Lupus Cystitis Research Articles

Two complications in patients with systemic lupus erythematosus: lupus cystitis and lupus enteritis.

1. Orth RW, Weisman MH, Cohen AH, . Lupus cystitis: primary bladder manifestations of systemic lupus erythematosus. Ann Intern Med1983; 98: 323-6. Google Scholar

Lupus Cystitis, From Myth to Reality: A Narrative Review.

Systemic lupus erythematosus is a multisystemic disease that usually involves the urinary tract, often in the form of lupus nephritis. However, another form of compromise of this system is lupus cystitis, which, despite being an unusual condition, turns out to be a challenging diagnosis due to the spectrum of nonspecific abdominal and urinary symptoms. Although the exact pathophysiological mechanism of bladder inflammation remains to be established, the role of small vessel vasculitis measured by immune complexes continues to be supported as a central axis for considering possible therapeutic targets. Additionally, there are no clinical studies that dictate a guideline regarding its treatment, however, the evidence from most cases described in the literature suggests the initiation of pulses of methylprednisolone and cyclophosphamide in treatment regimens similar to those of lupus nephritis. Despite its low prevalence, obstructive complications and kidney damage can lead to increased morbidity and mortality.

MO325YOU CAN BE DIAGNOSED AND CURED FOR LUPUS CYSTITIS AND LUPUS PODOCYTOPATHY DESPITE BEING A POOR, VILLAGE TEENAGE GIRL IN ALBANIA

Abstract Background and Aims We present the case of a 16 years old girl who was admitted to our Emergency Unit in May 2019 for diarrhea, nausea, dysuria, foamy urine, urinary incontinence, malnutrition, polyserositis, and hypertension. Her medical history started 8 months ago with diarrhea and urinary incontinence for which first was hospitalized in the Gastroenterology unit and then in the Infectious Disease Unit. There she was completed with colonoscopy, contrast CT scan and then was discharged with the diagnosis of Gastrocolitis. In January 2019 due to the persistence of symptoms they did a specialized consultation in Athens, Greece. After a series of examinations the patient was diagnosed with Anorexia Nervosa and antidepressant therapy was started. In February 2019, the patient was rehospitalized with nephritic grade proteinuria and the kidney ultrasound showed stage four bilateral hydronephrosis and urinary bladder with thick and trabecular walls. To exclude urological problems, an MRI was performed which results in no obstructive problems. Arterial hypertension and lower extremities edema were present. She was then transferred to our University Hospital “Mother Teresa”, Nephrology Department for further examinations. During hospitalization her blood investigation showed severe anemia (HGB = 6.7gr / dl), kidney failure (creatinine = 1.5mg / dl, urea = 83mg / dl), elevated liver enzymes (Alt:162u/ml, Ast:101u/ml), albuminemia: 2.9 g / dl, total proteinemia: 5.9g / dl. The lipid profile showed cholesterolemia: 300mg / dl, triglyceridemia: 170mg / dl. Electrolytes were within normal limits. Coombs test resulted positive. Urinalysis showed microscopic hematuria with leukocyturia and grave albuminuria around16gr/ 24 hours. Immunologic workup showed: AntiDna = 383.5U / ml, Ena profile SSA poz, ANA ++++, C3 101, C4 18. Tumoral markers and hepatitis resulted in negativ. Method Renal biopsy was performed which resulted: Lupus Podocytopathy Results The patient was diagnosed with a case of lupus cystitis with lupus podocytopathy. She was treated with methylprednisolone, immunosuppressive therapy, and Plaquenil. It was started with intravenous methylprednisolone 0.5 g / day for 3 days and then switched to oral methylprednisolone 0.5 mg /kg /day. Mycophenolate mofetil was started with 1 gr increased to 2 grams. After 6 months of therapy Hydroureteronephrosis completely disappeared and 24 h urinary protein became normal. The dose of therapy was tapered and switch to maintenance doses, methylprednisolone 8 mg, MMF 500gr, and Plaquenil. Laboratory examinations Hgb: 12gr / dl, Urea: 36mg / dl, Creatine: 0.6mg / dl, Alt: 23u / l, Ast: 26u / l. Urinalysis: albumin trace, RBC: 0, Wbc 8 / field. The autoimmune workup was normalized, AntiDna; C3, C4, Ana. In a realized ultrasound hydronephrosis was gone, kidney structure was in normal parameters and bladder wall was in a normal structure. Conclusion Disseminated Eritematous Lupus and its rare forms like Lupus Cystitis and Lupus Podocytopathies can be diagnosed, cured successfully, and followed up in the best way despite you are a simple teenager in a village of Albania or a noticed and famous actor or singer in the USA.

