilaterally and has significant complications.

    Renal neurectomy is followed by an excessive incidence of recurrent renal pain.


    What is Loin Pain Hematuria Syndrome?

    by Marc F. Brazie, MD
    Division of Nephrology
    University of Maryland Medical Center, Baltimore, MD

    What is Loin Pain Hematuria Syndrome (LPHS)?

    LPHS is a rare kidney disorder causing attacks of severe flank pain and blood in the urine.

    Who gets LPHS?

    LPHS was first reported in three patients in 1967 who were found to have recurrent attacks of severe flank pain and blood in the urine (hematuria) in whom no other explanation could be found [2]. Since that time, it has been reported in several hundred people worldwide, although it is likely that there are many more unreported cases. It is more common in women (70% of all cases) [3-5], and has been seen mostly in the United States, Great Britain, Australia, and Canada, suggesting a white predominance. While it can start at almost any age, most patients will first develop symptoms in their 20’s [6].

    How do I know if I have LPHS?

    The predominant features of LPHS are recurrent flank pain and blood in the urine. The pain can occur on only one side or both, and may radiate to the abdomen, groin, or inner thigh. Pain episodes may be associated with low-grade fevers and a burning discomfort with urination. The blood in the urine may be overt (called “macroscopic hematuria”), or only detectible on a urine dipstick test or by examining the urine through a microscope (called “microscopic hematuria”). Up to 47% of patients with LPHS also have a history of kidney stones [7], but stones must be absent during pain episodes for LPHS to be diagnosed.

    What causes LPHS?

    As LPHS is a rare disorder, not much is known about the underlying causes. It is thought that there may be a problem with the glomerular basement membrane (GBM), which is the blood “filter” in the kidney, causing bleeding into the kidney tubules. These tubules can then become blocked by the blood cells, causing swelling and increased pressure in the kidney. This in turn leads to stretching of the outer layer of the kidney, called the “capsule,” and pain. Indeed, studies examining biopsies of patients thought to have LPHS have shown that in over 50% of patients, the GBM is abnormally thick or thin [7].

    What should I do if I think I might have LPHS?

    There are many disorders that can cause flank pain and blood in the urine, and as there is no specific test to confirm LPHS, it is considered a diagnosis of exclusion. It is very important to have a thorough evaluation by a kidney doctor (nephrologist) to be sure there is no other cause for the symptoms. This evaluation may involve laboratory tests, radiology studies, or even a kidney biopsy.

    What can be done for LPHS?

    Again, as LPHS is a rare disorder, it is not clear what the optimal treatment is. What is known is that patients with LPHS tend to have normal kidney function, which does not deteriorate over time. Thus, treatment is aimed at pain control and improvement in quality of life. Multiple therapies have been tried, all with mixed results. These include:
    • Medications such at angiotensin-converting enzyme inhibitors (a family of blood pressure medicines which reduce the pressure in the kidney and have been shown to be of benefit to some patients [8]), non-steroidal anti-inflammatory drugs (such as ibuprofen, naproxen, or ketorolac), and narcotic pain medications.
    • Nerve block procedures to decrease the pain signal from the effected kidney, and
    • Surgery to strip the nerves from the kidney, “autotransplant” the kidney (in which it is removed from its normal position and reinserted in the lower abdomen – this has been shown to relieve pain in up to 70% of patient in some reports [3,4]), or in extreme cases, remove the kidney altogether.

    It is best to discuss all the treatment options with your doctor, and ideally as part of a “multidisciplinary” approach involving primary care doctors, nephrologists, psychiatrists, and chronic pain specialists.


    References
    1. Dube GK, Hamilton SE, Ratner LE, Nasr SH, Radhakrishnan J. Loin pain hematuria syndrome. Kidney Int. 2006; 70: 2152-2155.
    2. Little PJ, Sloper JS, deWardener HE. A syndrome of loin pain and haematuria associated with disease of peripheral renal arteries. Q J Med. 1967; 36: 253-259.
    3. Sheil AG, Chui AK, Verran DJ et al. Evaluation of the loin pain/hematuria syndrome treated by renal autotransplantation or radical renal neurectomy. Am J Kidney Dis. 1998; 32: 215-220.
    4. Chin JL, Kloth D, Paulter SE, Mulligan M. Renal autotransplantation for the loin pain-hematuria syndrome: long-term followup of 26 cases. J Urol. 1998; 160: 1232-1235.
    5. Greenwell TJ, Peters JL, Neild GH, Shah PJ. The outcome of renal denervation for managing loin pain haematuria syndrome. BJU Int. 2004; 93: 818-821.
    6. Weisberg LS, Bloom PB, Simmons RL, Viner ED. Loin pain hematuria syndrome. Am J Nephrol. 1993; 13: 229-237.
    7. Spetie DN, Nadasdy T, Nadasdy G, et al. Proposed pathogenesis of idiopathic loin pain-hematuria syndrome. Am J Kidney Dis. 2006; 47: 419-427.
    8. Hebert LA, Betts JA, Sedmak DD, et al. Loin pain-hematuria syndrome associated with thin glomerular basement membrane disease and hemorrhage into renal tubules. Kidney Int. 1996; 49: 168-173.

  • I was diagnosed with LPHS only about a month ago.

