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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 21  |  Issue : 5  |  Page : 943-946
Lupus cystitis in an Omani girl


1 Department of Pediatrics, Tawam Hospital, Al Ain, Saudi Arabia
2 Department of Pathology, Faculty of Medicine and Health Sciences, Al Ain, Saudi Arabia
3 Department of Nephrology, Tawam Hospital, Al Ain, Saudi Arabia
4 Department of Surgery, Tawam Hospital, Al Ain, Saudi Arabia

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Date of Web Publication31-Aug-2010
 

   Abstract 

Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by multiple organs involvement. Bladder involvement (Lupus cystitis) is a rare manifestation of SLE, and occurs in association with gastrointestinal manifestations. We report a case of lupus inters­titial cystitis with bladder irritation and bilateral hydroureteronephrosis in an adolescent female who was treated with intravenous methylprednisolone pulse therapy followed by oral pred­nisolone and mycofenolate mofetil (MMF). Her symptoms ameliorated, and the hydrouretero­nephrosis improved. She was presented again with systemic flare up of the disease together with hydrouretronephrosis, but without bladder irritation symptoms. The diagnosis of lupus cystitis was confirmed by radiographic abnormalities, cystoscopy and bladder biopsy.

How to cite this article:
Al-Shibli A, Al-Salam S, Bernieh B, Matta H, Al Attrach I. Lupus cystitis in an Omani girl. Saudi J Kidney Dis Transpl 2010;21:943-6

How to cite this URL:
Al-Shibli A, Al-Salam S, Bernieh B, Matta H, Al Attrach I. Lupus cystitis in an Omani girl. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2019 Oct 20];21:943-6. Available from: http://www.sjkdt.org/text.asp?2010/21/5/943/68898

   Introduction Top


Lupus cystitis is a rare manifestation of sys­temic lupus erythematosus (SLE) mainly re­ported in the Asian literature and in Asian pa­tients living in western counties. [1],[2] Clinical cha­racteristic includes bladder irritation symptoms, hydronephrosis, with or without gastrointesti­nal symptoms and abnormal urinalysis. [2],[3],[4] Early recognition and treatment of this condition is very important to prevent obstructive uro­pathy, which might result in renal failure. [3],[5],[6] Cystitis with collagen vascular diseases was first reported by Shipton in 1965. [4] Since that time there were more than 40 reports of this condition in both adults [7],[8],[9],[10],[11],[12],[13],[14] and adolescents. [15],[16],[17]


   Case Report Top


A 14-year-old Omani female patient was diagnosed as a case of systemic lupus erythe­matosus (SLE) in June, 2008 based on initial presentation of fever, weight loss, mouth ulcer, arthritis, photosensitivity, hair loss, positive antinuclear antibody titer, anti dsDNA 39 IU/ mL (normal < 30), hypocomplimentemia, and nephritis (class IIa World Health Organization morphological classification of lupus nephritis).

She was readmitted to hospital after four months with gastrointestinal symptoms, sero­sitis including pleural effusion and ascitis, and bladder irritation symptoms including supra­pubic pain, dysuria, frequency, urgency, and he­maturia. At the time of admission her laboratory investigations revealed erythrocyte sedimen­tation rate 50 mm/h, leukocytes 3,900/mm 3 with lymphopenia 311/mm 3 , anemia with hemoglo­bin 10 g/dL, blood urea nitrogen 1.5 mmol/L, serum creatinine 32 μmol/L, total protein 53 g/L, albumin 29 g/L, and IgG 5.2 g/L. Uri­nalysis showed a specific gravity of 1.015, protein 1(+), 1-5 erythrocytes per high-power field (HPF), 1-4 leukocytes per HPF and a sterile culture. An abdominal ultrasonography showed bilateral hydroureteronephrosis [Figure 1]a, b.
Figure 1 :(a) US of left kidney shows dilation of the kidney and ureter.
Figure 1b. US shows dilated right ureter.


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A computerized Tomography (CT) scan de­monstrated thickened urinary bladder wall [Figure 2]a with double layering and evidence of bilateral hydrouretronephrosis [Figure 2]b.
Figure 2 :(a) CT scan of bladder shows thickened wall.
Figure 2b. CT scan shows bilateral hydronephrosis.


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A micturation cystourethrogram (MCUG) was normal with no evidence of reflux. The patient was diagnosed as lupus cystitis and treated with methyl prednisolone pulse, which was given for three days followed by oral prednisolone and mycofenolate mofetil (MMF). Subsequently, the patient's symptoms disappeared and repea­ted abdominal ultrasonography disclosed no abnormalities in the kidneys [Figure 3].
Figure 3 :Shows normalization of hydronephrosis after treatment.

