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The History of IC Subtypes (Phenotypes)

Earlier this summer, there were some patients on Facebook who suggested that IC subtypes were not real. Here is the history of the four subtyping systems proposed for IC/BPS to date. This is a REAL thing that is now helping patients find their unique presentation of IC/BPS so that they can focus on the correct treatment for their unique case. For some, it’s lesions (possibly viral) while others might be struggling with Genitourinary Syndrome of Menopause (hormonal) or chronic infection (bacterial or fungal). For most, however, the pelvic floor muscles are the root cause of their symptoms. Even nerves can be injured and cause similar IC symptoms.

Subtyping has been a topic of debate and conversation among the top IC clinicians for the past decade and you can expect to see several more subtyping systems proposed in the coming few years, including very finely detailed subtypes based upon bladder biopsy results… but anyway… on to the history.

ESSIC

The first two subtypes were proposed by the European Society for the Study of IC/BPS (essic.eu) shortly after they were founded in the early 2000’s. They suggested, and world IC researchers quickly agreed, there were two distinct groups of IC patients – those with Hunner’s lesions and those without Hunner’s lesions. The pathological studies of their biopsy samples made it crystal clear that there were two distinct types of patients.

UPOINT

The second subtyping system proposed was in 2009 by Dan Shoskes (Cleveland Clinic) and J Curtis Nickel (Queens Hospital, Toronto). It is called UPOINT and asked doctors to evaluate patients in six distinct areas:

– what bladder symptoms are present?
– what’s the condition of the bladder (i.e. Hunner’s lesions)?
– does the patient have other related conditions (i.e. IBS)?
– what is the health of the pelvic floor muscles?
– does the patient struggle with anxiety / depression?
– has any infection been found?

This subtyping system has been validated in multiple research studies and now serves as the foundation for IC care in the Canadian Urology Association’s Guidelines for IC/BPS.

INPUT

The third subtyping system proposed is called INPUT. It was adapted by Dr. Shoskes to be more specific for the needs
of IC/chronic bladder pain patients. It asks doctors to assess five key points:

I – Is any infection present?
N – Does the patient also have any other neurologic or systemic conditions
P – Does the patient have any anxiety / depression or other psychosocial issues?
U – Does the patient have Hunner’s ulcers (now known as lesions)
T – Does the patient have tight or dysfunctional pelvic floor muscles.

PAYNE 5 SUBTYPES

The fourth subtyping system was proposed by Dr. Christopher Payne who ran the Stanford University IC research program for twenty years. This is the system that the IC Network currently uses in our coaching sessions because it includes pudendal neuralgia, which the above two do not.

Dr. Payne asks Drs to assess five key areas but he also believes that there are subtypes within subtypes. His system is also the most practical for educating patients.

#1 – IC: Hunner’s lesions

This small subset of patients (10% of the patient population) have inflammatory lesions visible on their bladder wall. In much of the world, only patients with Hunner’s lesions are diagnosed with “interstitial cystitis.” Patients without lesions are diagnosed with “bladder pain syndrome.” Hunner’s lesions require local lesion directed therapy (laser, fulguration, steroid injections) and do not generally respond to oral medications and bladder instillations. A new medical device currently under study, LiRIS (aka LiNKA) is the first treatment in history that has healed and/or reduced the size of lesions in just a two week treatment period.

#2 – BPS: Bladder Wall Phenotype

Patient symptoms often start with a UTI, chemotherapy, chemical exposure or other bladder insult (i.e. drinking excessive amounts of soda, coffee, etc.). Frequency and urgency can occur throughout the day and night. Estrogen atrophy can also influence bladder wall function and sensitivity. Pain increases as the bladder fills with urine and is reduced on emptying. These patients usually find that their pain decreases when an anesthetic (lidocaine) is instilled into the bladder. Treatment priority focuses on calming and soothing the bladder wall, diet modification, OTC supplements, oral medications and other bladder directed therapies.

#3 – BPS: Myofascial Pain Phenotype

These patients often have a history of sports, orthopedic injuries or childbirth trauma. Upon examination, they have pelvic floor tension and the presence of trigger points in their pelvis, abdomen, back and hips that trigger severe symptoms when touched. They may have less diet sensitivity, normal or larger voids, may sleep more comfortably when their muscles are relaxed. Bladder instillation of lidocaine is NOT generally helpful. Treatment priority is pelvic floor physical therapy.

