Nephrology Dialysis Transplantation 2004 19(12):3193-3195; doi:10.1093/ndt/gfh389
Nephrol Dial Transplant Vol. 19 No. 12 © ERA-EDTA 2004; all rights reserved
Nephroquiz
(Section Editor: M. G. Zeier)
Why is a shrunken bladder and a nephrotic kidney an expression of the same disease?
Sophie Georgin-Lavialle1,
Hassane Izzedine1,
Vincent Launay-Vacher1,
Benoît Barrou2,
Thierry Petitclerc1 and
Gilbert Deray1
1 Department of Nephrology,2
Department of Urology,
Pitié Salpétrière Hospital,
Paris, France,
Email: hassan.izzedine{at}psl.ap-hop-paris.fr
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Case
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A 29-year-old Chinese woman presented with a 4 week history
of progressive urinary frequency, dysuria and leg edema. She
was forced to urinate every 10 min, resulting in great irritability
and sleeplessness. Furthermore, she felt mild abdominal fullness
and dyspnea. She was therefore referred to our department for
evaluation. Her abdomen was bulging without tenderness or bowel
sounds arrest. There was no erythema, joint swelling or Raynaud's
phenomenon. On admission, her urinalysis showed heavy proteinuria.
She was found to have nephritic syndrome (proteinuria 4.5 g/24
h, serum albumin level 2.8 g/dl) and active urinary sediment
(6–8 red cells/high power field, but no leukocytes or
casts). Repeated urinary cultures showed no bacteria. Renal
function was altered: serum creatinine was 3.9 mg/dl and creatinine
clearance rate was 20 ml/min. There was no anaemia, but blood
lymphocyte count had decreased to 900/ml. No fluid levels were
seen on plain abdominal X-rays. Ultrasonogram and abdominal
scan CT showed severe bilateral hydroureteronephrosis and hydroureter
(
Figure 1a). Cystoscopy revealed a bladder capacity of 25 ml
with a contracted thickened bladder wall, diffuse hyperaemia,
edema and friability of mucosa without visualization of both
ureteral orifices. Voiding cystourethrogram showed a small and
contracted bladder with thickened and irregular walls without
diverticula, and no vesicoureteral reflux was demonstrated (
Figure 2).
Biopsy of the bladder wall revealed changes of interstitial
cystitis disclosing diffuse edema of the submucosal tissue,
and no active vasculitis. At that time an antegrade pyelography
confirmed bilateral hydroureteronephrosis. We immediately inserted
a bilateral ureteral JJ tube. However, because renal function
was stable 3 weeks later, we removed the JJ tubes and no further
surgical procedure was performed.
Although her antinuclear antibodies were positive, there was
no other finding suggestive of active systemic lupus erythematous
(SLE). Renal biopsy specimens obtained 1 week after admission
showed mild mesangial proliferative glomerulonephritis (
Figure 3).
Urodynamic studies that were performed 3 weeks after hospitalization
were normal.
The patient was treated with 1 mg/Kg/day of prednisone, and
her lower urinary tract symptoms markedly improved. Serum creatinine
decreased to 0.7 mg/dl within 2 months with a marked improvement
in the bilateral hydroureteronephrosis (
Figure 1b), and gastrointestinal
symptoms completely abated. Prednisone was tapered and the patient
was maintained on prednisone at 12.5 mg/day. Her urinary protein
excretion decreased markedly as she recovered from her contracted
bladder condition.
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Question
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What is your diagnosis?
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Answers to the quiz on the preceding page
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Pathological diagnosis: SLE with lupus cystitis associated ureterohydronephrosis and class 2, WHO classification lupus nephritis
The association of urinary frequency with ureterohydronephrosis
+ mild abdominal fullness and mesangial proliferative glomerulonephritis
in an Asian young woman led to two major diagnoses a lupus cystitis
and/or chronic intestinal pseudo-obstruction (CIPO).
