Franz Volhard Clinic and Departments of Urology, Pathology and Medicine, Klinikum Buch, Berlin, Germany
Case
A 64-year-old woman presented with dysuria. She was treated with various antibiotics for 8 months without relief. Three months prior to admission, she was prescribed a cream for vaginal mycosis which did not help. She claimed that she had lower abdominal pain above the pubic region, which was relieved by voiding. She also explained that she spent much of each night on the toilet and that urinating 20 times per night was nothing at all unusual. She had type 2 diabetes mellitus which required insulin for the past year. On examination, with the exception of suprapubic tenderness, nothing remarkable was found. Several urine cultures had been obtained, which were either confusing or non-revealing, perhaps due to the fact that the patient was invariably ingesting an antibiotic prescribed by one of numerous physicians.
The patient had a normal haemoglobin and haematocrit. Her blood leucocyte count was normal, although her C reactive protein level was elevated at 45 mg/l. The creatinine concentration was 238 µmol/l. Other serum chemistries were unremarkable, including autoantibodies. Her urinalysis revealed many leucocytes and erythrocytes per high power field. No casts or dysmorphic erythrocytes were seen, although a few bacteria were evident. She had a urine protein concentration of 0.75 g/l. A urine culture revealed Torulopsis glabrata. Cultures for tuberculosis were negative. Ultrasound examination revealed that the kidneys were reduced in size. The bladder was small with a thickened wall. A computerized tomographic (CT) scan of the bladder is shown in Figure 1. A diagnostic procedure was performed.
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Question
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The patient underwent cystoscopy with biopsy. Figure 2 shows a haematoxylin- and eosin-stained section of the bladder wall. The detrusor musculature shows evidence of some fibrosis. In the second panel, stained for chloracetate-ASD-esterase, a marked mast cell infiltrate can be appreciated (mast cell number 41/mm2). These findings are typical of interstitial cystitis. The patient refused any additional treatment and demanded discharge. Her request was granted and she was prescribed tramadol and itraconazole.
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The aetiology is unknown. Autoimmune, allergic, neurological, infectious, vascular and mechanical mechanisms, as well as a defective gycoprotein/ glycosaminoglycan barrier, have been proposed. The presence of mast cells, eosinophils and T lymphocytes within the bladder wall suggests involvement of the immune system.
Moreover, the association with other diseases including thyroid disorders, lupus erythaematosus, Sjögren's syndrome and inflammatory bowel disease also suggests an autoimmune pathogenesis [1]. Patients with interstitial cystitis have a 100-fold increased risk of developing inflammatory bowel disease and a 30-fold incresased risk of association with lupus erythematosus and Sjögren's syndrome [3,4]. Accordingly, laboratory findings often reveal reduced complement C4 and increased IgG levels, as well as ANA antibody titres >1:40, often including SS-A/Ro and SS-B/La [5]. Tryptase, a mast cell enzyme, is frequently elevated in 24 h urine samples. In patients with Sjögren's syndrome and distal renal tubular acidosis, the increased potassium content of the urine may lead to an increased influx into the bladder wall, causing sensory symptoms and tissue damage [6]. Interstitial cystitis may also be a local expression of a primary systemic disease.
As the aetiology and pathogenesis are unclear, there is no specific efficient treatment available for interstitial cystitis [7]. Bladder training with application of spasmolytic agents into the bladder may improve symptoms and inhibit further bladder shrinkage [8].
Notes
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References