Successful treatment of refractory cystitis associated with systemic lupus erythematosus with Belimumab

Lupus cystitis (LC) is a rare manifestation of SLE, the diagnosis of LC is challenging, especially in the absence of other systemic manifestations or the obvious disease activity index of SLE, further cystoscopy and bladder biopsy are crucial. The symptoms of cystitis could be controlled with high-dose GC, but in the process of GC reduction, the condition repeated. Here we report one case of refractory LC treated with Belimumab.The case is a 45-year-old female patient who had SLE and presented with urinary urgency, frequency and pain for more than 3 years. Laboratory assays revealed high ANA, reduced complement 3 level, proteinuria, significantly elevated Leukocyte esterase and leukocyte in urine, with the negative urinary culture of bacteria and mycoplasma, meanwhile, the cystoscopy and bladder biopsy showed interstitial inflammation. Confirming the diagnosis of refractory SLE-LC, recommended-dose Belimumab (10 mg/kg fortnightly for 3 times, monthly for 6 times) was administered, resulting in the normalization of urinary activity and significant reductions in leukocyte counts and protein levels of urine. No lupus cystitis relapse or SLE activity occurred during 10 months of follow-up.Our case confirmed the efficacy and good follow-up outcomes of Belimumab treatment for refractory SLE-LC.

The emergence of bladder dysfunction secondary to lupus cystitis in a patient with established lupus nephritis: the first case from Pakistan

Lupus cystitis is an uncommon manifestation of systemic lupus erythematosus (SLE). We describe the case of a 23-year-old woman with lupus that complained of abdominal pain, vomiting, and diarrhea for one week. Two years back, she was brought with an affirmative history that satisfied the clinical criteria of SLE which was renal biopsy-proven (class V membranous lupus nephritis). On radiologic workup, she was found to have bilateral hydronephrosis and a thick-walled bladder with diverticula. Immunosuppressive therapy was commenced however augmentation cystoplasty had to be opted for as the symptoms did not subside with medical therapy alone.

Risks of interstitial cystitis among patients with systemic lupus erythematosus: A population-based cohort study.

To investigate whether the risk of interstitial cystitis increases among the patients with systemic lupus erythematosus. This was a nationwide population-based cohort study. Data were obtained from the National Health Insurance Research Database in Taiwan. Women aged >18years newly diagnosed as systemic lupus erythematosus during 2001-2008 were identified as the control group. The comparison included individuals randomly selected from the National Health Insurance Research Database in the year of 2000, by matching one systemic lupus erythematosus participant with eight non-systemic lupus erythematosus participants with sex and age. These participants were followed up until being diagnosed as interstitial cystitis, or the end of 2011. Women diagnosed with lupus cystitis were excluded from this study. This study included 7240 women with systemic lupus erythematosus and 57920 women without systemic lupus erythematosus as controls. The incidence rate of interstitial cystitis was significantly higher in the systemic lupus erythematosus group, with an incidence rate ratio of 2.26 (95% confidence interval 1.57-3.27, P<0.0001). After adjustment, the risk increased by 2.45-fold (adjusted hazard ratio 2.45, 95% confidence interval 1.57-3.27, P<0.05). Age as a factor increases incidence rate ratios among all age groups, 2.12-, 3.32- and 4.65-fold. Age ≥45years had an increased adjusted hazard ratio (2.07, 95% confidence interval 1.37-3.13, P<0.05). Comorbidities, for example, hypertension, diabetes mellitus, dyslipidemia and renal disease, were insignificant. This is the first population-based cohort study showing a higher incidence of interstitial cystitis among patients with systemic lupus erythematosus. These findings support the concordance of interstitial cystitis with autoimmune diseases, and the temporal relationship to develop interstitial cystitis in patients with systemic lupus erythematosus.

