What is Interstitial Cystitis/Bladder Pain Syndrome?
Interstitial cystitis or bladder pain syndrome (IC/BPS), formerly simply known as interstitial cystitis, is a chronic pelvic condition lasting for more than 6 weeks. It seemingly affects the urinary bladder, causing symptoms like discomfort, pressure, or pain. It’s characterized by long-term inflammation and issues related to the lower urinary tract – the part of your body that makes, stores, and removes urine. This is not due to infection or any other easily identifiable causes.
Different health societies and organizations may have different definitions, but we use the definition provided by the American Urological Association (AUA). The diagnosis of IC/BPS is often delayed or mistaken for other conditions, particularly in men, such as chronic prostatitis (long-term inflammation of the prostate gland) or overactive bladder.
Patients often experience pain in the bladder or above the pubic bone, accompanied by a powerful urge to urinate. This feeling often gets worse when the bladder fills up, but can be momentarily relieved by urinating. This typically leads to frequent urination, both day and night. Regular treatments for overactive bladder tend to be ineffective against this urinary frequency, which might suggest the diagnosis of IC/BPS.
Patients with this condition may also experience other symptoms such as pain or burning during urination and discomfort during sexual intercourse, causing pain in women during intercourse and pain in men during ejaculation. These lasting symptoms can have a profound effect on a patient’s emotional, psychological, and social health, as well as their overall quality of life.
The urinary bladder is a hollow organ located in the pelvic region, before the rectum in both sexes and before the womb in women. It is partially covered by a layer of tissue known as the peritoneum on the top surface. The bladder is made up of 4 layers:
– The mucosa, which is the layer lining the bladder, stretches to allow the bladder to fill up. When the bladder is full, the mucosa is smooth, but when it is empty, there are folds in the mucosa, known as rugae. The mucosa also contains a substance that acts as a protective coating to prevent inflammation caused by potentially irritating substances in the urine.
– The submucosa is made of elastic connective tissue that helps the bladder stretch.
– The muscular layer, referred as detrusor muscle, consists of several layers of smooth muscle oriented in various directions, which contract together to empty the bladder. There’s a band of muscles, known as the internal urinary sphincter, between the bladder and urethra, controlled by the involuntary nervous system. There’s another band called the external sphincter, which is controlled voluntarily, around the urethra.
In women, the external sphincter is below the bladder neck towards the pubic bone, while the top part of the vagina is immediately behind it.
– The outside layer of the bladder is a fibrous connective tissue, except for the top surface, which is covered by the peritoneum.
There’s a fixed, triangular area of the bladder called the trigone which contains the locations where the ureters (tubes carrying urine from kidneys to bladder) enter the bladder and where urine exits to the urethra. When the bladder gets full, urine doesn’t flow back into the kidney due to the way the ureters enter the bladder’s muscle. This acts as a valve, preventing urine from flowing back into the kidneys.
What Causes Interstitial Cystitis/Bladder Pain Syndrome?
The cause of IC/BPS, also known as Interstitial Cystitis or Bladder Pain Syndrome, is not well understood. Today, it’s thought that the illness is caused by various factors.
Research suggests that there’s an underlying inflammation precisely causing this, but it is not yet clear. When someone with a normal bladder undergoes a procedure called cystoscopy, the inside of the bladder, called the mucosa, appears pink and without redness or swellings. In people with IC/BPS however, inflammation beneath the mucosa layer is observed. This is often seen as glomerulations and larger than usual groupings of immune cells called mast cells.
IC/BPS patients show reduced levels of GAG (glycosaminoglycans) and abnormalities in the structure of connective tissues, found through biopsies. This leads to the weakening of the bladder’s protective barrier, causing what’s known as a “leaky” bladder lining. That’s also the reason why more immune proteins and immune system regulators are found in the urine of suffering individuals.
In addition, long-term inflammation could result in the thickening of tissue (fibrosis), which leads to the overproduction of proteins in the extracellular matrix (i.e., material outside cells), increased myofibroblasts (cells involved in healing wounds), and reduced blood vessels’ density. This could lessen the bladder’s size, leading to additional stretching and irritation of nerve fibers that sense pain.
