Interstitial cystitis

Interstitial cystitis, also known as bladder pain syndrome, is a chronic, non-infectious, inflammatory condition of poorly understood etiology that affects the urinary bladder. It is defined as urinary bladder pain and irritative symptoms of more than six months duration. It usually represents a diagnosis of exclusion.

Epidemiology

It tends to commonly occur in young to middle-aged women ¹. The RAND Interstitial Cystitis Epidemiology (RICE) study in 2011 surveyed women in approximately 150,000 United States households and concluded that up to 6.5% have symptoms of interstitial cystitis ⁴.

Clinical presentation

• frequent urination

• urgency with urination

• feelings of pressure, pain, and tenderness around the bladder, pelvis, and the area between the anus and vagina or anus and scrotum (perineum)

• pain during sex

• in men, discomfort or pain in the penis and scrotum

• in women, symptoms may worsen around their period

• stress may also make symptoms worse, but stress does not cause symptoms

Pathology

The etiology of interstitial cystitis is unclear although its pathogenesis appears to be multifactorial. The main concepts include:

• urothelial dysfunction, especially in the epithelial and glycosaminoglycan (GAG) layers

• mast cell dysfunction

• vascular malformations, seen as glomerulations on cystoscopy

• neurogenic inflammation/edema

• autoimmune or immune-mediated process

• increase in sensory afferent fibers by upregulation

• fibrosis

• increase in grey matter volume leading to increase sensitivity to pain ⁸

Radiographic features

MRI

• DWI: may show diffuse restriction in bladder wall ⁷

Treatment and prognosis

The treatment options are conservative, pharmacological, and surgical:

Conservative

• manual physical therapy for pelvic floor tenderness

• bladder re-training

• stress management to avoid stress-driven symptoms

• special diets that avoid acidic substances that contribute to bladder irritation such as tomatoes, spices, chocolate and citrus fruits, as well as alcohol and caffeine

• transcutaneous electrical nerve stimulation (TENS)

• cognitive-behavioral therapy

Pharmacological

The options are mainly divided into oral and intra-vesical routes:

• oral

  • analgesics, e.g.: acetaminophen and ibuprofen

  • antihistamines (cimetidine)

  • antidepressants (amitriptyline)

  • pentosan-polysulfate

  • oxybutynin and gabapentin

• intravesical instillation

  • lidocaine and sodium bicarbonate

  • pentosan-polysulfate

  • heparin

  • dimethyl sulfoxide (DMSO)

  • hyaluronic acid and chondroitin sulfate

• cystoscopy guided treatment

  • low-pressure hydrodistention for a short period

  • if Hunner lesions are present, treat using direct fulguration through diathermy or laser, and/or injection of steroid, e.g., triamcinolone

• intra-detrusor Injection (with Botulinum toxin type A)

  • the patient should be warned that there may be a need for intermittent self-catheterization in the future

• systemic immunosuppression

  • only cyclosporine is indicated for use and the patient should be informed of the risks associated with systemic immunosuppression, such as vulnerability to infection and the development of malignancies

Surgical

After the introduction of intra-detrusor injections of botulinum toxin the recourse to surgical therapy has been considerably reduced even if it remains the last option for patients with intractable pathology. The options are:

• cystoplasty only

• cystoplasty with supra-trigonal resection

• cystoplasty with sub-trigonal resection

• urinary diversion, with or without cystectomy and urethral resection - this may be in the form of a urostomy

Differential diagnosis

For clinical presenantation consider

• overactive bladder (OAB): may share similar clinical features ⁶

See also

• cystitis: any form of inflammation involving the bladder