Systemic lupus erythematosus of the urinary tract: focus on lupus cystitis.

Systemic lupus erythematosus (SLE) frequently manifests as urinary tract disease, most commonly in the form of lupus nephritis. Bladder involvement in the disease course takes a subclinical form and may affect both children and adults. Lupus cystitis can precede SLE diagnosis and may present with very unspecific urinary and digestive tract symptoms or no symptoms at all. The exact mechanism of bladder inflammation in lupus is not fully understood; however, histopathological studies suggest a possible role of immune complex-mediated small vessel vasculitis. Lupus cystitis is a rare SLE manifestation, but poses a challenge for physicians, due to its complex diagnostics and treatment.

Lupus cystitis presenting with lower urinary tract symptoms: A case report

Lupus cystitis presenting with lower urinary tract symptoms: A case report

Atypical Presenting Symptoms of Acute Onset Systemic Lupus Erythematosus with Enteritis and Cystitis.

Lupus enteritis and lupus cystitis are relatively rare manifestations of systemic lupus erythematosus. Some patients develop severe complications such as bowel perforation, infarction, obstruction, or irreversible bladder dysfunction. Early diagnosis is critical for management of lupus enteritis and cystitis. We report a 48-year-old Japanese man who presented with initial manifestations of abdominal pain, severe diarrhea, and bloody feces. The diagnosis was delayed due to atypical initial symptoms, resulting in clinical worsening. Physicians should be aware of typical computed tomography findings of lupus enteritis and lupus cystitis.

Lupus cystitis presenting with hidronephrosis and gastrointestinal involvement

Lupus cystitis is a rare manifestation of systemic lupus erythematosus, characterized by thickening of the bladder wall, associated with ureterohydronephrosis. In several cases gastrointestinal symptoms are the main manifestation. The optimal immunosuppressive regimen is still unknown. 16-year-old girl with systemic lupus erythematosus was admitted with dysuria, renal impairment, diarrhea, abdominal pain, nausea and vomiting. An abdominal CT scan demonstrated moderate bilateral dilation from the pelvis to the ureterovesical junction, and the bladder exhibited reduced capacity and diffusely thickened walls. A diagnosis of chronic interstitial cystitis was performed and treatment with high dose methylprednisolone resulted in a significant relief of gastrointestinal and urinary symptoms and a reduction of the bladder thickness with improvement of their capacity. Lupus cystitis is a rare and underdiagnosed urinary manifestation of LES and the presence of mild urinary symptoms and abdominal pain may alert about this possibility.

Lupus cystitis in Korean patients with systemic lupus erythematosus: risk factors and clinical outcomes.

This study was performed to investigate the clinical characteristics of lupus cystitis and determine the risk factors and clinical outcomes of lupus cystitis in patients with systemic lupus erythematosus (SLE). We retrospectively reviewed 1064 patients at Seoul St. Mary's Hospital in Seoul, Korea, from 1998 to 2013. Twenty-four patients had lupus cystitis. Lupus cystitis was defined as unexplained ureteritis and/or cystitis as detected by imaging studies, cystoscopy, or bladder histopathology without urinary microorganisms or stones. Three-fourths of patients with lupus cystitis had concurrent lupus mesenteric vasculitis (LMV). The initial symptoms were gastrointestinal in nature for most patients (79.2%). High-dose methylprednisolone was initially administered to most patients (91.7%) with lupus cystitis. Two patients (8.3%) died of urinary tract infections. Sixty-five age- and sex-matched patients with SLE who were admitted with other manifestations were included as the control group. Patients with lupus cystitis showed a lower C3 level (p = 0.031), higher SLE Disease Activity Index score (p = 0.006), and higher ESR (p = 0.05) upon admission; more frequently had a history of LMV prior to admission (p < 0.001); and less frequently had a history of neuropsychiatric lupus (p = 0.031) than did patients with SLE but without lupus cystitis. The occurrence of lupus cystitis was associated with a history of LMV (OR, 21.794; 95% CI, 4.061-116.963). The median follow-up period was 3.4 years, and the cumulative one-year mortality rate was 20%. Complications developed in 33.3% of patients with lupus cystitis and were related to survival (log-rank p = 0.021). Our results suggest that the possibility of lupus cystitis should be considered when a patient with SLE and history of LMV presents with gastrointestinal symptoms or lower urinary tract symptoms. Development of complications in patients with lupus cystitis can be fatal. Thus, intensive treatment and follow-up are needed, especially in the presence of complications.