Further, IC/BPS patients seem to have more significant grey matter (a part of the brain) volume in areas responsible for sensing pain, as observed in brain scans.
Additionally, severe IC-like symptoms can occur after using illegally obtained ketamine, but the exact reason for this reaction is still unknown. It’s suggested that damage to the bladder lining, changes in the small blood vessels, autoimmune response, and infections by ketamine or its leftover molecules could contribute. These symptoms, cystoscopy results, and biopsy findings resemble IC, but they’re mainly distinguished by the use of ketamine for recreational purposes.
The leading theories behind IC/BPS’s causes are thought to include an autoimmune or immune response, long-lasting inflammation, chronic stress, fibrosis, heightened sensitivity to pain linked to increased grey matter volume, dysfunction or overactivity of mast cells, nerve-related inflammation/swelling, issues with the pelvic floor muscles, increased activation and growth of nerve fibers that sense changes, bladder lining dysfunction and exposure, and irregularities in blood vessels seen as glomerulations when cystoscopy is performed.
Risk Factors and Frequency for Interstitial Cystitis/Bladder Pain Syndrome
IC/BPS, also known as interstitial cystitis or bladder pain syndrome, is a hard condition to diagnose early because there isn’t a universally accepted screening tool. Because of this, the information we have on how many people have IC/BPS is limited.
Researchers have used two main methods to study how common IC/BPS is. Some studies based their results on diagnoses made by doctors or urologists, while others relied on patients’ answers to questionnaires about their symptoms. The rate of IC/BPS appears to be lower when doctors use questionnaires and tissue samples to make the diagnosis.
- In a large US study that used questionnaires, 2.7% of women and 1.9% of men met the criteria for IC/BPS.
- Other estimates suggest that more than 6% of people might have IC/BPS, with women being more affected than men.
- With regard to age, IC/BPS is most prevalent in women aged 50 to 59 and men aged 56 to 74.
- Between 3 and 8 million women and 1 to 4 million men in the US could have IC/BPS, but the actual number of affected men might be higher because many are misdiagnosed with chronic prostatitis, a prostate condition.
- Research indicates that IC/BPS is more common in women, with one study finding that women are five times more likely to have it than men.
- On the lower end of estimates, a study in the Netherlands found that only 8 to 16 out of every 100,000 people had IC/BPS.
- In the UK, it’s estimated that IC/BPS affects up to 400,000 patients, almost 90% of whom are women aged 50 to 69.
- There’s not much data on how common IC/BPS is in children, but most experts agree that it’s very rare in this age group.
Signs and Symptoms of Interstitial Cystitis/Bladder Pain Syndrome
Interstitial Cystitis/Bladder Pain Syndrome (IC/BPS) is a condition that presents with symptoms that persist for at least six weeks and can’t be easily explained by any standard tests or diagnoses. Common symptoms include frequently needing to urinate, discomfort during the night (nocturia), and pain above the pelvic area, which can linger even after using the washroom. People with IC/BPS may also experience a sudden, strong urge to urinate (urgency), discomfort or pain during urination (dysuria), but it’s less common for them to have loss of bladder control (incontinence). Typically, the discomfort can be somewhat eased through urination, but not entirely.
To effectively evaluate treatment options, it’s recommendable to maintain a record of the patient’s symptoms. This could be a pain scale index, a visual chart, or a diary recording a 24-hour voiding pattern. Several tools such as the Interstitial Cystitis Symptoms Index (ICSI), a visual analog chart, or the genitourinary pain index (GUPI) can be utilized to evaluate and record pain levels. The frequency of urination can be gauged by noting voided volumes and maintaining a 24-hour timed voiding diary.
The condition often shows periodic flares followed by periods of less severe symptoms. However, total recovery is rare, and over time, patients often struggle with anxiety, depression, and sleep disturbances.
- Patient symptoms to be recorded include:
- Number of voids per day
- Urine volume per void
- Incidences of incontinence, urgency or the presence of blood in urine (hematuria)
- Pain characteristics like character, duration, location, and severity
- If the pain is worsened during sexual intercourse (dyspareunia) in women or if there’s difficulty or pain during ejaculation in men
- Any pressure or urge to void
- Unexplained fevers
- Pain in the vulvar area (in women)
All patients suspected of having IC/BPS should undergo neurological and gynecological examinations, including tests for post-void residual urine volume. It’s critical to consider IC/BPS in patients diagnosed with an overactive bladder but aren’t improving with standard therapy. Infrequent voiding or unusually high voided volumes might suggest another diagnosis.
