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.

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    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.

  • 27 thoughts on “Loin Pain Hematuria Syndrome (LPHS)”

    1. hello,
      I am Rebecca a 21yr old female from Melbourne Australia who has LPHS, it first started in 2009 with only a little bit of burning pain on my left kidney area and a slight red colour in my urine. Its now 2013 and since then the pain has incressed by almost 100% with me crying almost every night and being grumpy because of the pain, each time i go to the bathroom my urine is now a deep red colour and often i find it hard to pee as i habe blood clots that block it and it can be almost 7-10hrs before they pass so having a very full bladder and trying to be happy is very hard when you cant pee. I have just seen my Renal Dr at the hospital this morning, i explained to him the trouble it is causing me and the pain im in, its not just the pain and the red urine i have to worry about its also about life, I’ve only just turned 21 and i cant do half the things i use to be able too.. eg; Rowing, Running, Netball, Boxing.. I was very active and use to always be outdoors, but now people find me eaither rugged up at home, shopping or in hospitals 🙁 and i hate it. I have asked the doctor to take my kidney out but first i am going to see the Chronic Pain Team at the hospital but in the meantime im taking lots of panadol, and on slow relase 10mg Oxicontin tablets, they do help with the pain but i still always feel it and even find it hard sitting in the car for ages, or sleeping on my left side. If you know of anything that may be of help for me please let me know.
      warm regards
      Rebecca Milic

      1. Hello Rebecca,
        I live in Arizona, USA and I also suffer from LPHS. There is an incredible support group on Facebook called “Living with LPHS”. Questions, ideas and support are on this page on a constant basis from fellow LPHS sufferers from all over the world. I don’t know what I’d do without the support and friends I’ve made from this group. It’s been a lifesaver. I hope you feel better soon.
        Renee Wilson

      2. wife had hers removed as a form of pai relief
        lphs transfers to the remaining kidney so hope you havent

        after ten years of systemic negligence and untreated pain
        finaly had an intrathecal morphine pump implanted in 2002
        has been ok since then

      3. Hi Rebecca
        I have had pain for the last 12yrs my pain started off as kidney stones and as the years went I had more stones pass and more operations to remove stones the pain became more and more apparent and stayed. I was diagnosed in 2014 with LPHS. I am now at breaking point because I want better quality of life. I like you enjoyed sports and outdoor activities and was competing in 4wd comps. I have had to stop all of this because of the flares after activities. I am in australia and getting answers from doctors is so difficult. I hope you have found a happy medium with managing your pain.

    2. Ik ben een vrouw 55 jaar, in 2005 is bij mij Loin Pain Hematuria Syndrome, gediagnostiseerd door mijn nefroloog in het LUMC te Leiden. Second opinion door professor in het VU te Amsterdam. Ben 1jaar in allerlei onderzoeken geweest en heb in de afgelopen jaren een paar keer een ernstige terugval gehad. Dan werd de medicatie aangepast en heb ook lang met een Tens – apparaat gelopen
      Bij mij is de flankpijn chronisch, sinds een aantal jaren bestaat mijn pijnmedicatie per dag uit: 25 mg Amitriptiline, 2x 300 mg Lyrica en 2x 10 mg Oxycontin. Hiermee is het pijnniveau draaglijk ( 2 tot 3 op schaal van 10) en kan ik 3 dagen per week mijn werk (leerkracht speciaal onderwijs) doen steeds met een herstel- dag na een werkdag. Ben wel voor 1 dag afgekeurd door LPHS.
      Helaas nu een terugval ( pijn 5 – 6 op schaal van 1 – 10) in afwachting van afspraak pijnpoli

    3. I have just been told I have this I seen many doctor and they all said no way and kidneys will not cause pain and been in metro hospital Parma hospital when I was out of town in Cleveland Ohio . I live in KY went to uk hospital and the doctor found it I must say they are very good there but every other doctor said I was find could not find any thing wrong and each one said there was no way that kidney cause pain well there wrong

      1. To Mr Kovar, Did you go to University of Kentucky, or the UK? This is very important. My son has suffered since the tender age of 6 with this LPHS. Possible Nutcracker Syndrome, and he has hyrdonephrosis in the left kidney. He has had test after test done. Need to know more please feel free to call you can block your number if you want. I have questions that I need to ask. Please any help for him i would be so very Greatful. I have read about the autotransplantion seems like to is 70% successful. We are looking into that now. Just wondering if you had that done? Thank you and God bless.

        1. Hi, I am interested as well. I was diagnosed with nutcracker syndrome in 2011, with ovarian vein syndrome, and 10 months ago I suffered the worst pain ever with haematuria. At first it was discussed that the nutcracker symdrome can cause!!! It is now discussed with different specialists that nutcracker symdrome doesn’t usually cause this pain and now I have been referred to another specialist to rule out LPHS. Any suggestions to manage? I need my life back being a mother of 2 beautiful kids.

