Severe Interstitial Cystitis Associated with Sjoegren's Syndrome

Abstract

A 53-year-old woman presented with oliguria, urinary frequency, abdominal pain and severe edema of the lower extremities. Her serum creatinine was 8.1 mg/dl. Computed tomographic and ultrasonographic studies showed a severely dilated urinary bladder, and bilateral hydroureteronephrosis. Examination of a urinary bladder biopsy specimen showed subepithelial edema and infiltration by lymphocytes and plasmacytes. However, the patient complainted of dry mouth and dry eyes. Ophthalmologically, the Schirmer test was positive. A biopsy of the minor salivary glands in the lip showed chronic sialoadenitis. A diagnosis of Sjögren's syndrome complicated by interstitial cystitis was made. Since she had been anuric, secondary to urinary obstruction, intermittent self-catheterization was started. Combination of corticosteroid and cyclosporin therapy was initiated. Spontaneous urination began, and gradually the patient's symptoms remitted. After 8 months of therapy, bladder capacity increased from 140 ml to 350 ml, and she voided approximately 1,200 ml by herself and 600 ml by catheterization daily. This case suggests that when severe interstitial cystitis is associated with Sjögren's syndrome, a therapeutic trial of corticosteroids and cyclosporin may be beneficial. (Internal Medicine 43: 248-252, 2004)

Interstitial cystitis: a rare manifestation of primary Sjögren’s syndrome, successfully treated with low dose cyclosporine

“…During the course of pSS, interstitial cystitis may develop, albeit rarely [2, 3]. In 1993, Van de Merwe et al [3] studied 10 patients with IC, 2 of whom were diagnosed as having pSS.…”

“…Although traditional disease-modifying agents including hydroxychloroquine and AZA are frequently used in the treatment of extraglandular involvement of pSS, data regarding their efficacy in general and especially in suppressing the symptoms of IC are not available. However, CSA in combination with corticosteroids has been found to be more effective, even in cases of dryness associated with pSS [2]. So, it may be worthwhile to consider CSA treatment in patients with pSS with extraglandular organ involvement.…”

“…Supporting this view, CSA was favorably used in the treatment of patients with chronic idiopathic IC, as reported in more recent studies. Shibata et al [2] reported a case with pSS, who presented with bilateral hydroureteronephrosis, acute renal failure and IC, and successfully treated with CSA and steroids. CSA was given at a dose 100 mg daily in that case.…”

“…During the progression of pSS, nearly 40% of the pSS patients also develop extraglandular manifestations including pulmonary, hepatic, renal involvement. Other than these extraglandular system/organ involvements, chronic interstitial cystitis (IC) may also occur, albeit rarely, in the course of the pSS, as recently observed in several patients [2–4]. …”

A case of interstitial cystitis accompanying Sj�gren?s syndrome

“…7 To the best of our knowledge, only three reports on interstitial cystitis accompanying Sjögren's syndrome have been published in Japan over the last 10 years. [11][12][13] In this report, we present a case of interstitial cystitis accompanying Sjögren's syndrome.…”