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Original article

Sjögren’s syndrome complicated by interstitial cystitis: A case series and literature review Christelle Darrieutort-Laffite a,b , Vincent André c , Gilles Hayem d , Alain Saraux e , Véronique Le Guern a , Claire Le Jeunne a , Xavier Puéchal a,f,∗ a National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, hôpital Cochin, université Paris Descartes, Assistance publique–Hôpitaux de Paris, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France b Department of Rheumatology, Nantes University Hospital, 44000 Nantes, France c Department of Rheumatology, Le Mans General Hospital, 72000 Le Mans, France d Department of Rheumatology, hôpital Bichat, Assistance publique–Hôpitaux de Paris, 75018 Paris, France e Department of Rheumatology, hôpital de la Cavale Blanche, université de Brest, 29200 Brest, France f Inserm U1016, CNRS UMR 8104, institut Cochin, 75014 Paris, France

a r t i c l e

i n f o

Article history: Accepted 11 December 2014 Available online xxx Keywords: Sjögren’s syndrome Inflammatory cystitis Acute renal failure

a b s t r a c t Objectives: To characterize the interstitial cystitis (IC) associated with Sjögren’s syndrome (SS). Methods: Report of three new cases. Only cases fulfilling the American-European consensus criteria for SS and the European Society for the Study of Interstitial Cystitis criteria with positive histological findings for IC were included. Results: Thirteen cases of SS and IC have been reported in women, including the three reported here, with a mean age of 54 years. SS appeared first in 77% (n = 10) of cases, a mean of 6.6 years before IC. The symptoms of IC included pollakiuria (n = 11), lower abdominal pain (n = 8), urinary urgency (n = 5), painful micturition (n = 6), hematuria (n = 3) and dysuria (n = 3). Urinary dilatation occurred in three cases, leading to acute renal failure in two patients. The diagnosis of IC was confirmed by anatomical evidence of cystitis inflammation on bladder biopsy in all (n = 13) patients. Treatment was reported for nine patients, seven of whom (78%) received corticosteroid treatment, which was partially or completely effective in six cases. Immunosuppressive treatment was added in three cases (cyclosporine, n = 2; azathioprine, n = 1; cyclophosphamide, n = 1). Local bladder treatments were performed, with hydraulic distension in five cases and DMSO instillation in one patient. A urinary catheter was inserted in the two cases of acute obstructive renal failure. Conclusions: Urinary symptoms without infection should lead the physician to consider a diagnosis of IC in SS patients. Urinary dilatation may occur, leading to acute obstructive renal failure. Corticosteroid treatment may be effective and local treatments have been tried. © 2015 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.

1. Introduction Interstitial cystitis (IC) is a chronic inflammatory disease of the bladder occurring mostly in middle-aged women [1]. It is characterized by pollakiuria, nycturia, urinary urgency, pelvic or suprapubic pain and negative results on urine culture. Diagnostic criteria were first proposed by the National Institute of Diabetes and Digestive

∗ Corresponding author. National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, hôpital Cochin, université Paris Descartes, Assistance publique–Hôpitaux de Paris, 27, rue du Faubourg SaintJacques, 75014 Paris, France. Tel.: +33 1 58 41 32 41; fax: +33 1 58 41 29 68. E-mail address: [email protected] (X. Puéchal).

and Kidney Diseases (NIDDK) in 1988 [2]. However, only one third of the patients whom experts considered to have IC actually fulfilled these criteria [3]. The International Continence Society (ICS) subsequently defined “painful bladder syndrome” (PBS). PBS is “the complaint of suprapubic pain related to bladder filling, accompanied by other symptoms, such as increased daytime and nighttime frequency, in the absence of proven urinary infection or other obvious pathology” whereas the diagnosis of IC is restricted to patients with typical inflammatory cystoscopy and histological features [4]. However, a fraction of patients (34%) believed to have PBS did not fulfill these criteria and those meeting the defined conditions for ICS did not differ in any major way from those who did not, suggesting that this definition lacks sensitivity [5]. New criteria were established in 2008 by the European Society for the Study of

http://dx.doi.org/10.1016/j.jbspin.2014.12.007 1297-319X/© 2015 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.

