Refractory case of Interstitial Cystitis associated with Sjogren’s syndrome : Beneficial effect of Steroids in Treatment

NVM James, MM Bheri and M Taube

Department of Urology, West Wales General Hospital, Carmarthen SA31 2AF.

Mr.MM Bheri

Associate Urologist, Department of Urology, West Wales General Hospital, Carmarthen – SA31 2AF

E mail: sum71114@aol.com

SMJ 2006 51(3): 50

Abstract

Interstitial Cystitis (IC) is of unknown aetiology, but recently has been associated with Sjogren’s syndrome. This association is much stronger than was originally recognised. In these patients the question has always been whether the bladder symptoms were a manifestation of IC or an autoimmune process. The beneficial response seen in some patients to steroids tends to support the latter. We report a case of severe IC associated with Sjogren’s syndrome in whom bladder symptoms responded to a course of steroids where other conventional management did not help. We believe that a high index of suspicion for the association of these two diseases would help treat some of the refractory cases of IC with a trial of corticosteroids.

Key Words: Interstitial Cystitis, Sjogren’s syndrome

 

Introduction

Interstitial Cystitis (IC) is a syndrome of unknown aetiology characterised by bladder and/or pelvic pain and irritable voiding symptoms without evidence of overt infection. It is recognised that IC is sometimes associated with a number of diseases such as allergies, Irritable bowel Syndrome, Fibromyalgia, Inflammatory Bowel Disease, Systemic Lupus Erythematosus and Sjogren’s syndrome. Studies have shown a strong association between IC and Sjogren’s syndrome. This association is much stronger than was generally recognised. We report a case supporting this observation and stress on the need to have a high index of suspicion for this combination in all patients with IC.

 

Case Report

A 50-year-old male presented with rapid onset of urinary frequency, urgency, dysuria, nocturia (often as much as 6 to 8 times) and reduced flow rate. He was diagnosed in the past to have Sicca Syndrome having suffered from dry gritty eyes and dry mouth. Schirmer’s test had confirmed dry eyes. It was interesting to note that the patient had been suffering from frequency of micturition and nocturia for some time but had put it down to excessive water consumption, associated with his dry mouth. He had no signs of a concurrent connective tissue disorder and had a negative Rheumatoid factor, ANA and ESR. He was placed on antibiotics by his GP for clinically suspected UTI.  However multiple urine cultures were negative for infection. Cystoscopy done initially revealed an inflamed bladder and biopsies confirmed severe inflammation along with the presence of numerous plasma cells (Figure 2 & 3). Intravesical Sodium Hyaluronate infusions over a 3 month period were tried. These did not help and the patient had worsening symptoms that greatly affected his family and professional life.

 

He was brought in once again for a cystourethroscopy, which revealed bladder appearance typical of Interstitial Cystitis with Glomerulations and Hunner type Ulcers (Figure 1). Repeat bloods for immunological markers revealed a positive titre for Anti Nuclear Antibody and elevated Rheumatoid Factor.

 

Having already had an unsuccessful course of Sodium Hyaluronate Infusion he was commenced on oral steroids. Patient improved and was symptomatically better on discharge. Patient has since been reviewed by an Immunologist and has been commenced on Azathioprine and has had Intravesical Dimethylsulfoxide installation. Patient has been symptomatically better on follow up.

 

Discussion

IC is a Chronic Inflammatory Bladder disease .It was first described in1915, when Hunner described it as a ‘rare type of bladder ulcer in women’.4 Despite intensive research its prevalence, aetiology, diagnosis and appropriate treatment remain elusive.5 Although aetiology is poorly understood, autoimmune mechanism is implicated as one of the causes of IC.8 90% of IC occurs in women and prevalence is reported to be 52-67 cases per 100,000 women.11 Its impact on quality of life, loss of work time, associated depression and anxiety are only now becoming apparent.10 Fister1 first reported a case of IC in a patient with SLE in 1938, linking IC with an autoimmune disease. In 1993, Van De Merwe et al12 studied 10 patients with IC, 2 of who were diagnosed with Sjogrens syndrome. Further studies at Rotterdam showed a strong association between IC and Sjogren’s syndrome.13 In this study 28% of IC patients had definite or probable Sjogren’s syndrome. This association was much stronger than generally recognised. This study also showed the increased expression of certain growth factor (PD –ECGF, FGF & VEGF) in the bladder of patients with IC.In addition the expression of CD 44 was higher in ulcer type IC than in non-ulcer IC.Further studies by Ochs RL et al7 of 96 IC patients have shown almost 36% to be positive for ANA at a titre of 1/40 or greater.