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In September 2009, the patient presented with gastrointestinal symptoms including dia­rrhea, abdominal pain, and vomiting, without urinary symptoms, necessitating hospital ad­mission. Her abdomen US revealed presence of bilateral hydrouretronephrosis. Cystoscopy showed congested, hyperemic mucosa with normal position and shape of ureteral orifices. A bladder biopsy showed a picture of inters­titial cystitis with diffuse edema and mixed in­flammatory cells infiltration of the lamina propia consisted mainly of lymphocytes [Figure 4].
Figure 4 :Interstitial cystitis with edema and mixed inflammatory cells infiltration of the lamina properia consisted mainly of lymphocytes, H & E stain.

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   Discussion Top


SLE cystitis is characterized by bladder irri­tation symptoms such as suprapubic pain, ur­gency, frequency and nocturia, reduction of urinary bladder volume and hydroureteroneph­rosis with or without abnormal urine sediment and sterile urine. [18]

Gastrointestinal manifestations such as abdo­minal pain, nausea, vomiting and diarrhea are noted in almost all patients with lupus cys­titis. [2],[5],[6] Moreover, intestinal pseudo-obstruc­tion, bowel perforation, malabsorption, paraly­tic ileus with uretrohydronephrosis were des­cribed as well. [6],[15],[19] Common auto antigens,which present in both bladder and gastrointes­tinal wall, play an important role in patients with lupus cystitis. [19],[20],[21]

Our patient had cystitis during the initial presentation of SLE, which improved and re­occurred with a flare-up of her disease activity. The diagnosis of lupus cystitis is based on ultrasonography, CT scan and histology of the bladder biopsy, which showed typical inters­titial cystitis with diffuse edema, mixed infla­mmatory cell infiltration, and absence of vas­culitis [Figure 4].

Subclinical interstitial cystitis is not rare in SLE patients. [18] Therefore, when a patient with SLE complains of gastrointestinal symptoms, even in the absence of bladder irritation symp­toms, lupus cystitis should be suspected. Lupus cystitis responds well to treatment with cortico­steroid pulse treatment and other immunosup­pressive agents such as cyclosporine and cyclo­phosphamide. [21]

The estimated prevalence of this entity is from 0.5-1% of SLE patients. [23] Early diagnosis and treatment may prevent progressive bladder fibrosis, which may result in irreversible hy­dronephrosis and renal failure. [6],[20]

The pathogenesis of lupus cystitis is not clear yet, and it is related to immune complex­ mediated vasculitis [15],[22],[23] and smooth muscle dysmotility due to vasculitis and/or common autoantibodies against the smooth muscle of ureters and intestine. [22] Segawa et al [21] 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 (up to 92% of the cases) with hydroure­teronephrosis, [24] which is usually due to distal uretral obstruction at the uretrovesical junction because of inflammation and edema of the bladder and to detrusor muscle spasm second­dary to inflammation. [3] Some of the patients will not present with bladder irritation symp­toms, especially if they present early. This is attributed to mild interstitial bladder inflammation, which is reflected clinically by the absence of urinary sediment.

We conclude that lupus cystitis is an unco­mmon manifestation of SLE mainly reported in Far East Asians. To the best of our know­ledge this is the first case reported from the Middle East. Lupus cystitis should be suspec­ted, regardless of the ethnic origin, in any patient with SLE showing vague gastrointes­tinal symptoms, with or without bladder irri­tation symptoms and hydrouretronephrosis. Early diagnosis and treatment are essential to prevent renal damage.