Some experts suggest that chronic prostatitis in men might often be misdiagnosed as IC/BPS. Both conditions can cause overlap in symptoms like chronic pelvic pain, urinary urgency, and frequency. However, they are primarily differentiated based on the absence of specific bladder conditions in chronic prostatitis.
Patients with IC/BPS or chronic prostatitis often have more issues with sexual function compared to those without these conditions, and are also more likely to experience depression, pain, and stress.
If standard therapies for IC/BPS are not showing success, it’s possible the patient could be experiencing chronic prostatitis, and a change in treatment plan should be considered. Other conditions like pudendal neuralgia, characterized by intense pelvic pain and urinary dysfunction, can mimic IC/BPS and should be considered as a possible diagnosis for unresponsive patients.
Testing for Interstitial Cystitis/Bladder Pain Syndrome
For suspected IC/BPS (Interstitial Cystitis/Bladder Pain Syndrome) cases, your doctor might propose a series of tests to help make an accurate diagnosis. The goal is to rule out any other conditions that might have similar symptoms to IC/BPS. These tests might include standard blood tests, urine tests, and sexually transmitted infection screening. Certain patients, even with negative urinalyses, might need urine cultures to identify any low levels of bacteria that could still be significant.
Although cystoscopy and urodynamics – two procedures that inspect the bladder – are not necessary according to the American Urological Association (AUA) guidelines, they might be conducted if IC/BPS diagnosis remains uncertain. Cystoscopy refers to using a special instrument to look inside the bladder, and urodynamics tests your bladder’s functionality.
Patients of IC/BPS who have ‘Hunner ulcers’, typically experience higher symptom scores, urinate more frequently, especially at night, and have a decreased bladder capacity. Also, they’re more likely to have an autoimmune disorder. A Hunner ulcer is a specific type of lesion or sore in the bladder.
There are a few substances or biomarkers tied to inflammation, like TNF-α, PGE2, IL-6, and IL-8, that might be higher in patients with IC/BPS, however, whether these could be used for diagnosis is still uncertain.
A cystoscopy might be needed if there’s a potential for bladder cancer, or any foreign bodies, outlet obstruction, strictures, or stones in the bladder. The findings on a cystoscopy are usually non-specific. But, a Hunner ulcer diagnosis might be confirmed as it’s characteristic of IC/BPS. Patients over the age of 50 typically get this diagnosis and might therefore justify having a cystoscopy. The procedure is advised against in younger folks due to the potential for complications and adverse effects.
An explanation of a Hunner ulcer typically describes it as a central scar in a red inflamed area. Small blood vessels are seen radiating towards this scar. Post the pressure expansion of the bladder (hydrodistension), the surrounding area breaks open, leading to bleeding commonly seen with this lesion. Patients having Hunner ulcer usually respond well to treatment.
Generally, bladder biopsies or the removal of cells or tissues to view under a microscope for signs of cancer, are not suggested or required, unless cancer is suspected. However, some medical bodies recommend performing hydrodistension with cystoscopic random biopsies due to the overlap in visual symptoms between IC/BPS and bladder cancer.
A cystoscopy is primarily done to rule out cancer, narrowing (strictures), Hunner ulcers, and bladder outlet blockages. Hunner ulcers are considered diagnostic for IC, but they are quite rare, only seen in about 5 to 10% of cases. Patients having Hunner ulcers usually have the most inflammation and require a more intensive treatment.
Lastly, Urodynamic studies can be helpful in cases where patients don’t respond to standard medication, or there’s evidence of bladder outlet obstruction, weak bladder muscle (detrusor hypotonicity), neurogenic bladder, or other conditions that could explain the patient’s symptoms. However, these studies are not normally advised.
Treatment Options for Interstitial Cystitis/Bladder Pain Syndrome
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What else can Interstitial Cystitis/Bladder Pain Syndrome be?