          1. Hi, my pain story started in 2009, first thinking ovarian cysts, then a CT scan in 2011 showing ovarian vein syndrome with pelvic congestion. Went to vascular surgeon, then told that I have nutcracker syndrome and vein embolization is not an option, pain medication. In 2015 the pain worsened and I started asking for solutions as my quality of life was deteriorating. I saw another vasular surgeon and after a veinograph it was decided to embolised, then 2 weeks after this procedure I went into renal colic and hospitalised for pain relief. More specialist and now in 2016 it has been discussed LPHS. Now life is about pain meds, my pain specialist is trying PENS therapy but out of the 3 session only one helped with 2 days relief. Does anyone have treatments that has given some quality of life back??

        2. my wife did and pain was gone on the right, now has pain on the left flank which she never had before. in the process of scheduling another auto transplant. Its a major surgery but it worked on her right side. No issues. Now we have to deal with the left side. facebook has a lphs page and I believe that auto transplant is the only way to go with out spending a life on narcotics. its a major decision that requires many second opinions and everything else that could be causing the pain should be ruled out accordingly. good luck, stay positive, be strong, and take it day by day. Do your due diligence.

          1. I’m in the process of meeting with a surgeon for autotransplant of my right kidney. Any chance you or your wife would be willing to email/chat somehow? It would be incredibly helpful. I’m a mom, wife…and want to be able to fully be able to fill my role in our family. I’m just so nervous about the surgery and having the pain go to my left kidney. Thank you, I appreciate any feedback.

        3. I have suffered with LPHS for about 20 years , with autotransport it might work for about 6 months & then the disease will move to the good kidney thats what my doctor has told me. I have a spinal cord stimulator implant in me and it works pretty good I still need my pain killers but the implant shots a electrical pulse to the area over my kidney & it dulls the pain a bit. I am in australia & we have some of the best specialists here. i would be lost without my stimulator, I hope this has helped someone out there.

      2. For the past 16 years I have had one doctor after another say you can’t feel your kidneys…..well I sure could and had blood in my urine every time I complained…..13 years ago damage was found in my kidneys to which a referral for biopsy was done, but since my kidneys were “working” they wouldn’t do it. It took all this time and finally a repeated referral to an experienced nephrologist to finally figure it out. But I have been very insistent over the years as I knew exactly where the pain was coming from…my kidneys were speaking to me and the Dr’s wouldn’t listen!! That’s why we have pain … The body is trying to tell us something!!!!

    4. I am a 40-year-old woman from SC. Beginning in November 2009, I began experiencing severe pain in my left flank area with visible blood in the urine. Over the past five years, I have had every test possible with many doctors. In five weeks, I will have a kidney autotransplant. I am currently having severe pain management issues. The stigma associated with chronic pain issues is my driving force in deciding to have this surgery. My transplant surgeon has informed me that he has never done this surgery for someone with LPHS, but has done the research. He has informed me that there is a risk the right kidney could be affected by the LPHS over time. At this point, I am unable to take any more and truly pray for success.

      1. What was your outcome after surgery? I’m in the works of getting this done and I’m absolutely terrified.

      2. I have bilateral LPHS and plan to have both sides done. I have read of cases where the pain transfers to the other if not done. 16 years ago I only had pain in the right kidney several years several years ago I started with both sides with no relief! I feel your pain!!!

    5. I am a 26 year old female that lives in Dallas, TX (been here for a year, originally from Wisconsin). As of today, my nephrologist is very confident that LPHS is what I have. I have episodes of kidney pain that can’t be explained. Ever since I was 6 or 7 I will have severe pain attacks every few months or so and each time I have felt like I was going to die. About 2 1/2 years ago, I was diagnosed with Chronic Kidney Disease, Stage 1. My kidney function has always been ok, however, both of my kidneys are scarred, especially my right and my right is also quite small. Until now, no one could explain why this was. I’ve had numerous tests and procedures done. The severe pain attacks that I’ve had in the past have been isolated, only happening once with the pain dissipating after about an hour or so. The past couple of months have been different. Back in March I didn’t have the severe attack, but a pain that just wouldn’t go away no matter what I did. I’ve been also dealing with reoccuring utis/kidney infections so everyone thought it was that, until my labs all came back negative. I was hospitalized for a week and no one could figure out anything. The past few days I’ve had multiple severe attacks, almost every time I pee and also now nagging pain that likes to switch from my right flank to my left flank. Everyone thought it was kidney stones or an obstruction, but of course nothing showed up on scans or on my labs. Finally today when I saw my nephrologist he sat down with me, went over everything from this weekend, and then told me that whats going on right now is only further confirming his thought on me having LHPS. My only question to others is, does everyone experience blood in their urine? I only ask because even with my infections I’ve had I have not had blood show up. The only time it did was a a year and a half ago and that’s because I had an infection.