 
   References Top

1.Chen MY, Lee KL, Hsu PN, Wu CS, Wu CH. Is there an ethnic difference in the prevalence of lupus cystitis? A report of six cases. Lupus 2004; 13:263-9.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Kinoshita K, Kishimoto K, Shimazu H, et al. Two cases of lupus cystitis with no bladder irritation symptoms. J Intern Med 2008;265:1477-9.  Back to cited text no. 2      
3.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;98:323-6.  Back to cited text no. 3  [PUBMED]    
4.Shipton EA. Hunner's ulcer (chronic interstitial cystitis): a manifestation of collagen disease. Br J Urol 1965;37:443-8.  Back to cited text no. 4  [PUBMED]    
5.Moriuchi J, Ichikawa Y, Takaya M, et al. Lupus cystitis and perforation of the small bowel in a patient with systemic lupus erythematosus and overlapping syndrome. Clin Exp Rheumatol 1989;7:533-6.  Back to cited text no. 5  [PUBMED]    
6.Eberhard A, Shore A, Silverman E, Laxer R. Bowel perforation and interstitial cystitis in child­hood systemic lupus erythematosus. J Rheumatol 1991;18:746-7.  Back to cited text no. 6  [PUBMED]    
7.Weisman MH, McDanald EC, Wilson CB. Studies of the pathogenesis of interstitial cys-titis, obstructive uropathy, and intestinal mal-bsorption in a patient with systemic lupus ery-thematosus. Am J Med 1981;70:875-81.  Back to cited text no. 7  [PUBMED]    
8.Meulders Q, Michel C, Marteau P, et al. Associa­tion of chronic interstitial cystitis, protein-losing enteropathy and paralytic ileus with seronegative Systemic lupus erythematosus: case report and review of the literature. Clin Nephrol 1992;37: 239-44.  Back to cited text no. 8  [PUBMED]    
9.De la Serna AR, Alarcon-Segovia D. Chronic in­terstitial cystitis as an initial major manifestation of systemic lupus erythematosus. J Rheumatol 1981;8:808-10.  Back to cited text no. 9  [PUBMED]    
10.De Arriba G, Velo M, Barrio F, Garcia-Martin F, Hernando L. Association of intestinal lupus cys­titis with systemic lupus erythematosus. Clin Nephrol 1993;39:287-8.  Back to cited text no. 10      
11.Koike T, Takabayashi K. Lupus cystitis in the Japanese. Intern Med 1996;35:87-8.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]  
12.Hawwada M, Hashimoto K. Systemic lupus erythe­matosus relapse with lupus cystitis. Clin Exp Rheumatol 1995;13:645-8.  Back to cited text no. 12      
13.Odeh M. Severe lupus cystitis with obstructive uropathy. Nephron 1996;74:493-4.  Back to cited text no. 13  [PUBMED]    
14.Kim HJ, Park MH. Obstructive uropathy due to interstitial cystitis in a patient with systemic lupus erythematosus. Clin Nephrol 1996;74:205-8.  Back to cited text no. 14      
15.Weisman MH, McDanald EC, Wilson CB. Studies of the pathogenesis of interstitial cystitis, obs­tructive uropathy, and intestinal malabsorption in a patient with systemic lupus erythematosus. Am J Med 1981;70:875.  Back to cited text no. 15  [PUBMED]    
16.do Socorro Teixeira Moreira Almeida M, Carvalho LL, Carvalho AG, Almeida JV, Borges e Silva J. Interstitial cystitis and systemic lupus erythe­matosus in a 20-year-old woman, Rheumatol Int 2008;29:219-21.  Back to cited text no. 16      
17.Cochat P, Glastre C, Raveau J, Parchoux B, Larbre F. Cystitis: a rare feature in systemic lupus erythematosus. Pediatr Nephrol 1992;6:C124.  Back to cited text no. 17      
18.Alarcon-Segovia D, Abud-Mendoza C, Reyes­Gutierrez E, Iglesias-Gamarra A, Diaz-Jouanen E. Involvement of the urinary bladder in sys­temic lupus erythematosus. A pathologic study. J Rheumatol 1984;11:208-10.  Back to cited text no. 18      
19.Mok MY, Wong RW, Lau CS. Intestinal pseudo­obstruction in systemic lupus erythematosus: an uncommon but important clinical manifestation. Lupus 2000;9:11-8.  Back to cited text no. 19  [PUBMED]  [FULLTEXT]  
20.Nakauchi Y, Suehiro T, Tahara K, et al. Sys­temic lupus erythematosus relapse with lupus cystitis. Clin Exp Rheumatol 1995;13:645-8.  Back to cited text no. 20  [PUBMED]    
21.Segawa C, Wada T, Furuichi K, Takasawa K, Yokoyama H, Kobayashi K. Steroid pulse the­rapy in lupus cystitis. Intern Med 1996; 35:155-8.  Back to cited text no. 21  [PUBMED]  [FULLTEXT]  
22.Boye E, Morse M, Huttner I, Erlanger BF, MacKinnon KJ, Klassen J. Immune complex­mediated interstitial cystitis as a major manifes­tation of systemic lupus erythematosus. Clin Immunol Immunopathol 1979;13:67-79.  Back to cited text no. 22  [PUBMED]    
23.Georgin-Lavialle S, Izzedine H, Launay-Vacher V, et al. Why is a shrunken bladder and a neph­ritic kidney an expression of the same disease? Nephrol Dial Transplant 2004;19(12):3193-5.  Back to cited text no. 23      
24.Shimuzo A, Tamura A, Tago O, et al. Lupus cystitis: a case report and review of the literature. Lupus 2009;18(7):655-8.  Back to cited text no. 24      

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Correspondence Address:
Amar Al-Shibli
Departments of Pediatrics, Tawam Hospital, P.O. Box 15258, Al-Ain
Saudi Arabia
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PMID: 20814138

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