There is a long list of potential diagnoses that could be mistaken for other conditions. This list isn’t comprehensive, but gives you an idea of how these causes can be grouped:
Infections:
- Sexually transmitted infections like chlamydia and gonorrhoea
- Bladder infections caused by tuberculosis
- Urinary tract infections
Urological and Urogynecological Conditions:
- Bladder stones
- Chronic urethral syndrome
- Lower ureter stones
- Pelvic organ fall
- Prior pelvic surgery, especially if it involved mesh
- Prostate conditions (including benign prostatic hyperplasia, prostate cancer, and chronic prostatitis)
- Bladder tumors
- Neurogenic bladder
- Overactive bladder
- Stress incontinence
- Urge incontinence or bladder instability
Gynecological Conditions:
- Endometriosis
- Pelvic inflammatory disease
- Pregnancy
- Vulvodynia (chronic pain in the vulva)
Neurological Conditions:
- Cauda equina or cord compression
- Demyelinating diseases
- Diabetic neuropathy
- Pudendal neuralgia (chronic pain in the pudendal nerve)
- Stroke
Other Causes:
- Birth defects of the bladder
- Diabetes
- Diverticular disease
- Inflammatory bowel disease
- Radiation effects
- Rheumatoid arthritis
- Trauma
- Bladder diverticula (outpouchings of the bladder wall)
What to expect with Interstitial Cystitis/Bladder Pain Syndrome
Most patients experience long-lasting symptoms that come and go over varying periods of time, but the way the condition progresses, resolves, and its overall prognosis can greatly differ from person to person. We have noticed a few different patterns of how the disease can unfold. These include:
- Total resolution, which means all symptoms go away completely
- Relapsing-remitting, where symptoms disappear and reappear over time
- Intermittent disease flares, where symptoms periodically become worse
- Chronic progression, where symptoms persist and may slowly get worse over time
It’s also possible that patients may see their symptoms resolve on their own and then unexpectedly come back, regardless of any treatments they are receiving.
From what we’ve seen, IC/BPS (Interstitial Cystitis/Bladder Pain Syndrome) seems to be a very stable condition. This means that urinary symptoms and discomfort generally stay the same in severity for up to 9 years.
Possible Complications When Diagnosed with Interstitial Cystitis/Bladder Pain Syndrome
If bladder issues are not treated or don’t naturally resolve, further hardening might happen, making the bladder smaller and worsening symptoms. The person would likely experience problems with sleep, sexual function, mood, and social embarrassment.
The complications from treatment can vary depending on the approach, like medications, cystoscopy, or surgery. Prior proper counseling and agreement to treatment are critical. Some common complications could be:
- Bleeding
- COVID-19
- Death (from the procedure or anesthesia)
- Infection (from the treatment or surgery)
- Bladder tearing during high-pressure treatments
- Injury
There could also be complications from anesthesia, mostly due to human errors, with a minor chance of death (around 0.4 per 100,000 patients). Anesthesia-related complications could include:
- Severe allergic reactions
- Choking on food or drink
- Sudden drop in full-body blood flow
- Errors in medication delivery
- Nausea and vomiting
- Severe slowing of breathing
Also, there is a risk of urinary tract infections. Symptoms might change and get more severe during this time, so tests for infections should always be done under such circumstances. This happens when the bladder lining is damaged, which lets bacteria in, leading to bladder injury and more symptoms. In some cases, a short course of antibiotics might be used, but long-term treatment is not advisable.
Preventing Interstitial Cystitis/Bladder Pain Syndrome
Once a doctor has diagnosed your condition, they will explain what your diagnosis means, why you’re experiencing the symptoms you have, and that the causes of the condition aren’t entirely understood yet.
You will be encouraged to join self-help and local support groups. These groups provide a platform to share experiences and strategies for managing the condition. In the United States, you can consider joining groups like the Interstitial Cystitis Association (https://www.ichelp.org/), while in the United Kingdom there are options like Bladder Health UK and PainUK. Some patients found these self-help resources to be an effective way of living with the condition.
The treatment for your condition could vary, and the outcomes could also differ from person to person. It might take several months before you notice any improvements, and even then, they might be only slight. It’s important to understand that your condition might not be completely curable and symptoms might recurr. However, with the right treatment, there can be significant improvements in your symptoms, which can enhance your quality of life in a big way.