Please cite this article in press as: Darrieutort-Laffite C, et al. Sjögren’s syndrome complicated by interstitial cystitis: A case series and literature review. Joint Bone Spine (2015), http://dx.doi.org/10.1016/j.jbspin.2014.12.007

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Interstitial Cystitis (ESSIC) that defined interstitial cystitis/bladder painful syndrome (IC/BPS) [6]. IC/BPS was defined as chronic pelvic pain, pressure or discomfort perceived to be related to the urinary bladder accompanied by at least one other urinary symptom, such as persistent urge to void or high frequency of urination. Differential diagnoses, such as carcinoma, infection, urethral diverticulum, bladder or ureteric stone, neurogenic bladder, or irradiation- or drug-related problems, should be excluded as possible causes of symptoms. A classification of BPS was suggested on the basis of cystoscopy and histological findings. An association between Sjögren’s syndrome (SS) and IC was first reported by van de Merwe in 1993 [7], but only a few cases of this association have since been reported [8–13]. We report three new cases of IC associated with SS. We also performed an exhaustive literature review, with the aim of determining the clinical and pathological characteristics and outcomes of this syndrome in SS patients. 2. Patients and methods We present three new cases of IC associated with SS and review all published reports on this type of cystitis. The three cases were selected by computerized searches of the data for SS patients followed at five departments of rheumatology/internal medicine responsible for the care of more than 1000 patients with SS. We selected patients fulfilling both the American-European consensus criteria for SS [14] and the 2008 IC criteria of the European Society for the Study of Interstitial Cystitis (ESSIC) [6]. For inclusion in this study, the cases also had to have a histological confirmation of IC diagnosis. Medical records were reviewed and analyzed according to predetermined criteria. Published cases of IC in SS patients were identified by a computerized Medline search of articles published from 1980 to 2013. The keywords used were SS, autoimmune disease, systemic disease, cystitis, inflammatory cystitis, hydronephrosis, renal failure, urinary tract symptoms. Only publications in English or French were selected for review. We also searched the references of the reports found, to identify additional reports. We included only those cases for which sufficient detail was provided for individual analysis. Cases were included only if patients fulfilled the American-European consensus criteria for SS [14] and pathological data demonstrated inflammatory infiltration of the bladder wall and/or cystoscopy abnormalities, such as glomerulation or Hunner’s lesion, as described in the ESSIC criteria. This led to the exclusion of two cases from our departments without documented anatomical cystitis and of two published cases of urinary tract symptoms for lack of details about clinical features [15]. 3. Case reports 3.1. Case no. 1 A 40-year-old woman was admitted with recurrent vomiting, diarrhea, abdominal pain and urinary symptoms of 15 months’ duration. Primary SS had been diagnosed six years earlier (dry eyes and mouth, inflammatory arthralgia, high serum immunoglobulin G concentration, lymphopenia, presence of antinuclear antibodies and anti-SSA (anti-Ro) antibodies and lymphocytic infiltration of the labial salivary gland [focus score = 1]). This patient had been treated with hydroxychloroquine and anetholtrithione for four years. One year before admission, she presented chronic abdominal symptoms (for > 6 months), including pain, vomiting, diarrhea and weight loss (8 kg). Laboratory analysis showed low serum albumin concentration (31.8 g/L), normal C-reactive protein

Fig. 1. Patient no. 1: abdominal computed tomography scan showing bilateral urinary dilatation.

