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Interstitial cystitis/bladder pain syndrome

Hunner lesion
Hypersensitive bladder

EDITOR: Jane Meijlink.
May 2017

CURRENT DEFINITIONS

Interstitial Cystitis/Bladder Pain Syndrome (IC/BPS). Persistent or recurrent chronic pelvic pain, pressure or discomfort perceived to be related to the urinary bladder accompanied by at least one other urinary symptom such as an urgent need to void or urinary frequency. Diagnosed in the absence of any identifiable pathology which could explain these symptoms.

Interstitial Cystitis/Hunner lesion. Interstitial cystitis with Hunner lesion has the same symptoms as IC/BPS. Identified on the basis of cystoscopic findings.

Hypersensitive Bladder (HSB). Hypersensitive bladder symptoms (increased bladder sensation, usually associated with increased urinary frequency and night, with or without bladder pain) in the absence of pathology explaining the symptoms. (Homma et al, 2016)

TAXONOMY: chronic pelvic pain syndromes; lower urinary tract domain; bladder.


EXPLANATORY NOTES FOR CURRENT ICS TERMINOLOGY AND DEFINITIONS


These terms/definitions and descriptions derive from “A standard for terminology in chronic pelvic pain syndromes: A report from the chronic pelvic pain working group of the international continence society.”
Doggweiler R, Whitmore KE, Meijlink JM, Drake MJ, Frawley H, Nordling J, Hanno P, Fraser MO, Homma Y, Garrido G, Gomes MJ, Elneil S, van de Merwe JP, Lin ATL, Tomoe H. A standard for terminology in chronic pelvic pain syndromes: A report from the chronic pelvic pain working group of the international continence society. Neurourol Urodyn. 2017 Apr;36(4):984-1008. doi: 10.1002/nau.23072. Epub 2016 Aug 26.
A Working Group on Chronic Pelvic Pain was set up by the ICS Standardisation Steering Committee in 2011 to address many relevant issues and controversies in the field of chronic pelvic pain syndromes, including suitable terminology, taking global aspects and different viewpoints into account. This resulted in the publication of this first Standard for terminology in chronic pelvic pain syndromes. The new terminology and definitions supersede previous ICS standardisation documents.


This new ICS CPPS Standard should be seen as complementary to other CPPS and IC/BPS standards and guidelines notably:
- The American Urological Association (AUA) guidelines for the diagnosis and the treatment of IC/BP. Available online at: https://www.auanet.org/guidelines/interstitial-cystitis/bladder-pain-syndrome-(2011-amended-2014)
- The IASP Taxonomy, which classified pain on the basis of “organ + pain + syndrome” and applied it to pain of urogenital origin. Available online at: http://www.iasp-pain.org/files/Content/ContentFolders/Publications2/ClassificationofChronicPain/Part_II-F.pdf
- The EAU Guidelines on Chronic Pelvic Pain which place CPP in a clinical context. Available online at: http://uroweb.org/wp-content/uploads/25-Chronic-Pelvic-Pain_LR_full.pdf
- The International Society for the Study of Bladder Pain Syndrome (ESSIC) which published diagnostic criteria, classification, and nomenclature for bladder pain syndrome (BPS) in 2008.
Van de Merwe JP, Nordling J, Bouchelouche P, Bouchelouche K, Cervigni M, Daha LK, et al. Diagnostic criteria, classification, and nomenclature for painful bladder syndrome/interstitial cystitis: an ESSIC proposal. Eur Urol. 2008 Jan;53(1):60-7. Epub 2007 Sep 20.
- The East Asian IC Study Group/Society of Interstitial Cystitis of Japan (SICJ) guidelines which revived the concept of hypersensitive bladder.
Homma Y, Ueda T, Tomoe H, Lin AT, Kuo HC, Lee MH, Oh SJ, Kim JC, Lee KS. Clinical guidelines for interstitial cystitis and hypersensitive bladder updated in 2015. Int J Urol. 2016 Jul;23(7):542-9. doi: 10.1111/iju.13118. Epub 2016 May 24.
- International Consultation on Incontinence. Bladder Pain Syndrome Committee of the International Consultation on Incontinence (Hanno et al. 2009) (note: new version awaited)


