Interstitial cystitis (IC) and painful bladder syndrome (PBS) is a chronic and debilitating condition characterised by recurring pain, pressure, and discomfort in the bladder and pelvic region.

Bladder pain syndrome (BPS) forms part of the greater chronic pelvic pain syndrome, with the diagnosis of IC previously reserved for cases with specific cystoscope findings. Nowadays the terms IC and BPS are used interchangeably. This condition primarily affects the urinary system, causing symptoms that can significantly impact a person's quality of life. While the exact cause of interstitial cystitis remains elusive, researchers have made significant strides in understanding its pathophysiology, diagnosis, and treatment options.

Pathophysiology and causes

The underlying mechanisms of interstitial cystitis are multifaceted and not fully understood. However, several theories have emerged to explain its development. One prominent theory suggests that interstitial cystitis involves a dysfunction of the bladder's protective lining (called the glycosaminoglycan or GAG layer), allowing harmful substances in the urine to irritate the bladder wall. This sets off a complex immunological response leading to chronic inflammation, progressive bladder damage and activation of neuronal pain pathways. The inflammation then causes further damage to the GAG layer, leading to further urine solute leakage. Additionally, abnormalities in the immune system and nervous system interactions may contribute to the chronic pain and inflammation characteristic of IC. Genetic factors and autoimmune responses are also believed to play a role in predisposing individuals to the condition. Environmental triggers, such as infections or injuries, could potentially initiate or exacerbate IC symptoms in susceptible individuals. Although mainly found in female patients, males are sometimes affected where it can form part of prostate pain syndrome. Worryingly, the condition is being diagnosed more frequently in pubertal patients.

Symptoms and diagnosis

Interstitial cystitis manifests with a range of symptoms that can vary in intensity and frequency among affected individuals. Common symptoms include urinary urgency, frequent urination (day and night), pain or discomfort in the bladder and pelvic region, and a sensation of pressure in the lower abdomen. Pain during sexual intercourse, known as dyspareunia, is another common symptom that can greatly impact a person's emotional well-being and relationships. Diagnosing interstitial cystitis is challenging due to the absence of a definitive test. Diagnosis is made by a process of exclusion, with an extensive differential diagnosis list. Symptoms should be present for at least six months duration. Patients often developed superimposed urinary tract infections and will often report some improvement of symptoms while taking antibiotics, with symptoms recurring as soon as antibiotics are stopped.

Evaluation should include a combination of clinical history, symptom presentation, physical examination, urine analysis and ultrasound. Urine cytology should be done in individuals with risk factors for urogenital malignancies. Pain diaries, quality of life scores, frequency/volume charts, urodynamic testing etc are important tools to establish a symptom baseline prior to treatment and to assess treatment progress. Cystoscopy with hydrodistension, is one of the diagnostic tools used to observe characteristic signs of IC, such as pinpoint bleeding spots on the bladder lining (known as glomerulations) or Hunner's ulcers. A biopsy can also be done during a cystoscope.

Treatment approaches

As interstitial cystitis is a complex and heterogeneous condition, treatment approaches vary based on individual symptoms and responses. There is no one-size-fits-all solution, and a combination of therapies are often employed to manage the condition.

Treatment principles include:

1. Empathetic acknowledgment: Recognise the patient's disease and validate their concerns, building trust.
2. Reassurance and patient education: Offer emotional support to alleviate patient anxiety and stress. Help the patient to develop insight in into the disease pathophysiology and treatment principles.
3. Conservative start: Begin with less aggressive treatments to minimise risks.
4. Stepwise progression: Treat gradually, starting with one or two modalities and advancing as needed.
5. Patience for results: Allow 6-12 weeks for treatments to take effect before assessing further options.
6. Cease ineffective treatment: Discontinue treatments that show no positive results within a reasonable time.
7. Combined approaches: Use combinations of treatments for potentially better outcomes.

These principles prioritise patient well-being, effective treatment strategies, and a compassionate approach to healthcare.

Treatment options include:

1. Lifestyle modifications: Patients are advised to avoid triggers such as certain foods (spicy foods, caffeine, citrus), alcohol, and smoking, which can exacerbate symptoms. Maintaining a healthy weight, practicing stress management techniques, and adopting proper pelvic floor exercises are also recommended.
2. Pharmacological interventions: Various oral medications, including pain relievers, antihistamines, and medications that help protect the bladder lining, may be prescribed to alleviate symptoms. For severe cases, bladder instillations involving the direct administration of medications into the bladder might be considered.
3. Physical therapy: Pelvic floor physical therapy can be beneficial in addressing muscular tension and dysfunction in the pelvic region, which can contribute to pain and discomfort.
4. Bladder treatments: Bladder instillations of substances like heparin or lidocaine may be used to provide relief from inflammation and pain directly to the bladder lining. Hyaluronic acid is another intravesical installation option which is believed to help alleviate symptoms by providing a protective barrier on the bladder lining, potentially reducing irritation and inflammation.
5. Nerve stimulation: Neuromodulation techniques, such as sacral nerve stimulation, involve electrical stimulation of nerves to modulate bladder function and reduce symptoms.
6. Surgical interventions: In severe cases, surgical options like bladder augmentation or removal of Hunner's ulcers might be considered, though these interventions are typically reserved for individuals who have not responded to other treatments.
7. Complementary therapies: Some individuals find relief from acupuncture, dietary modifications, herbal supplements, and mind-body approaches like meditation or yoga.

Challenges and future directions:

Interstitial cystitis poses significant challenges due to its poorly understood nature and the variability of symptoms among individuals. The lack of a definitive diagnostic test and the absence of a universally effective treatment add to the complexity of managing the condition. Research continues to unravel the underlying mechanisms of IC, aiming to develop targeted therapies that address its root causes.

In recent years, a better understanding of the role of the bladder's microbiome, immune system, and neural pathways has opened doors to potential new treatment avenues. Personalised medicine approaches, guided by a patient's specific symptom profile and biological markers, hold promise for tailoring treatments that are more effective and precise.

Conclusion:

Interstitial cystitis is a chronic condition that significantly impacts the lives of those affected. While its exact cause remains unclear, advances in research have shed light on its underlying mechanisms and led to a range of treatment options aimed at improving the quality of life for patients. A multidisciplinary approach, involving urologists, pain specialists, physical therapists, and other healthcare professionals, is essential to providing comprehensive care for individuals with interstitial cystitis.

References available on request.