Loin Pain Hematuria Syndrome, or LPHS, is a rare and inadequately understood disorder. LPHS can inflict anyone at any age however, it most commonly occurs in young women. Patients with LPHS experience severe unexplained chronic bilateral or unilateral flank pain. This pain is accompanied by gross and/or microscopic hematuria that appears to represent glomerular bleeding. Most patients present with both pain and hematuria although in some cases patients experience just one of the two symptoms.
Causes
Loin Pain Hematuria Syndrome is thought to be the cause of glomerular capillary hemorrhage that results in tubular obstruction and interstitial edema. Eventually, chronic edema is thought to cause capsular distention, pain, hemorrhage and further tubular obstruction. A number of patients with Loin Pain Hematuria Syndrome who undergo biopsy display irregularities in the glomerular basement membrane leading experts to believe LPHS is on the same continuum as Thin Basement Membrane Disease.
Diagnosis
LPHS is a diagnosis of exclusion, all other causes of renal pain and hematuria must be ruled out before a diagnosis of LPHS can be given. Almost half of patients with LPHS also have a history of kidney stones, or nephrolithiasis, suggesting that formally passing renal stones makes a person more probable to acquire the condition. However, stones must be absent while the pain persists in order for LPHS to be properly diagnosed.
LPHS is considered primary when it exists in the absence of an underlying glomerular disease, such as IGA Nephrology, and secondary when it occurs in the presence of one. The loin pain may be a continuous dull ache, or can come and go intermediately. It can range from a mild annoyance to debilitating, effecting every aspect of ones life.
The Hematuria, or blood in the urine, may be visible to the naked eye, coming in sporadic episodes possibly accompanied by occasional blood clots. In other instances the amount of blood is too small to be seen, but when tested reveals microscopic [trace] hematuria.
In many cases pain attacks are triggered by exhaustion, stress, dehydration, physical activity and even simple daily routines such as riding in vehicles or walking the dog. Fatigue, nausea and sometimes fever may occur and spasms or inflammation of the kidneys are often reported. The chronic painful nature of LPHS is likely to lead to depression therefore a supportive team of medical professionals is required. It is important to note that in no case has there been any evidence of a reduction in kidney function or renal failure due to Loin Pain Hematuria Syndrome.
There is little known about the cause of LPHS, making it a challenge to diagnosis & treat. First described in 1967, the modern nature of this illness, lack of evidence and it’s inability to manifest in a recognizable manner makes LPHS very difficult to diagnose, taking years in many cases. Given the rarity of the condition itself and it’s brief history under the medical microscope, little has been done in the way of research on this conundrum. It should be noted that research studies seem to have increased since 2011, however, cause, diagnosis, & treatment outcomes all vary.
Treatment
There is no guaranteed way to treat LPHS. Without a definitive cause, treatment plans focus on treating the symptoms of LPHS. Pain management is the primary focus for patients and doctors dealing with LPHS. Surgical procedures such as auto transplant and denervation have offered mixed results with auto transplant being the most promising, offering pain relief to almost 30% of patients who undergo the procedure. Such treatments are considered invasive and may lead to further complication. In some cases the pain is reported to come back or appear in the opposite kidney. In other cases, post-op complications lead to nephrectomies. Many doctors won’t consider invasive surgery or treatments until more studies with successful outcomes are published. Treatment is between the patient & their primary care & specialists. If you survey a group, you might learn that many manage their LPHS in vastly different ways. Each person experiences LPHS differently, with some commonalities. LPHS also tends to progress & symptoms change over time. Flare ups can sometimes reflect a pattern, but generally do not. It’s a guessing game and managing the illness can take a toll on the patient & their loved ones.
Many patients have a team of doctors & treat LPHS holistically. Being a diagnosis of exclusion, there is a lot of testing and trial & error to find a treatment that gives the patient some level of pain relief and quality of life. Geography dictates treatment as well. LPHS doesn’t discriminate & stretches across the globe. With all the healthcare & insurance changes around the world, quality of care is drastically effected. Treatments vary country to country as well in relation to the countries healthcare system. Ever changing opioid laws & guidelines have effected many treatment options. Many primary care physicians, PCP, historically management medications for their patients because urologists & nephrologists do not manage chronic pain. Urologists tend to manage acute pain for stones & infection. Over the last decade, medication management has shifted almost exclusively to pain management specialists, especially in the United States of America.
My Sun Is Rising 