concentration (0.3 mg/dL), creatinemia (76 ␮mol/L; clearance = 78 mL/min (MDRD)), and normal results in hepatic function tests. Complete digestive endoscopy results were normal. Abdominal computed tomography showed a severe bilateral ureteral dilatation with bilateral ureterohydronephrosis (Fig. 1). Cystoscopy showed a left obstructive ureteral meatus. A ureteral catheter was inserted, relieving all symptoms. This catheter was removed three months later. A few months later, the patient presented digestive symptoms, with frequent urination, nycturia and left lumbar pain. Urine cultures were sterile. Abdominal ultrasound scans showed a recurrence of bilateral dilatation of the urinary tract. Cystoscopy showed severe trigonal inflammation with ulcerated ureteral meatus. Acute renal failure rapidly developed (creatinemia: 236 ␮mol/L, clearance = 21 mL/min (MDRD)). Prednisone treatment (0.5 mg/kg/day) was initiated, leading to a rapid decrease in serum creatinine concentration (110 ␮mol/L). Bilateral ureteral catheters were then inserted and creatinemia decreased towards normal values (72 ␮mol/L). Bladder biopsy revealed an inflammatory infiltrate of the bladder wall, with lymphocytes and plasmocytes. A diagnosis of IC associated with primary SS was given. Urinary urgency recurred when the patient decreased the dose of prednisone to below 10 mg/day. Ultrasound scans again showed bilateral ureteral dilatation, but with the urinary catheters still in position. Creatinemia remained normal. The patient was then treated with two boluses of 100 mg methylprednisolone, with no improvement in symptoms. Despite monthly infusions of cyclophosphamide over a period of three months, urinary dilatation persisted. However, no evidence of inflammation was found on cystoscopy. The patient died six months later from metastatic breast cancer. 3.2. Case no. 2 A 64-year-old woman had presented urinary symptoms since 2002, with urinary urgency, pollakiuria and nycturia. Renal ultrasound, computed tomography and intravenous urography results were normal. Urine cultures were sterile, including BK cultures. Cystoscopy showed macroscopic inflammation of the bladder and biopsies showed a lymphoplasmocytic infiltration of the bladder wall. IC was diagnosed. In 2004, the patient presented subjective and objective sicca syndrome, arthralgia, myalgia of the lower limbs and paresthesia of

Please cite this article in press as: Darrieutort-Laffite C, et al. Sjögren’s syndrome complicated by interstitial cystitis: A case series and literature review. Joint Bone Spine (2015), http://dx.doi.org/10.1016/j.jbspin.2014.12.007

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the feet. An electromyogram revealed an axonal sensory and motor neuropathy affecting the legs. No antinuclear antibodies or antiSSA and SSB antibodies were detected. Minor salivary gland biopsy showed lymphocytic sialadenitis (Focus score = 1). The patient was diagnosed with SS. Two hydraulic bladder distensions were transiently effective. Anticholinergic and alpha-blocker treatments were unsuccessful. Urinary symptoms improved on corticosteroid treatment (between 5 and 7 mg/day). The patient is still on this treatment, which has been maintained over the last seven years.

3.3. Case no. 3 A 79-year-old woman with eye dryness and keratitis was diagnosed with SS in 2009. The minor salivary gland biopsy revealed sialadenitis (focus score = 1). The patient tested positive for rheumatoid factor and AAN, but without specificity for Ro (SSA) or La (SSB). She was treated with anetholtrithione. Two years after SS diagnosis, she presented lower limb pain with burning sensations. Electromyography showed axonal sensorimotor polyneuropathy. In December 2011, the patient complained of frequent urination during both the day and the night, associated with pain on urination. Urodynamic investigations showed a reduced bladder capacity and cystoscopy revealed a fibrous bladder wall with partial obstruction of the ureters. Creatinemia was normal. Bladder biopsies revealed an abraded urothelial epithelium with inflammatory infiltrate in the chorion consisting of lymphocytes and large numbers of plasmocytes. Different treatments, such as anticholinergic treatment, hydrodistension and sacral neurostimulation, were tried to improve symptoms, but were unsuccessful.