Low compliance bladder plays a role in hydronephrosis in lupus cystitis: a case report

Lupus cystitis is uncommon in patients with systemic lupus erythematosus (SLE). Lupus cystitis is usually associated with hydronephrosis. To our knowledge, it is considered to be due to distal ureteral obstruction at the ureterovesical junction. However, in a Chinese female, we found low compliance bladder played a role in hydronephrosis.

Lupus Mesenteric Vasculitis With GI and Genitourinary Tract Involvement

A53-year-old woman presented with watery diarrhea of 6 months’ duration. She had no systemic disease or surgical history and did not take any medications. The associated symptoms included nausea, vomiting, dull abdominal pain without radiation, urinary urgency, frequency, and incontinence, and marked body weight loss (21 kg in 6 months). On admission, the physical examination showed hypoactive bowel sounds with slight periumbilical tenderness. Neither rebound tenderness nor guarding was elicited. No leg edema or skin rash was noted. Laboratory data showed no leukocytosis, mild increase of C-reactive protein level (1.69 mg/dL), mildly increased liver function tests (aspartate aminotransferase, 84 IU/L; alanine aminotransferase, 59 IU/L), and hypoalbuminemia (2.1 g/dL). Urine analysis showed proteinuria and pyuria. Routine stool analysis showed no evidence of infection or bleeding. Enhanced computed tomography of the abdomen showed diffuse wall thickening of stomach, small bowel, colon, and rectum (Figure A, target sign, arrow) and prominent engorgement of the mesenteric vessels with a palisade pattern (Figure B, comb sign, arrow). Bilateral hydroureteronephrosis and swelling of the bladder wall also were noted (Figure C, arrow). Further laboratory studies showed increased antinuclear antibody level (1:320, diffuse type), increased anti–double-stranded DNA (85 IU/mL), and low complement level (C3, 32 mg/dL; C4, 5.87 mg/dL). The clinical presentation and computed tomography image were compatible with lupus mesenteric vasculitis and lupus cystitis. The patient received cyclophosphamide and methylprednisolone pulse therapy, followed by steroid maintenance treatment. The patient’s clinical condition and follow-up computed tomography 17 days after admission showed significant improvement. Lupus mesenteric vasculitis is a rare presentation (0.2%–9.7%) among patients with systemic lupus erythematosus (SLE), but it is more common in SLE patients with abdominal pain (29%–65%). Typical image findings of lupus mesenteric vasculitis include focal or diffuse bowel-wall thickening with target sign, prominent engorgement of mesenteric vessels with comb sign, and ascites. Lupus cystitis frequently is associatedwith bowel involvement. Edema of the vesical triangle resulted in hydronephrosis and contraction of the bladder, and further induced urinary frequency and inflammation of the bladder. It is worthwhile to note that vasculitis and cystitis can be the initial manifestations of SLE.

Cystectomy for obstructive uropathy secondary to lupus cystitis: a learning point for urologists?