- The new first Joint RCOG/BSUG Guideline on Management of Bladder Pain Syndrome, a joint initiative of the Royal College of Obstetricians and Gynaecologists (BJOG) and the British Society of Urogynaecology (BSUG). Available online at http://onlinelibrary.wiley.com/doi/10.1111/1471-0528.14310/epdf.
Tirlapur SA, Birch JV, Carberry CL, Khan KS, Latthe PM, Jha S, Ward KL, Irving A on behalf of the Royal College of Obstetricians and Gynaecologists. BJOG. 2017 Jan;124(2):e46-e72. doi: 10.1111/1471-0528.14310. Epub 2016 Dec 9. RCOG Green-top Guideline No. 70

NOTES ON ADJUSTMENTS TO TERMINOLOGY AND DEFINITIONS
Discussions on nomenclature by the working group partly focused on the risk of inadequate patient care if diagnostic terminology is changed without taking into account the practical impact of its application on the patient’s access to appropriate treatment, reimbursement and social benefits as well as ensuring an accurate diagnosis.

Change from PBS to BPS in line with other society guidelines and nomenclature. However, placing IC before BPS (i.e. IC/BPS) is specifically aimed at ensuring access (in electronic systems) by the patient to appropriate treatment, reimbursement and social benefits. See Discussion concerning nomenclature and “pain in the name” below.

Hunner lesion is considered preferable to Hunner’s ulcer. These lesions are not true ulcers, but an inflammatory infiltrate. Continued use of the old term Hunner’s ulcer may lead clinicians to believe they are looking for ulcers and consequently miss lesions and fail to correctly diagnose the patient.

Hypersensitive bladder: newly introduced, although it is a revival by Japanese and East Asian IC study groups of an earlier ICS term in a 1988 standardisation document where it denoted sensory urgency. See more below under discussion.

Urgency: The Working Group proposes that this should be changed to “a compelling need to urinate which is difficult to defer (pain, pressure, discomfort)”. The Working Group identified the following adjustments as applying more descriptively to Interstitial Cystitis/Bladder Pain Syndrome/Hypersensitive Bladder patients: a compelling need to urinate, due to pain or an unpleasant sensation, that is difficult to defer.
The proposed change in the definition of urgency in this CPPS standardisation document is removal of the word “sudden” which is not appropriate for IC/BPS patients.

The ICS 1988 definition of urgency (“Urgency may be associated with two types of dysfunction: a. Overactive detrusor function (motor urgency), b. Hypersensitivity (sensory urgency)”) was changed in the 2002 LUTS document to “a sudden compelling desire to void”, thereby effectively restricting this term to urgency incontinence in overactive bladder. There was now no mention of any other sensation of urgency due to pain or hypersensitivity. Two practical consequences of this re-definition were a) that many researchers and physicians were now claiming that on the basis of the 2002 LUTS standardisation document IC patients do not suffer from urgency even though urgency has been a key symptom of IC/BPS for almost 2 centuries and b) no progress was made in research into urgency in IC patients. It is hoped that the Standardisation Steering Committee’s current LUT working group will produce patient-centred solutions.
Abrams P, Blaivas JG, Stanton SL, Andersen JT. The standardisation of terminology of lower urinary tract function. The International Continence Society Committee on Standardisation of Terminology. Scand J Urol Nephrol Suppl. 1988;114:5-19.
Abrams P, Cardozo L, Fall M, Griffiths D, Rosier P, Ulmsten U, van Kerrebroeck P, Victor A, Wein A; Standardisation Sub-committee of the International Continence Society. The standardisation of terminology of lower urinary tract function: report from the Standardisation Sub-committee of the International Continence Society. Neurourol Urodyn. 2002;21(2):167-78.