4. Literature review A literature review identified 13 reported cases of IC associated with SS, including our three cases [8–13]. The clinical, biological and pathological characteristics of these cases are shown in Table 1. It should be pointed out that, in the first report of this association in 10 consecutive patients with IC [7], only two were suffering from SS according to the criteria defined by Daniels and Talal [16]. However, on the basis of the 2002 American-European consensus [14], four of these 10 patients could be considered to have SS. The 13 cases identified were all women, with a mean age of 54 [40–77] years. SS appeared first in 77% of cases (n = 10/13) with a mean time interval of 6.6 (± 6.2) years until the diagnosis of IC. Antinuclear antibodies were present in 62% (n = 8/13), anti-SSA (Ro) in 69% (n = 9/13), anti-SSB (La) in 31% (n = 4/13), and rheumatoid factor in 36% (n = 4/11) of the patients. Minor salivary gland biopsy was positive in all cases, with a focus score ≥ 1. On the basis of Chisholm staging of biopsy specimens, four patients were considered to have stage III disease and six were considered to have stage IV disease (stage not reported for three patients). Nine patients presented systemic signs of SS, with arthralgia/arthritis, peripheral neuropathy and Raynaud’s phenomenon in seven, two and one patient, respectively. The classical symptoms of IC detected were pollakiuria (n = 11), lower abdominal pain (n = 8), urinary urgency (n = 5), painful micturition (n = 6), hematuria (n = 3) and dysuria (n = 3). Urinary dilatation occurred in three cases and resulted in acute renal failure in two of these three patients. The diagnosis of IC was confirmed by bladder biopsy in all patients (n = 13). Histological examination of the bladder wall provided evidence of ulceration and submucosal inflammation. The inflammatory infiltrate consisted predominantly of lymphocytes

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and large numbers of plasma cells [17]. Other lesions, such as detrusor mastocytosis and fibrosis have been described. There is no consensus concerning the most appropriate treatment for IC. The various treatments used in these patients are summarized in Table 2. Seven of the nine patients for whom treatment was reported (78%) received corticosteroid treatment orally or intravenously. This treatment was partially or completely effective in six cases, with an increase in urine volume in two cases [10,11] and an improvement of urinary symptoms in four cases [8,12, patients’ nos. 1 and 2]. Corticosteroids were only transiently effective in the remaining patient [patient no. 1]. Immunosuppressive therapy was proposed in three cases [10,12, patient no. 1] (cyclosporine n = 1 [10]; azathioprine followed by cyclosporine n = 1 [12]; cyclophosphamide n = 1 [patient no. 1]). Cyclosporine was effective in the two cases in which it was used, increasing bladder capacity and decreasing abdominal pain [10] or urinary symptoms [12]. By contrast, azathioprine and cyclophosphamide did not improve urinary symptoms [12, patient no. 1). Local treatments of the bladder, consisting of hydraulic distension (n = 5) or DMSO instillation (n = 1), were also performed. A urinary catheter was inserted in the two cases of acute obstructive renal failure. In the two cases of severe hydronephrosis leading to acute renal failure, residual urinary dilatation persisted after catheterization, as in patient no. 1, but there was no evidence of inflammation on subsequent cystoscopy examination. 5. Discussion The association of IC with SS is rare, but may be severe. Our screening of more than 1000 patients with SS followed at five departments of rheumatology/internal medicine identified only three confirmed cases, giving a prevalence of less than 0.3/100 patients. Nevertheless, the frequency of this association may have been underestimated due to misidentification. Among 222 patients with interstitial cystitis, SS has been diagnosed in only 1 patient (0.4%) [18]. On the other hand, a prevalence of 5% of probable IC (diagnosed according to the O’Leary-Sant questionnaire) has been reported in 870 patients from the Finnish Sjögren’s syndrome patients’ organization [19]. Our first patient was the only one to present gastrointestinal symptoms. Such an association of gastrointestinal symptoms and IC has been reported in systemic lupus erythematosus (SLE) [20]. In some cases, these symptoms correspond to an intestinal pseudoobstruction, occurring at the same time as ureterohydronephrosis. In SLE, abdominal CT scans frequently reveal the presence of dilated small and large intestines, with a thickened intestinal wall, in patients with bilateral ureteral dilatation [20]. Pathological examination of the gastrointestinal tract may reveal widespread myocyte necrosis in the muscularis propria, with active inflammatory cell infiltration, severe atrophy of the muscularis and fibrosis [21]. However, gastrointestinal imaging and biopsy results were normal for our patient. The 3 following methods of diagnostic have the higher level of recommendation [22]. Cystoscopy after hydrodistension is often carried out, although it is somewhat controversial because of the lack of specific or pathognomonic findings; cystoscopy often disclosed urothelial glomerulations, petechiae or submucosal hemorrhages; mucosal lesions (Hunner’s ulcers) are characteristic of IC, but are described in only 5–15% of cases. Hydrodistension during cystoscopy can also help diagnose IC showing haemorrhage [22]. Moreover, cystoscopy is useful to rule out differential diagnosis. Histological data combined with those of cystoscopy are useful to classify the disease [23]. Urodynamic can be interesting in the diagnostic process showing a reduction of volumes and reduced bladder compliance. Urodynamic findings differ from those of overactive bladders [24].

