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| Year : 2010 | Volume
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| Issue : 5 | Page : 943-946 |
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| Lupus cystitis in an Omani girl |
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Amar Al-Shibli1, Suhail Al-Salam2, Bassam Bernieh3, Hilal Matta4, Ibrahim Al Attrach1
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 Publication | 31-Aug-2010 |
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 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 2021 Mar 1];21:943-6. Available from: https://www.sjkdt.org/text.asp?2010/21/5/943/68898 |
| Introduction | |  |
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 | | |
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].
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 | | |
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.
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Correspondence Address:
Amar Al-Shibli
Departments of Pediatrics, Tawam Hospital, P.O. Box 15258, Al-Ain
Saudi Arabia
  | Check |
PMID: 20814138 
[Figure 1], [Figure 2], [Figure 3], [Figure 4] |
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