Ketamine cystitis (street ketamine associated cystitis) is now mentioned in the Standard as a new condition and differential diagnosis not previously described in earlier standardisation documents. It is caused by recreational ketamine abuse and includes increased voiding, frequency, dysuria, bladder pain and hematuria. The affected bladder exhibits a denudation of the urothelium with inflammatory cell infiltration. The upper urinary tract is also damaged in patients who use a higher dose and with a longer duration. Katamin e is not only cheaply available but is also highly addictive. Attention by both medical organizations and social workers for this increasing social phenomenon particularly among young people is now urgently needed since in the worst-case scenario it can necessitate cystectomy.
Chen CH, Lee MH, Chen YC, Lin MF. Ketamine-snorting associated cystitis. J Formos Med Assoc. 2011;110(12):787-91.
Winstock AR, Mitcheson L, Gillatt DA, Cottrell AM. The prevalence and natural history of urinary symptoms among recreational ketamine users. BJU Int. 2012;110(11):1762-6.

DISCUSSION
- Pain in the Name
The issue of pain in the name of the condition – painful bladder syndrome or bladder pain syndrome – has been controversial since 2002 when Abrams et al. introduced it in the ICS Standardisation of Terminology of Lower Urinary Tract Symptoms. The authors reserved the historic term Interstitial Cystitis (IC) for patients with “typical cystoscopic and histological features”. However, they did not specify exactly what these typical features were, thereby leading to more confusion.
Pain in the name gives the impression that all patients must have pain and that the disease revolves around the symptom of pain only, whereas urgency and frequency have also been key symptoms since the condition first began to be identified.
Instead of pain, some patients may feel an unpleasant sensation, discomfort, irritation, pressure or a feeling of fullness (even when there are only a few drops of urine in the bladder). However, the patient does not consider this to be “pain” and therefore when questioned by the physician may deny having pain. Patients do not interpret discomfort or pressure as being pain.
This led the Japanese Society of Interstitial Cystitis of Japan (SICJ) to publish a guideline in which they revived the old ICS term of bladder hypersensitivity, ultimately resulting in the umbrella term “hypersensitive bladder (HSB)”. Homma et al believe that the persistent need to void due to discomfort or irritation when there is only a little urine in the bladder is suggestive of increased sensation of the bladder or urothelium. Hence, they proposed the term hypersensitive bladder. This concept was further developed within the framework of an East Asian IC research group and updated in 2015. (Homma Y, Ueda T, Tomoe H, Lin AT, Kuo HC, Lee MH, Oh SJ, Kim JC, Lee KS. Clinical guidelines for interstitial cystitis and hypersensitive bladder updated in 2015. Int J Urol. 2016 Jul;23(7):542-9. doi: 10.1111/iju.13118. Epub 2016 May 24.).
Around the same time, a French research group in Nantes, led by J.J. Labat MD, developed a visceral hypersensitivity proposal in relation to bladder, bowel, vulva, urethra and prostate. This has attracted considerable interest in the abdominal and pelvic pain world.
In fact, the umbrella term hypersensitive bladder – as used in East Asia - covers all patients, excludes no-one and lends itself to sub-division or sub-typing. Furthermore, it can ensure that the group of patients with discomfort rather than pain are not excluded from research.

- CONFUSION AND LACK OF INTERNATIONAL CONSENSUS IN TERMINOLOGY AND DEFINITIONS
Guy Hunner realized in 1918 that consensus on terminology and definitions is essential to make progress. Regrettably, we still have no international consensus in this field and in recent years many societies have been creating their own definitions, nomenclature, classifications and taxonomy, leading to confusion and controversy amongst researchers, clinicians and patients. (Meijlink JM. Interstitial cystitis and the painful bladder: a brief history of nomenclature, definitions and criteria. Int J Urol. 2014 Apr;21 Suppl 1:4-12. doi: 10.1111/iju.12307.) Still more confusion is created by the fact that the same nomenclature may have different definitions and usage in different parts of the world.
In practical terms, this means that statistics are unreliable, including prevalence and incidence figures, and accurate comparison of studies and drug trials is impossible. The result is that we have progressed no further with insight or meaningful phenotyping, with treatment consequently remaining trial and error. It is ultimately a disaster for the patient.
We live in an electronic world of local, national and international medical storage systems and coding, search engines and the internet. Researchers, clinicians, healthcare authorities and patients are all seeking information. Multiple terms, abbreviations and definitions for the same condition not only cause confusion but may lead to refusal by health authorities to reimburse the cost of treatment or even to recognise a diagnosis if the name differs from the coding name. Furthermore, the name and definition of a condition must ensure that it truly reflects all the key symptoms as experienced by the patient. This means going back to the full spectrum of patients and listening to their symptoms and not imposing unproven and unrealistic theories.
The goal should be to achieve international agreement and patient-centred standardisation by working closely with guideline organisations such as the AUA, East Asian IC Study Group/SICJ, EAU, ESSIC, FGIDS, IASP and others as well as the patient associations which have a wealth of information about all aspects of the condition and the impact on the patient at their fingertips.