Please cite this article in press as: Darrieutort-Laffite C, et al. Sjögren’s syndrome complicated by interstitial cystitis: A case series and literature review. Joint Bone Spine (2015), http://dx.doi.org/10.1016/j.jbspin.2014.12.007

Van de Merwe et al., 1993 Van de Merwe et al., 1993 Van de Merwe et al., 1993 Van de Merwe et al., 1993 Higuchi et al., 1998 Fauchais et al., 2001 Shibata et al., 2004

F/57 F/44 F/42 F/62 F/53 F/61 F/53

52 39 39 NS 43 44 52

Fukaya et al., 2005 Emmungil et al., 2011

F/51 F/42

Liang et al., 2014 Darrieutort et al., 2015

Age at IC onset ANA

SSA (Ro)

SSB (La)

RF

High SIGC

MSGB biopy

Systemic signs

U symptoms

GI symptoms

U dilatation

Renal failure

LAP, PM LAP, PM, HU LAP, PM, PO LAP, PM, PO LAP, PO LAP, PO LAP, PO, Dys Urg, Ol HU, PO Dys, Urg, PO, LAP PM, Urg, PO PO, Dys, Urg

No No No No No No No

NS NS NS NS NS NS Yes

No No

Yes NS

NS NS NS NS NS NS Yes 712 ␮mol/L No No

No V, D

No Yes

Urg, PO

No

No

No Yes 236 ␮mol/L No

PO, PM, HU

No

No

No

54 43,5 37 58 48 61 50,5

P N N N P P N

N P P N P P P

N N P N N P N

N N N N P P N

NS NS NS NS Yes Yes Yes

P P P P P P P

No Arthralgia Arthralgia Arthralgia No Arthralgia No

50,5 36

51 41

P P

P P

P N

P NS

Yes NS

P P

Arthritis Raynaud

F/64 F/40

45 34

62 39

P P

P P

P N

N N

N Yes

P P

No Arthritis

Darrieutort et al., 2015

F/64

62

61

N

N

N

NS

NS

P

Darrieutort et al., 2015

F/77

75

77

P

N

N

P

N

P

Neuropathy Arthromyalgia Neuropathy

SS: Sjögren’s syndrome; IC: interstitial cystitis; P: positive; N: negative; NS: not specified; ANA: antinuclear antibodies; RF: rheumatoid factor; MSGB: minor salivary gland biopsy; SIGC: serum IgG concentration; GI: gastrointestinal; U: urinary; LAP: lower abdominal pain; PM: painful micturition; Dys: dysuria; Urg: urgency; Ol: oliguria; HU: hematuria; PO: pollakiuria; V: vomiting; D: diarrhea.

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Authors Year

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Table 1 Clinical, biological and pathological findings for 13 reported patients with interstitial cystitis associated with Sjögren’s syndrome.