Interstitial cystitis, which itself is an uncommon lesion of the urinary bladder, was first described by Nitze [1] in 1907. The first case of interstitial cystitis with SLE, in which the urinary bladder contained deposits of IgG, IgM, IgA, and complement was called lupus cystitis by Orth et al. [2], and this nomenclature has been widely accepted. In SLE patients, interstitial cystitis was found in 11 out of 35 autopsies, and is estimated to occur in 0.5–1% of all SLE cases. Lupus cystitis may occur as an initial and/or isolated manifestation of SLE as in our patient. Chronic interstitial cystitis (CIC) occurs mainly in middle-aged women who present with frequency and urgency. Of 34 patients with CIC, 25 were ANA positive among whom seven had SLE [3]. The manifestations of lupus cystitis in adults and children are similar and include vomiting, weight loss, frequency and urgency. Urinalysis reveals either no abnormality or only microscopic haematuria and is characterized by a negative cytology. The precise mechanisms involved in lupus cystitis remain obscure. Bladder biopsies of lupus cystitis show granular deposits of immune complex (IgG, IgA, IgM and C3, C1q) with mononuclear and polymorphonuclear leukocytes along blood vessel walls [4]. These findings suggest that immune complex-mediated vasculitits is one of the attributable causes of lupus cystitis. Segawa et al. [5] noted the elevation of interleukin-8 and monocyte chemotactic and activating factors, such as urinary chemokines, and their resolution after treatment. These findings provide insight into the mechanism of lupus cystitis. In contrast to idiopathic cystitis, lupus cystitis is frequently associated with hydroureteronephrosis, which is usually due to fibrosis of the ureterovesical junction, and to detrusor muscle spasm secondary to inflammation [2]. Mild gastrointestinal manifestations are common in SLE. Nausea, vomiting, diarrhoea and abdominal pain are found in more than 50% of these patients. These symptoms associated with uereterohydronephrosis in our patient mimicked an intestinal pseudo-occlusion syndrome.
Chronic intestinal pseudo-obstruction is a syndrome defined by the presence of chronic intestinal dilation and dysmotility in the absence of mechanical obstruction or gross inflammatory disease. The megacystis-microcolon intestinal hypoperistalsis syndrome was first described in 1976 as a cause of intestinal obstruction in the newborn. Neonatal presentation of this syndrome includes intestinal occlusion, abdominal distension and bilious vomiting. Late manifestations include abdominal bloating constipation and bilious vomiting with an initially normal intestinal transit [6]. The incidence of urological involvement varies from 33 to 92% and includes megacystis, hydronephrosis, urinary retention and infection [7]. There is an apparent association between lupus-related CIPO and ureterohydronephrosis: 66.7% (12/18) of patients had a concomitant dilated urinary pelvicalyceal system and a contracted urinary bladder, 33.3% (6/18) of these patients had documented histological features of CIPO and 38.8% had glomerulonephritis (7/18) [8].
We believe that our patient presented with SLE with lupus cystitis and without CIPO evidence for this is the absence of megacystis and because plain abdominal X-ray films showed no gaseous distension of the small bowel and, in the upright films, no presence of fluid in the bowel loops. Treatment of SLE is with corticosteroids and immunosuppressive drugs. These are used and improve the prognosis of lupus cystitis [9] as in our patient.
Conflict of interest statement. None declared.
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References
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- Nitze M. Lehrbuch der Kystoskopie: Ihre Technik und Klinische Bedeutung. JE Bergman, Berlin 1907, p. 410
- Orth RW, Weisman MH, Cohen AH, Talner LB, Nachtsheim D, Zvaifler NJ. Lupus cystitis: primary bladder manifestations of systemic lupus erythematosus. Ann Intern Med 1983; 98: 323–326[ISI][Medline]
- Christmas T, Page SL, Maddison P, Isenberg D. Antinuclear antibodies in interstitial cystitis (abstract). Br J Rheumatol 1989; 28: 37[Medline]
- Nagata T, Umeda S, Taniguchi T, Inada M, Kokura M. A case of lupus cystitis complicated by membranous lupus nephritis. Nippon Naika Gakkai Zasshi 1990; 79: 820–821[Medline]
- Segawa C, Wada T, Furuichi K, Takasawa K, Yokoyama H, Kobayashi K. Steroid pulse therapy in lupus cystitis. Intern Med 1996; 35: 155–158[Medline]
- Goulet O, Jobert-Giraud A, Michel JL et al. Chronic intestinal pseudo-obstruction syndrome in pediatric patients. Eur J Pediatr Surg 1999; 9: 83–89[Medline]
- Faure C, Goulet O, Ategbo S et al. Chronic intestinal pseudoobstruction syndrome: clinical analysis, outcome, and prognosis in 105 children. French-Speaking Group of Pediatric Gastroenterology. Dig Dis Sci 1999; 44: 953–959[Medline]
- Segovia AD, Mendoza AC, Gutierrez RE et al. Involvement of urinary bladder in systemic lupus erythematosus. A pathologic study. J Rheumatol 1984; 11: 208–210[ISI][Medline]
- Arriba G, Velo M, Barrio V, Garcia-Martin F, Hernando L. Association of interstitial lupus cystitis with systemic lupus erythematosus. Clin Nephrol 1992; 37: 287
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