    I was looking around on the web because I was diagnosed with LPHS only about a month ago. I thought I’d add my story! I’m only 16, and I’m not really sure if that’s young for this condition but I’ve been suffering from it for 5 years. I had my first kidney stone when I was in 7th grade and from then on, the pain just got worse. I developed more kidney stones but the doctors kept saying they weren’t causing the pain and that I was faking it. Of course, my Mum believed me and I was furious. Doctor after doctor had no cure for me and we were starting to lose hope… Then we moved. I use to live in P.A but in June of this year, 2006, we moved to South Carolina. My pain started getting worse and became chronic. It was so bad, I had to stop going to school and now I’m being homeschooled. Again, we kept going through doctors when finally we met a kidney doctor who said everything I’m feeling sounds like LPHS. We were relieved! My Mum even started to cry! He did a few tests and it turns out that that’s what I have.

    I’m put on Blood pressure medicine instead of pain killers because at my age, I couldn’t take a large dose and the largest I was taking wasn’t even working anymore! Amazingly, the blood pressure medicine is working fine. It dulls the pain enough so that I can walk around and not be stuck in bed all day. In January, I’ll be taking another kind of medicine to hopefully mask the pain all together. I’m really excited! 5 years of being in pain and everyone thinking your faking really takes a toll on you!

    Anyway, just as an added thing, my doctor said that what is happening with me is that the capsule around the kidney is stretching and that the blood pressure medicine will relax that, or something. I really can’t remember everything he said but maybe, those of you who are taking Pain killers and are finding no relief, should try blood pressure medicine. Everyone is different, so I don’t know if it will work, but my doctor puts his LPHS patients on blood pressure medicine so I’m confident in it.

    Ok, I guess I should stop talking because this is getting really long! Thankyou for whoever happens to read this and reply to it!!

    Kisato

    LPHS

    My name is Joy and at 39 my life is far from a joy. Amoung a multitude of other health issues, about three years ago I began to have blood in my urine and a nagging constant pain in my right loin region. I was sent to many specialist and endured many tests to no avail. Having a history of major depression and bipolar many of the doctors seemed to allow themselves to write it all off as an extension those somehow. I was tired of being in pain, I was scared everytime I went to the bathroom and saw the blood and wondered why with such an objective symptom the doctors could possibly be blowing me off as “mental”.

    Finally, last year I was diagnosed with Lion Pain Hematuria Syndrome and although I was relieved to have a name for my problem I am still very frustrated. My pain is constant and sometimes nearly unbearable. My quality of life has gone way downhill. I have two children and a home to care for and have found myself increasingly unable to do so. I am on large doses of narcotics for the pain but I tend to grow a tolerence for anything they give me and it stops working. In another week I will be going to pain management. My Nephrologist has told me several options pain management might give me one of them being a nerve block. If there is someone out there who has had a nerve block I would be interested in knowing if it helped. Also anyone who has had success with any treatment for LPHS and might like to share with someone who is desperate for relief and maybe more options for treatment I would appreciate any feedback.

    Thank you for any help or advice anyone may have,
    Joy

    Afraid of pain

    My name is Tara and I am from Ohio. I am 29 years old. I have a daughter that is 8. Everything she knows is mommy being sick.

    I have Lupus and Loin Pain Hematuria Syndrome. It struck me in 2000. I had several kidney stones and had to have surgery to remove them. Ever since I have had dibilitating pain. I went to many urologist and they all told me the pain was in my head. went on antidepressants and began thinking maybe it was me! I met a wonderful urologist who said I was the first patient he had diagnosed with LPHS. That’s when all my medications started being prescribed. The pain was not being taken care of. I could barely get out of bed. I was vomitting every other day. I had lost 30 pounds in 4 weeks. I slept 20 hours out of everyday. How could I take care of my daughter? My pain was not my first worry but my daughter seeing me that way and worrying what kind of chances she will have in getting these diseases. That thought just killed me. My depression sunk even lower and I was going through a divorce. I noticed I was a totally different person and hated it.

    I look back now and I am so glad I made journal enteries. I often thought how much easier it would be if I just died. Now that I am on a road to becoming myself again, I can’t believe what I wanted to do to myself.

    I have recently had a pain pump implanted. I am having alot of success with the procedure. However it is causing new pains. My right leg is completely numb and I am having awful pain that meds are not helping. They think that my pump is sitting on a nerve. The only way to fix that is to have the surgery done again, moved and have the possibility of it happening on the other side.
    I would recommend this to anyone with chronic pain if you are a canidate for it. It really has given me a piece of my life back. I no longer feel like I am dying. I am able to take my child outside to play and out shopping. I am still living with pain but it is much tolerable. My Lupus still is something I am dealing with daily. But the rare kidney disease is so much better. I am lucky that I have wonderful doctors that have supported me. I was a guinea pig in there office for the pain pump. They knew of how bad the pain had effected me and decided this was my only hope. God bless smart doctors.

    If anyone would like information, I would be glad to answer your questions the best I can. Thanks for reading my story. Hopefully I will chat with those of you who share similar situation. I find it is a stress reliever to know there are people out there that are in my same boat. My husband doesn’t understand so it has been a long, hard road alone.

    twehe@wideopenwest.com
    Tara