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Table 2 Treatment of 13 reported cases of interstitial cystitis associated with Sjögren’s syndrome. Authors year

Treatment

Dose and effects

Length of follow-up and outcome

Higuchi et al., 1998

Hydraulic dilatation

NS CS successfully tapered

Fauchais et al., 2001

CS (prednisolone, 30 mg/day) Methenamine Vesical instillations of DMSO

Transitory improvement of symptoms (1 month) Improvement of symptoms Ineffective Disappearance of abdominal pain and slight decrease in urinary frequency Increase in diuresis, normalization of serum creatinemia Start of spontaneous urination and increase in urinary volume Increase in spontaneous urination and bladder capacity and improvement of abdominal pain (after 8 months) Increase in urinary volume, persistence of a high frequency of urination Decrease of urinary frequency Ineffective

Shibata et al., 2004

Urinary catheter for acute renal failure CS (3 MP pulse therapv (500 mg) and prednisolone (40 mg/day)) CS + Cyc (100 mg/day)

Fukaya et al., 2005

Emmungil et al., 2011

Liang et al., 2014

Darrieutort et al., 2014

Darrieutort et al., 2014

CS (prednisone 30 mg/day)

Hydraulic dilatation CS (MP 0.3 mg/kg/day and decrease to 4 mg/day in 3 months) + AZA (2 mg/kg/day) Intravesical lidocaine + amitriptyline Cyclosporine (1.5 mg/kg/day) + MP 0.3 mg/kg/day decrease to 4 mg/day in 2 months CS (dexamethasone 5 mg/day–1.5 mg/day) CS (prednisone 20 mg/day) Tamsulosin Hydraulic dilatation CS (prednisone 0.5 mg/kg/day) Urinary catheter

2 boluses of MP (100 mg) Cyclophosphamide (3 boluses of 500 mg) Hydraulic dilatation twice Oxybutynin Tamsulosin CS (prednisone, 5–7 mg/day)

Darrieutort et al., 2014

Anticholinergic treatment Sacral neurostimulation Hydraulic dilatation

Transitory improvement of symptoms (2 months) Decrease in symptoms after 4 months of treatment Transitory mild improvement of symptoms Mild improvement of symptoms Ineffective Ineffective Disappearance of acute renal failure, decrease in urgency and urinary frequency and GI symptoms but recurrence of urinary urgency and dilatation at doses under 10 mg/day

NS

2 years, Cyclosporine (100 mg/day): Preservation of bladder capacity Persistence of bilateral ureterohydronephrosis

NS

1 year, Cyc same dose + MP 4 mg/day: clinical remission

6 months

6 months, Persistence of bilateral ureterohydronephrosis No more cystic inflammation Death (metastatic breast cancer)

Ineffective Ineffective Transitory decrease in urinary frequency Ineffective Ineffective Improvement of urinary symptoms Ineffective Ineffective Ineffective

7 years, CS dependence (5–7 mg/day)

1 month, Persistence of urinary symptoms

CS: corticosteroids; DMSO: dimethyl sulfoxide; Cyc: cyclosporin; AZA: azathioprine; MP: methylprednisolone; GI: gastrointestinal.

Several treatments have been used in IC/BPS. In 2011, the American Urological Association proposed guidelines for treatment based on a systematic review of the literature [25]. Many oral therapies have been used empirically. Amitriptyline has been proposed and reported to be effective in open-labelled, not randomised and uncontrolled studies [22]. For intravesical therapies, botulinum toxin [26], lidocaine [27] and DMSO [22] have been administered. Hydrodistension, used for the diagnosis of IC/BPS, can also be used for its treatment [28]. In IC associated with SS, some of the above therapies have been tried with an inconstant efficiency. Corticosteroids are the most frequently used treatment in reported cases while they are not recommended in IC/BPS treatment. Immunosuppressive treatments have also been tried in SS-associated IC.