      1. the blood is always there most times when you aren’t having a flare it is microscopic. I have blood in my urine and when I have a flare the test it and it never grows anything. I am in Wisconsin and have had a run around even though I have been diagnosed.

      2. I am almost 44 with issues with this over the past 16 years and every time I have had pain I have had blood…..if that helps…..

      3. Bless you.l have the same!For 30 yrs. Everyone said it was my back surgery so l have had alot of procedures never needed.l am angry,in pain and hopeless.l still have that bit of hope but am practically giving up. Lin

    6. I started having horrible pain and urinating blood when I was 11 years old. I would be constantly using the ER to manage my pain art least 3-4 times a week. I went through every test known to man. Only coming up with kidney crystals in my 24 hour urin tests. Finally being prescribed dilaudid pills for my pain when i was 16 even though they could not tell me why I www having this happen. When I was 18 finally had a kidney biopsy and was diagnosed with TBMD with LPHS. I had an autotrandplant done at Lyola hospital in 2010. I was 23 years old. The bleeding and pain stopped and I got to go of all the really strong pain medicine! Now I am 29 and my pain and bleeding came back a year ago. I’m trying to figure out what to do now. I’m going to a methadone clinic just so I don’t have to go to a pain doctor and be back on pills, which through time I had gotten very addicted to. Now the methadone had stopped being able to take the pain away boater how high if a dose I’m at. I know I’ll be back to hospitals and reliving most of my childhood all over again and am so stressed out. I just know that the next step will be talking out my kidney altogether.

    7. I was recently diagnosed with LPHS, about 2 years ago, at the beginning it was really hard, I just want to die, but I realised I have so much yet to do, and I have to be strong. Its still hard for me,till this day, but I promise you, this disease is just a tiny speed bump, you can get over it and go on with your lives as any other normal person would. Be strong, take precautions and never let it control you

    8. I need some help and direction. I was just in the hospital a few days ago. And recently have found out about lphs. I have been having kidneys problems since in my early to mid twentys. I have all symptom for this disorder. But with no insurance I’m unable to see the Dr and specialist I Nees to. Anyone have an thought or ideas?

    9. My name is Brandy I am a 29 yr old mother of 2… I have been in pain my entire life but found out I had chronic kidney stones 10 years ago.. I had a hysterectomy done at 24 for endometrisis and years of having lithotripsys to bust stones…I have had many blood infections caused from stones and been septic…I had surgery on my right kidney to remove 7 stones and insert a stint and also had 8 stones in my left kidney at the same time…all stones were removed and infection taken care of but I have continued to be in chronic pain..most doctors brush my symptoms aside and say I’m too young or I am looking for attention… I don’t have insurance and I can’t work due to my condition so at this point I am desperate… I am looking for any advice on doctors that may take me serious and help me without having insurance and low income…I live in Myrtle Beach if anyone can help please contact me at brandy.brest17@gmail.com… My husband is a disabled combat veteran and I am his caregiver… I need to be treated so I am able to continue taking care of him and our children… Thank you all for any advice

    10. Hello to all reading,
      It’s Rebecca Milic and I thought after 3 years I better give you all an update. So I ended up getting diagnosed with LUPUS SLE in early 2014, I’ve started a chemotherapy Called methotrexate and I take that weekly along with other lupus medication called Plaquenil and Prednisone along with my regular medications of
      •Somac (treatment for acid reflux)
      •Creon 10,000 (Treatment for pancreatic malabsorption, caused by lupus)
      •Ondansetron (Anti nausea)
      •Oxynorm (pain relief
      •OxyContin (Pain Relief)
      •Panadol (4x daily, pain relief)
      •B12 Injections (weekly)
      •Iron Infusion (Monthly)
      So anyway, I feel like everything has gotten slightly better, or I’ve just learnt to cope with it more. I cant work anymore as it is just too hard and I go into terrible flares, so I’m now 24 and having to be supported by my parents, I moved back home and I do odd things around the house and help out when I can but most of the time I’m in bed sick.
      I am always staying positive and hope one day we will find a cure both for LPHS and lupus, but for now, I just pray
      If you at all want to contact me feel free to do so , my email is rebecca.milic@yahoo.com

      Stay smiling, stay positive

      Regards
      Rebecca

    11. Anyone that has had an autotransplant done, please contact me. ashleysarah723@gmail.com
      I’ve been dealing with this pain since 2004, but was officially diagnosed about 2 months ago. I’m in the process of setting up surgery and would love to talk to someone that has had it done. Thank you!

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