#4 – BPS: Neuralgia Phenotype

These patients can have pelvic floor muscle tension or other causes of pudendal nerve compression. This causes severe burning or electric pain when sitting, “sensory abnormalities in the pudendal distribution” and a positive Tinel’s sign (tingling or pins and needles when the nerve is tapped gently). Pain is not typically linked to bladder function. Treatment starts with identifying causes of injury/irritation, physical therapy and stretching for tight muscles and analgesics specific for neuropathic pain. Nerve blocks can be both diagnostic and therapeutic when conservative measures fail. Surgical nerve release is infrequently needed but can be effective in carefully selected patients.

#5 – Multiple Pain Disorders/ Central Sensitization

These patients have multiple pain disorders (i.e. IBS, vulvodynia, fibromyalgia, etc.). Dr. Payne wrote “Their prognosis is inherently different and the invasive treatments that may be appropriate for pelvic pain phenotypes could actually make things worse…Clinicians should proceed much more cautiously.” These patients often demonstrate other signs of neurosensitization including extremely sensitive skin, diet sensitivity, drug sensitivity, chemical sensitivity and even visual sensitivity. Most also have an extremely sensitive sense of smell. Treatment priorities focus on treating all pain generators to reduce the overall volume of pain in the nervous system, as well as avoiding therapies that can be traumatic and/or irritate nerves. Patients are also encouraged to try cognitive behavioral therapies so that they can learn to control stress and other potential flare triggers. The goal is to maximize the patient’s ability to function in normal activities. Much current research is directed toward this phenotype including the NIDDK MAPP program.

REFERENCES

– Payne C. A New Approach To Urologic Chronic Pelvic Pain Syndromes: Applying Oncologic Principles To ‘Benign’ Conditions. Current Bladder Dysfunct Rep. Topical Collection on Pelvic Pain. March 2015
– Shoskes D, et al. Clinical phenotyping in chronic prostatitis/chronic pelvic pain syndrome and interstitial cystitis: a management strategy for urologic chronic pelvic pain syndromes. Prostate Cancer and Prostatic Diseases (2009) 12, 177–183;
– Crane A, et al. Improving the utility of clinical phenotyping in interstitial cystitis/painful bladder syndrome: From UPOINT to INPUT. JUROL April 2017 Volume 197, Issue 4, Supplement, Pages e386–e387

By |2018-08-20T13:53:01+00:00August 18th, 2018|Awareness, Interstitial Cystitis Network Blog, Research|Comments Off on The History of IC Subtypes (Phenotypes)

About the Author:

My Google Profile+ Jill Heidi Osborne is the president and founder of the Interstitial Cystitis Network, a health education company dedicated to interstitial cystitis, bladder pain syndrome and other pelvic pain disorders. As the editor and lead author of the ICN and the IC Optimist magazine, Jill is proud of the academic recognition that her website has achieved. The University of London rated the ICN as the top IC website for accuracy, credibility, readability and quality. (Int Urogynecol J - April 2013). Harvard Medical School rated both Medscape and the ICN as the top two websites dedicated to IC. (Urology - Sept 11). Jill currently serves on the Congressionally Directed Medical Research Panel (US Army) where she collaborates with researchers to evaluate new IC research studies for possible funding. Jill has conducted and/or collaborates on a variety of IC research studies on new therapeutics, pain care, sexuality, the use of medical marijuana, menopause and the cost of treatments, shining a light on issues that influence patient quality of life. An IC support group leader and national spokesperson for the past 20 years, she has represented the IC community on radio, TV shows, at medical conferences. She has written hundreds of articles on IC and its related conditions. With a Bachelors Degree in Pharmacology and a Masters in Psychology, Jill was named Presidential Management Intern (aka Fellowship) while in graduate school. (She was unable to earn her PhD due to the onset of her IC.) She spends the majority of her time providing WELLNESS COACHING for patients in need and developing new, internet based educational and support tools for IC patients, including the “Living with IC” video series currently on YouTube and the ICN Food List smartphone app! Jill was diagnosed with IC at the age of 32 but first showed symptoms at the age of 12.