Systemic lupus erythematosus with obstructive uropathy: Case report and review

Abstract

We report a case of patient with documented SLE who displayed dysuria, gastrointestinal (GI) symptoms and renal insufficiency associated with the unusual occurrence of bilateral hydroureteronephrosis due to urterovesical junction stricture (obstructive uropathy). Pathologic investigations disclosed chronic interstitial cystitis (IC) with evidence of focal immune complex deposition in the blood vessel walls of the bladder. The GI symptoms and dysuria regressed with initial therapy for SLE with steroids. However, the persistent obstructive uropathy (OU) and renal insufficiency required bilateral nephrostomy followed by steroids plus intravenous pulse injection of cyclophosphamide. The obstructive uropathy was relieved even after removing the nephrostomy tube and renal function remained stable. Including this case, nineteen SLE patients associated with clinical and radiographic findings of OU were found in the world literature and reviewed to find any consistent pattern of clinical features. Most of the patients with OU in SLE were female (mean age, 31.7 yr) and orientals (63%), and had interstitial cystitis (89%) as a common underlying cause with concomitant involvement of the GI tract (89%) and WHO class IV or V advanced glomerulonephritis (67%). Despite the remarkable response (68%) to steroids in majority of OU patients associated with SLE, certain patients still required surgical correction (32%) and some even died (32%). OU, potentially reversible, was not an exception in patients with SLE, which might be overshadowed by other major organ involvement of SLE.

Recognizing Concomitant Lupus Enteritis and Lupus Cystitis

“…13 Typical symptoms are suprapubic discomfort, dysuria, urgency, frequency, and nocturia. 6,14 The urine is sterile, ultrasound or CT scan demonstrate a markedly reduced bladder size with thickened bladder mucosa, and cystoscopy additionally may show stellate scars and ulcers. Lupus interstitial cystitis is frequently associated with an obstructive uropathy (in 17 of 21 in 1 review, 6 ) especially hydroureteronephrosis resulting from edema or fibrosis at the ureterovesical junction.…”

“…15,16 Nephrostomy tubes or other surgical intervention were required in a third of 1 series of 19 patients. 6 A literature review demonstrates that once lupus enteritis is found, there is a high likelihood of also demonstrating lupus cystitis, and vice versa. GI symptoms including vomiting, diarrhea, abdominal pain, ileus, malabsorption, colon ulcers, or ascites were described in 17 of 19 lupus cystitis patients in 1 review 6 and in 10 of 12 lupus patients with obstructive uropathy in 2 other case series.…”

“…The differential diagnosis of interstitial cystitis and obstructive uropathy in SLE includes bacterial and mycobacterial infection, transverse myelitis, inflammatory polyneuropathy, drug side effects, and malignancy. 6 Therapy is with corticosteroids, and with incomplete responses, CYC, azathioprine, or cyclosporine-A. 15,16 Nephrostomy tubes or other surgical intervention were required in a third of 1 series of 19 patients.…”

“…SLE in the genitourinary tract has traditionally been associated with glomerulonephritis, but lower urinary tract involvement like obstructive uropathy and interstitial cystitis have been reported rarely as well. 6 Here, we present a lupus patient who demonstrates that lupus enteritis and lupus cystitis frequently occur concomitantly, with bowel wall and bladder thickening and bilateral hydronephrosis.…”

Involvement of Lupus Enteritis in a Patient with Lupus Cystitis and Nephritis

“…The frequency is reported to occur in 0.5–1% of patients with SLE. Typical symptoms are suprapubic discomfort, dysuria, urgency, frequency, and nocturia [12]. The urine is sterile, ultrasound and CT scan show a markedly reduced bladder size with thickened bladder mucosa.…”