We present the case of a 72-year-old woman with obstructive uropathy secondary to interstitial cystitis-like pathology as the presenting features of systemic lupus erythematosus (SLE). After her initial presentation with visible haematuria, she was shown to have hydroureteronephrosis and a bulky bladder mass. Histology of bladder biopsies demonstrated severe inflammation. Following unsuccessful attempts to stent the ureters, worsening renal function and a concern regarding possible malignancy, a cystectomy and ileal conduit were performed. Final histology showed her bladder had only benign inflammatory changes. Subsequent investigations led to a diagnosis of SLE. We suggest that in cases of obstructive uropathy in association with severe inflammation of the bladder, lupus cystitis should be considered at an early stage and managed with steroids in the first instance. This may lead to resolution of the underlying pathology and thus avoid the need for urinary diversion and cystectomy.

Lupus mesenteric vasculitis: Clinical features and associated factors for the recurrence and prognosis of disease

ObjectiveTo evaluate the clinical characteristics of lupus mesenteric vasculitis (LMV) and identify the potential factors and appropriate treatments that are associated with disease relapse and prognosis in LMV. MethodsA retrospective cohort study was performed among patients admitted to the First Affiliated Hospital of Sun Yet-sen University between 2002 and 2011. Demographic information, clinical symptoms, laboratory findings, imaging characteristics like abdominal CT scan, ultrasonography, medications including corticosteroid, cyclophosphamide, and other immunosuppressive agents, and outcomes were documented. The endpoints of the study were defined as occurrence of severe complications that needed surgical intervention, disease recurrence, or death. ResultsOut of 3823 systemic lupus erythematosus (SLE) patients, 97 were diagnosed with mesenteric vasculitis with the overall prevalence of 2.5%. Among these 97 LMV patients, 13 died because of serious complications (13/97, 13.4%) and 2 presented intestinal perforation during the induction therapy stage. The logistic regression multivariate analysis indicated that leukopenia [peripheral WBC, odds ratio (OR) = 0.640, 95% confidence interval (CI): 0.456–0.896, P = 0.009], hypoalbuminemia (serum albumin, OR = 0.891, 95% CI: 0.798–0.994, P = 0.039) and elevated serum amylase (OR = 7.719, 95% CI: 1.795–33.185, P = 0.006) were positively associated with the occurrence of serious complications, while intravenous cyclophosphamide (CYC) therapy inhibited the occurrence of serious complications (OR = 0.220, 95% CI: 0.053–0.903, P = 0.036). A total of 79 patients who achieved remission were followed-up for 2–96 months and 18 cases experienced disease relapse (18/79, 22.8%). The statistical analysis adjusted by Cox proportional hazards models indicated that high-dose CYC therapy (≥1.0g/m2/month) was a protective factor for disease relapse and led to better outcomes [hazard ratio (HR) = 0.209, 95% CI: 0.049–0.887, P = 0.034], while the severe thickness of the bowel wall (>8mm) was a risk factor (HR = 7.308, 95% CI: 1.740–30.696, P = 0.007). LMV and lupus cystitis occurred concurrently in 22 (22/97, 22.7%) patients, and the symptoms of urinary tract resolved after treatment with corticosteroid and immunosupressants. ConclusionLMV is one of the serious complications of SLE with high mortality. The current study demonstrated that leukopenia, hypoalbuminemia, and elevated serum amylase were associated with severe adverse events, while CYC therapy led to better outcomes during remission-induction stage. Severe thickness of the bowel was a risk factor while high-dose CYC therapy was a protective factor for disease relapse in intensification therapy stage. It is necessary to evaluate the urinary tract involvement once LMV is diagnosed due to the frequent coexistence of these 2 diseases.

Two cases of lupus cystitis and literature review

To improve the understanding of lupus cystitis. Clinical manifestations, laboratory Results , and image information of 2 cases of lupus cystitis were analysed retrospectively, and another 6 cases in the literature were reviewed. Two patients were female. The urinary symptoms followed the gastrointestinal symptoms. Ureterectasia and hydronephrosis were detected in both patients, and intestinal pseudo-obstruction was detected in one patient. In the 6 cases from the literature, ureterectasia and hydronephrosis were detected in all patients, and intestinal pseudo-obstruction was detected in 4. The possibility of lupus cystitis should be considered when lupus patients complain of urinary or bowel symptoms. Glucocorticoid and immunodepressant are effective for lupus cystitis.