The etiology of IC/BPS remains unknown, although this condition has certain characteristics typical of autoimmune diseases [29,30]: frequent association with rheumatoid arthritis, systemic lupus erythematosus, inflammatory bowel disease, and Hashimoto thyroiditis; frequent blood test abnormalities, such as high levels of IgG, low levels of C4, the presence of antinuclear antibodies (ANA) and antibodies directed against mitochondria and parietal cells; lymphocyte infiltration, deposits of immunoglobulin and complement in the inflamed bladder wall of patients. On the basis of the available data, it remains unclear whether a similar pathogenic process is responsible for IC and SS. However, some common features have been reported, such as high levels of HLA-DR expression in the epithelia and a large proportion of

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CD4+ T cells in the inflammatory infiltrate [11]. Van de Merwe and coworkers suggested that autoantibodies blocking muscarinic receptors may contribute to IC [31]. According to this hypothesis, autoantibodies against the muscarinic M3-receptor (M3R) for acetylcholine (Ach) may block the effect of Ach on exocrine secretion in the lachrymal and salivary glands of patients with SS and stimulate contraction of the detrusor smooth muscles in the bladder in cases of IC. Indeed, the passive transfer of IgG with inhibitory anti-M3R activity from patients with SS into mice induced a paradoxical increase in the contractile response of the detrusor to cholinergic stimulation [32]. However, it has been reported that most SS patients have autoantibodies directed against the M3R (9 of 11) [33]. This suggests that the occurrence of IC cannot be accounted for solely by the presence of such antibodies. Further studies are required to clarify the role of anti-M3R antibody in the development of IC in patients with SS and to identify other factors potentially involved in the association of these diseases. In conclusion, IC is a very rare, but potentially severe extraglandular feature of primary SS, the frequency of which may be underestimated. Clinicians should accept a diagnosis of IC only if objective signs of inflammation are found on cystoscopy or biopsy, in the absence of infection, because urinary complaints are frequent among SS patients (61%) [34]. Treatment remains difficult. Corticosteroids may be given, but there is still little evidence that this treatment is effective. Relapses are frequent and residual dilatation may occur, especially in cases of severe hydronephrosis associated with renal failure. This persistent dilation may favour urinary infection. Emergency urinary catheterization may be required in rare cases of acute obstructive renal failure due to hydronephrosis. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Propert KJ, Schaeffer AJ, Brensinger CM, et al. A prospective study of interstitial cystitis: results of longitudinal follow-up of the interstitial cystitis data base cohort. The interstitial cystitis data base study group. J Urol 2000;163: 1434–9. [2] Gillenwater JY, Wein AJ. Summary of the National Institute of Arthritis, Diabetes, Digestive and Kidney Diseases Workshop on interstitial cystitis, National Institutes of Health, Bethesda, Maryland, August 28–29, 1987. J Urol 1988;140:203–26. [3] Hanno PM, Landis JR, Matthews-Cook Y, et al. The diagnosis of interstitial cystitis revisited: lessons learned from the National Institutes of Health Interstitial Cystitis Database study. J Urol 1999;161:553–7. [4] Abrams PH, Cardozo L, Fall M, et al. The standardisation of terminology of lower urinary tract function: report from the standardisation sub-committee of the International Continence Society. Neurourol Urodyn 2002;21:167–78. [5] Warren JW, Meyer WA, Greenberg P, et al. Using the International Continence Society definition of painful bladder syndrome. Urology 2006;67: 1138–42. [6] Van de Merwe JP, Nordling J, Bouchelouche P, et al. Diagnostic criteria, classification, and nomenclature for painful bladder syndrome/interstitial cystitis: an ESSIC proposal. Eur Urol 2008;53:60–7. [7] Van de Merwe JP, Kamerling R, Arendsen E, et al. Sjögren’s syndrome in patients with interstitial cystitis. J Rheumatol 1993;20:962–6.

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Please cite this article in press as: Darrieutort-Laffite C, et al. Sjögren’s syndrome complicated by interstitial cystitis: A case series and literature review. Joint Bone Spine (2015), http://dx.doi.org/10.1016/j.jbspin.2014.12.007

Sjögren's syndrome complicated by interstitial cystitis: A case series and literature review.

To characterize the interstitial cystitis (IC) associated with Sjögren's syndrome (SS)...
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