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Book Review

Interstitial Cystitis

N Engl J Med 1998; 339:1253October 22, 1998

Article

Interstitial Cystitis
Edited by Grannum R. Sant. 284 pp. Philadelphia, Lippincott–Raven, 1997. $95. ISBN: 0-397-51695-9

For over a hundred years, interstitial cystitis has remained a multifaceted and enigmatic syndrome characterized by variable motor and sensory dysfunction of the bladder. The diagnosis is usually based on symptoms, a urologic evaluation including cystoscopy, and the exclusion of other recognizable bladder diseases. The most common symptoms are urinary frequency and urgency and suprapubic, pelvic, or perineal pain. The initial description of interstitial cystitis by Hunner was of a solitary ulceration of the bladder, but this presentation is rare; the majority of affected patients have bladders with strawberry-like hemorrhages on distention that are commonly referred to as glomerulations.

Because of the high incidence of interstitial cystitis in the United States, which has been estimated to be 2.6 per 100,000, and the substantial social and economic costs of the disease, the National Institute of Diabetes and Digestive and Kidney Diseases initiated the Interstitial Cystitis Database Study in early 1991 to advance the knowledge of the disease and to define its history and treatment more clearly. By 1995, 377 patients were enrolled in the study. They were predominantly female (91 percent) and middle-aged (50 percent were 35 to 54 years of age).

In Interstitial Cystitis, Sant and his coauthors present a thorough and multidisciplinary review of current research and of the diagnostic and treatment options that are available. The causes of interstitial cystitis are unknown but may include bacteria, viruses, toxic substances in the urine, physical trauma to the bladder wall, and bladder-specific autoimmune disease. By definition, patients with interstitial cystitis cannot have symptoms related to common bacterial pathogens. The possibility of occult infection has been evaluated by Domingue, who used the polymerase chain reaction to identify bacterial DNA sequences in bladder-biopsy specimens from affected patients. Although his initial studies identified bacterial DNA in over one third of the patients, subsequent investigators have failed to detect viral or bacterial DNA, suggesting that the initial studies may have identified contaminants.

Another hypothesis is that the relatively impermeable urothelium lining (the glycosaminoglycans layer) is impaired as either a cause or a result of interstitial cystitis and that leakage of urine aggravates the underlying neuromuscular layer. Efforts to replace the glycosaminoglycans layer with heparin-like substitutes appear to have promise. The possibility that an initial inflammatory event is reinforced by neurogenic mechanisms, resulting in changes in neuroprocessing, pain pathways, and perturbations in the function of the peripheral nervous system that outlast the initial event, is intriguing and nicely ties together several aspects of the aforementioned hypotheses. Other hypotheses implicating mast cells and mucosal immunity are also intriguing.

The authors correctly emphasize that in the assessment of patients with potential interstitial cystitis, it is helpful to keep in mind that this is a symptom complex and that there are no pathognomonic findings associated with the disease. The onset of symptoms is usually acute, and symptoms may be present for several years before the patient is evaluated. Periods of exacerbation are followed by spontaneous remissions. The results of abdominal, pelvic, and neurologic examinations are nonspecific and often unremarkable. Other possible causes of the patient's symptoms, including infection and neurologic disorders, must be ruled out. Only cystoscopy with the patient under anesthesia can sufficiently distend the bladder to afford visualization of glomerulations and Hunner's ulcers. Biopsies and cytologic analysis are nonspecific but imperative to rule out carcinoma in situ.

The final chapters of the book provide practice guidelines and measurements of the health-related quality of life that will help clinicians determine which patients will require medical or surgical management. Therapy is empirical and directed at the patient's symptoms. Amitriptyline, one of the tricyclic antidepressants, has become widely used as an oral therapy for interstitial cystitis. Hydrodilation of the bladder and intravesical instillations of oxychlorosene sodium or dimethyl sulfoxide appear to be effective. Procedures to manipulate the innervation of the bladder, augmentation of the bladder, and ultimately, cystectomy should be reserved for intractable cases.

This book is an excellent resource for medical providers who are confronted with the challenging task of identifying, evaluating, and caring for patients with interstitial cystitis.

Anthony J. Schaeffer, M.D.
Northwestern University Medical School, Chicago, IL 60611-3008

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