Resources:
International Continence Society’s most recent publication on this topic:
Doggweiler R, Whitmore KE, Meijlink JM, Drake MJ, Frawley H, Nordling J, Hanno P, Fraser MO, Homma Y, Garrido G, Gomes MJ, Elneil S, van de Merwe JP, Lin ATL, Tomoe H. A standard for terminology in chronic pelvic pain syndromes: A report from the chronic pelvic pain working group of the international continence society. Neurourol Urodyn. 2017 Apr;36(4):984-1008. doi: 10.1002/nau.23072. Epub 2016 Aug 26.
Books:
Urological and Gynaecological Chronic Pelvic Pain. Current Therapies. Moldwin RM (Editor). Springer 2017.
Bladder Pain Syndrome, A Guide for Clinicians. Jørgen Nordling, Jean-Jacques Wyndaele, Joop P van de Merwe, Pierre Bouchelouche, Mauro Cervigni, Magnus Fall (Editors.) Springer 2013.
Articles:
Abrams P, Blaivas JG, Stanton SL, Andersen JT. The standardisation of terminology of lower urinary tract function. The International Continence Society Committee on Standardisation of Terminology. Scand J Urol Nephrol Suppl. 1988;114:5-19.
Abrams P, Cardozo L, Fall M, Griffiths D, Rosier P, Ulmsten U, van Kerrebroeck P, Victor A, Wein A; Standardisation Sub-committee of the International Continence Society. The standardisation of terminology of lower urinary tract function: report from the Standardisation Sub-committee of the International Continence Society. Neurourol Urodyn. 2002;21(2):167-78.
Chen CH, Lee MH, Chen YC, Lin MF. Ketamine-snorting associated cystitis. J Formos Med Assoc. 2011;110(12):787-91.
Homma Y, Ueda T, Tomoe H, Lin AT, Kuo HC, Lee MH, Oh SJ, Kim JC, Lee KS. Clinical guidelines for interstitial cystitis and hypersensitive bladder updated in 2015. Int J Urol. 2016 Jul;23(7):542-9. doi: 10.1111/iju.13118. Epub 2016 May 24.
Meijlink JM. Interstitial cystitis and the painful bladder: a brief history of nomenclature, definitions and criteria. Int J Urol. 2014 Apr;21 Suppl 1:4-12. doi: 10.1111/iju.12307.
Meijlink JM. Patient-centred standardization in interstitial cystitis/bladder pain syndrome—a PLEA. Transl Androl Urol 2015;4(5):499-505.
Rosier PF, de Ridder D, Meijlink J, Webb R, Whitmore K, Drake MJ. Developing evidence-based standards for diagnosis and management of lower urinary tract or pelvic floor dysfunction. Neurourol Urodyn. 2012 Jun;31(5):621-4. doi: 10.1002/nau.21253. Epub 2012 Mar 6.
Van de Merwe JP, Nordling J, et al. Diagnostic Criteria, Classification, and Nomenclature for Painful Bladder Syndrome/Interstitial Cystitis: An ESSIC Proposal. Eur Urol 2008;53:60-7. Epub 2007 Sep 20.
Winstock AR, Mitcheson L, Gillatt DA, Cottrell AM. The prevalence and natural history of urinary symptoms among recreational ketamine users. BJU Int. 2012;110(11):1762-6.