Clinical Features of Lupus Cystitis Complicated with Hydroureteronephrosis in a Chinese Population: Table 1.

Lupus cystitis is a relatively rare complication of systemic lupus erythematosus (SLE). The clinical characterization of it remains obscure. We sought to provide insight for timely recognition and diagnosis of lupus cystitis, and for efficacious management of this disease entity. The clinical files of 18 patients with lupus cystitis treated at Peking Union Medical College Hospital were reviewed. Clinical manifestations, laboratory investigations, therapeutic measurements, and clinical outcomes were analyzed. There were both male and female patients with a wide range of ages. The interval from onset of SLE to hydroureteronephrosis varied from 0 to 5 years. The most common clinical presentations were gastrointestinal (GI) symptoms, while urinary symptoms were less common and relatively mild. Lupus nephritis often presented concomitantly with lupus cystitis but was relatively less active pathologically. Laboratory findings showed a high rate of positive anti-SSA. Therapy of prednisone plus cyclophosphamide was effective for lupus cystitis. Delayed diagnosis and treatment may lead to irreversible obstructive uropathy and permanent loss of renal function. Lupus cystitis may not be so rare as has been thought. The diagnosis of lupus cystitis should be considered when patients with SLE present with GI symptoms, and therapy should begin as early as possible.

Comparison between Japanese and Non-Japanese Features of Lupus Cystitis Based on Case Reports Including Novel Therapy and a Literature Review

This study was carried out to determine the characteristics of lupus cystitis and to compare those of Japanese cases with those of non-Japanese cases. We also report a novel therapy for lupus cystitis refractory to corticosteroid. For the literature search, published reports on lupus cystitis were searched by MEDLINE and ICHUSHI WEB. The inclusion criterion was interstitial cystitis, fulfilling the SLE classification criteria of American College of Rheumatology in 1997 and with either hydronephrosis detected by image studies (either computed tomography or ultrasonography) or bladder histopathology consistent with lupus cystitis. Interstitial cystitis without the fulfillment of the classification criteria was excluded. Patient demographic data and clinical data were retrieved from the literature and analyzed. Including the present 2 cases, a total of 78 cases were identified as definitive cases of lupus cystitis (35 non-Japanese cases and 43 Japanese cases). Female patients accounted for 90.7%. The preceding gastrointestinal symptoms and subsequent urinary symptoms were the most frequent. Anti-double strand DNA antibody most often expressed in the 76.1% of the patients. Mean age and the prevalence of vomiting were significantly higher among Japanese patients compared to non-Japanese cases and the prevalence of CNS involvement was lower among Japanese patients (p=0.03, 0.04 and 0.001). We report a novel therapy (cetirizine hydrochloride) for lupus cystitis refractory to corticosteroid in one of the present cases. Lupus enteritis, female gender, and positive anti-dsDNA antibody are risk factors for lupus cystitis. Japanese cases showed older age, a higher prevalence of vomiting and a lower prevalence of CNS involvement. We also report the efficacy of the novel use of cetirizine hydrochloride for lupus cystitis refractory to corticosteroid.

Lupus cystitis: a case report and review of the literature

Lupus cystitis is a rare complication associated with systemic lupus erythematosus (SLE). This rare disorder has been reported mainly from East Asia, especially from Japan. In this study, we describe a patient with lupus cystitis and review 37 reported cases in Japan. Bowel symptoms preceded urinary symptoms in 13 of 37 (35%) patients, whereas five patients complained of only bowel symptoms. Hydronephrosis was detected in 34 of 37 patients (92%). The possibility of lupus cystitis should be considered when patients complain of bowel or urinary symptoms. Examination for hydronephrosis is critical for diagnosis.

Lupus Cystitis Successfully Treated With Cetirizine. A Report Of Four Cases And A Review Of 44 Cases Of Lupus Cystitis In Japan.

Lupus Cystitis Successfully Treated With Cetirizine. A Report Of Four Cases And A Review Of 44 Cases Of Lupus Cystitis In Japan.