 

The prevalence of various European criteria’s for Sjogren’s syndrome was studied in 100 patients with IC.14 The prevalence was Ocular symptoms 68%, Oral symptoms 60%, Abnormal ocular test 16%, Abnormal salivary histology 16% and Antibody to SS-A/Ro or SS-B/La 12%.

 

 It also appears that systemic and autoimmune disorders are more prevalent in the IC population than the general population with allergy being the most common IC associated condition.9 Further studies in Finland have showed patients with Sjogren’s syndrome having symptoms of lower urinary tract with urinary frequency (27%) and suprapubic pain (36%) being the most common symptoms. This supports the concept that autoimmune disorders also have bladder affection.2 Thus the question has always been whether the bladder symptoms in patients with an autoimmune disorder and associated IC are due to the association between these two-disease processes and, rather a manifestation of autoimmune involvement of the bladder. The beneficial response of cystitis of autoimmune aetiology to steroids tends to support the latter.6 Studies have showed when severe IC is associated with Sjogren’s syndrome a therapeutic trial of steroid and cyclosporin may be beneficial.11 Horiki et al demonstrated this beneficial effect of steroids in a case of SLE with various central and peripheral neurogenic disorders presenting with motor paralytic bladder as a major manifestation.3

 

However it must be understood that there is no single treatment that is effective in treating IC. Most patients however can be maintained in a satisfied, although definitely not asymptomatic state, punctuated by exacerbations and remissions.15

 

Conclusion

Interstitial Cystitis can be a debilitating disease. Its association with autoimmune disorders needs to be recognised, as there is a beneficial effect of steroids in treatment of patients with Interstitial Cystitis in association with an autoimmune disorder.

 

References

  1. Fister GM. Similarity of IC (Hunner’s Ulcer) to Lupus erythematosus. J Urol 40:37,1938

  2. Haarala M et al: Lower Urinary Tract Symptoms in patients with Sjogren’s syndrome and SLE. International Urogynecology Journal. 11(2): 84-6, 2000

  3. Horiki et al: A case of SLE with various central and peripheral neurological disorders presenting with motor paralytic bladder as a major manifestation.Ryumachi.1995 Oct; 35(5): 821-6

  4. Hunner GL. A rare type of bladder ulcer in women: Report of 2 cases Boston med Surg J 172:660,1916

  5. Leppilahti M et al: Prevalence of symptoms related to IC in women: a population based study in Finland. Journal of Urology. 168(1): 139-43, 2002 July

  6. Meulder et al: Association of Chronic IC, Protein losing enteropathy and paralytic Ileus with Seronegative SLE. Clin Neph 1992: 37: 239-244

  7. Ochs RL et al: Auto antibodies in IC. J Urol 1994 Mar; 151(3): 587-92

  8. Oravisto KJ: Interstitial cystitis as an autoimmune disease. Eur Urol 1980; 6:10-13

  9. Peeker et al: Intercurrent autoimmune condition in classic and non ulcer IC: Scandinavian Journal of Urology & Nephrology: 31) 60-3,2003

  10. Shea 0’Malley et al: Quality of life in IC.Urol Intergrada Invest 1999,4:303-306

  11. Shibata et al: Severe IC associated with Sjogren’s syndrome. Internal Medicine. 43(3): 248-52, 2004 March.

  12. Van de Merwe et al : Sjogren’s syndrome in patients with IC. J Rheumatol 20:962-966,1993

  13. Van de Merwe et al: Systemic aspect of IC, immunology and linkage with autoimmune disorders. International Journal of Urology. 10 suppl: S35-8, 2003 October

  14. Van de Merwe. IICPN Publ Nr 2; 2004

  15. Wein et al: IC current and future approaches to diagnosis and treatment. Urol Clin North Am 1994:21:153-161

 

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