Interstitial cystitis and ileus in pediatric-onset systemic lupus erythematosus

A girl aged 11 years presented with autoimmune hemolytic anemia with thrombocytopenia, and subsequently developed severe abdominal pain, vomiting, and pollakiuria. X-ray findings of her abdomen demonstrated paralytic ileus with intestinal wall thickening. Intravenous pyelography revealed bilateral hydroureter with mild hydronephrosis and contracted bladder. Pathological examination of her bladder revealed interstitial cystitis, with evidence of focal deposition of IgG and C3 in a granular pattern on small blood vessel walls. She was diagnosed as having systemic lupus erythematosus (SLE) associated with paralytic ileus and chronic interstitial cystitis. Although initiation of high-dose prednisolone therapy resulted in a gradual improvement in clinical symptoms, reducing the dosage of prednisolone caused a relapse. To our knowledge, the combination of paralytic ileus and chronic interstitial cystitis is quite uncommon in pediatric-onset SLE. Received: 1 September 1999 / Revised: 2 December 1999 / Accepted: 3 December 1999     

Interstitial cystitis in a woman with systemic mastocytosis

Studies have reported detrusor mastocytosis in patients with interstitial cystitis. The author describes a patient with systemic mastocytosis who was confirmed to have detrusor mastocytosis and interstitial cystitis. She responded to therapy with pentosanpolysulfate. The literature on systemic mastocytosis and the role of mast cells in the pathophysiology of interstitial cystitis are reviewed.     

Interstitial cystitis

Interstitial cystitis (IC) is a multifactorial syndrome with symptoms of pelvic or perineal pain, urinary frequency and urgency. The etiologies are unknown, but several theories have been proposed. Diagnosis is often delayed because most of the conventional evaluation is normal. Pelvic examination is normal except for bladder tenderness. Urodynamics are normal except for increased bladder sensitivity and low capacity. Urinalysis, urine culture and office cystoscopy are also normal. The diagnostic test is cystoscopy under anesthesia with bladder distension. Small submucosal hemorrhages (glomerulations) or ulcers appear after distension. Many empiric treatments have been proposed for IC. None is universally effective, and so treatments are tried sequentially until good symptom relief is achieved. Bladder distension gives excellent (but transient) relief in some patients, especially those with severe bladder inflammation (who also tend to be older). A variety of oral, intravesical and adjunctive treatments are also described.     

Mucormycosis in systemic autoimmune diseases

Mucormycosis is an emerging infection in systemic autoimmune diseases. All published cases of systemic autoimmune diseases complicated by mucormycosis were reviewed. The clinical features, diagnostic procedures and the main principles of treatment were analyzed. Twenty-four cases of mucormycosis have been reported in systemic auto-immune diseases, of which 83% in systemic lupus erythematosus, all occurring during immunosuppressants. In most cases, the infection was disseminated or rhinocerebral and it had mimicked a flare of the underlying connective tissue disease. A fatal outcome was reported in 58.3% of these patients. In conclusion, mucormycosis often mimics a flare of the underlying systemic disease and is associated with a high mortality rate. Systemic lupus erythematosus is by far the most common associated systemic autoimmune disease. A high degree of awareness is warranted to rapidly rule out infection, of which mucormycosis, in immunocompromised patients with systemic autoimmune disease before a disease flare is conclusively diagnosed.     

Interstitial cystitis.

Twenty histologically proven cases of interstitial cystitis were investigated in an attempt to find evidence of a possible autoimmune mechanism in its aetiology. HLA tissue typing was performed, autoantibody profiles were obtained, and a questionnaire of the patients' past history completed, especially with respect to other autoimmune conditions. An experiment was designed to test the effect of sodium cromoglycate on rat bladders. The results are discussed in relation to the management of patients with interstitial cystitis.     

Interstitial cystitis following colocystoplasty

A case of interstitial cystitis occurring in a patient following colocystoplasty for intractable symptomatology resultant from interstitial cystitis is presented. The recurrence of the inflammatory disease in the transposed colonic patch does not appear to be immunologically mediated in this patient.     

Interstitial cystitis in Japan

OBJECTIVE: To determine the prevalence of interstitial cystitis in Japan, and to analyse the diagnostic criteria and treatments most commonly used by Japanese urologists. Materials and methods Questionnaires were sent to 300 urologists at major hospitals, including medical university hospitals, throughout Japan, asking about the number of patients with interstitial cystitis, its epidemiology, diagnosis and therapy. RESULTS: The prevalence of interstitial cystitis was 1.2 per 100 000 patients and 4.5 per 100 000 female patients; the male/female ratio was 1 : 5.8. Most Japanese urologists used two or more diagnostic criteria; bladder biopsy was the most common diagnostic method. Dimethyl sulphoxide instillations and corticosteroids were the most frequently used therapies. CONCLUSION: The prevalence of interstitial cystitis in Japan is markedly lower than that reported in Europe and North America. The reasons for this difference were not only racial but also the lack of awareness among Japanese urologists and patients about interstitial cystitis.     

Interstitial cystitis in men

Interstitial cystitis is an uncommon disease reported predominantly in females. Recently we were involved in the management of 4 men who had the clinical, endoscopic and pathological features consistent with the diagnosis of interstitial cystitis. The rarity of occurrence of the disease in males prompted us to report these cases.     

Interstitial cystitis and painful bladder syndrome

PURPOSE: We quantified the burden of interstitial cystitis/painful bladder syndrome on the health care system in the United States. MATERIALS AND METHODS: The analytical methods used to generate these results were described previously. Interstitial cystitis was defined based on International Classification of Diseases, 9th revision code 595.1 (interstitial cystitis). For painful bladder syndrome we used the definition International Classification of Diseases, 9th revision code 788.41 (urinary frequency) with code 625.8 (other specified symptoms associated with female genital organs) or 625.9 (unspecified symptoms associated with female genital organs). RESULTS: Between 1992 and 2001 there was a 2-fold increase in the rate of hospital outpatient visits and a 3-fold increase in the rate of physician office visits related to interstitial cystitis. The annualized rate was 102 office visits per 100,000 population. Ambulatory surgery for interstitial cystitis decreased. A diagnosis of interstitial cystitis was associated with a 2-fold increase in direct medical costs. Between 1994 and 2000 annual national expenditures for interstitial cystitis were stable at $37 million but annual costs for painful bladder syndrome increased from $481 million to $750 million. CONCLUSIONS: Although interstitial cystitis accounts for a small percent of health care visits, its economic burden is substantial. Because of misdiagnosis, the true burden of interstitial cystitis/painful bladder syndrome on the health care system in the United States is probably underestimated in administrative data that rely only on physician coding to identify the disorder. The greatest part of the disease burden is likely not captured in this economic analysis.     

间质性膀胱炎的诊断进展

IC病因不明,发病机制复杂,其临床表现与多种疾病间有交叉重叠,又缺乏统一诊断标准,其漏诊、误诊比例极高,目前尚没有有效的诊断手段,本文就间质性膀胱炎诊断方面近几年所取得的新进展作一综述.