Association of Elmiron (pentosanpolysulphate sodium) with pigmented maculopathy: An update for urologists and patients

2021 ◽  
pp. 205141582110536
Author(s):  
Mithun Kailavasan ◽  
Jonathan Charles Goddard
Keyword(s):  
Treatment Options ◽  
Pain Syndrome ◽  
Evidence Base ◽  
Bladder Pain Syndrome ◽  
Good Evidence ◽  
Current Evidence ◽  
Exact Nature ◽  
Level Of Evidence ◽  
Bladder Pain ◽  
Number Of Treatment

Introduction: Bladder pain syndrome (BPS) is a difficult disorder to diagnose and manage with a limited number of treatment options that have a good evidence base. Elmiron (pentosanpolysulphate sodium (PPS)) is recognised as a main second-line treatment option in many international guidelines. Recently published retrospective studies suggest that there is an association between Elmiron and pigmented maculopathy, a rare disease associated with visual impairment. Objectives: To review the known literature on Elmiron and pigmented maculopathy and alternative oral pharmacotherapy options for BPS. To promote awareness on this topic and aid urologists in managing Elmiron prescription. Methods: We reviewed the most commonly used oral treatments for BPS, in particular Elmiron, the current evidence to support this association with eye disease and its position in the management pathway of this complex condition. Conclusions: Recent retrospective longitudinal studies have demonstrated an association of Elmiron with pigmented maculopathy. However, as yet the exact nature of this relationship is unknown. Nevertheless, Elmiron remains an important part of the treatment pathway for BPS. We suggest clinicians should fully inform patients of this association, including patients who have been previously comenced on Elmiron. There should be ongoing and continuing reviews of all treatments to review its efficacy in indiviual patients. Level of evidence: Not applicable

scholarly journals Current standard of care in treatment of bladder pain syndrome/interstitial cystitis

2021 ◽  
Vol 13 ◽  
pp. 175628722110224
Author(s):  
Sabela Rodriguez Lopez ◽  
Naşide Mangır
Keyword(s):  
Interstitial Cystitis ◽  
Cell Activation ◽  
Treatment Options ◽  
Pain Syndrome ◽  
Bladder Pain Syndrome ◽  
Mast Cell Activation ◽  
Relaxation Techniques ◽  
Level Of Evidence ◽  
Bladder Pain ◽  
Cardinal Symptom

Bladder pain syndrome/interstitial cystitis (BPS/IC) is a debilitating, systemic pain syndrome with a cardinal symptom of bladder related pain with associated systemic symptoms. It is characterized by an inflammation that partially or completely destroys the mucus membrane and can extend into the muscle layer; however, the etiology and pathogenesis is still enigmatic. It has been suggested that mast cell activation, defects in the glycosaminoglycan layer, non-functional proliferation of bladder epithelial cells, neurogenic inflammation, microvascular abnormalities in the submucosal layer, autoimmunity and infectious causes may cause BPS/IC. Available treatment options include general relaxation techniques, patient education, behavioral treatments, physical therapy, multimodal pain therapy, oral (amitriptyline, cimetidine, hydroxyzine) and intravesical treatments (heparin, lidocaine, hyaluronic acid and chondroitin sulfate), hydrodistension and other more invasive treatments. Available treatments are mostly not based on a high level of evidence. Lack of understanding of disease mechanisms has resulted in lack of targeted therapies on this area and a wealth of empirical approaches with usually inadequate efficacy. The aim of this article is to review the available evidence on the pathophysiological mechanisms of BPS/IC as they relate to available treatment options.

Bladder pain syndrome

2017 ◽  
Author(s):  
Magnus Fall
Keyword(s):  
Treatment Options ◽  
Correct Diagnosis ◽  
Pain Syndrome ◽  
Bladder Pain Syndrome ◽  
Clinical Syndrome ◽  
Personal Life ◽  
Medical Community ◽  
Bladder Pain ◽  
Profound Impact ◽  
Medical Consultations

Bladder pain syndrome has a profound impact on the patients’ physical, professional, and personal life. Generally, recognition of this clinical syndrome is hampered by insufficient expertise in the medical community. The correct diagnosis is often delayed by several years and may be preceded by multiple medical consultations and treatment attempts. Still, this is one of the most painful, non-malignant conditions to be encountered in urology and it is probably more common than generally believed. Problems as to diagnosis, phenotyping, terminology, and management persist, but during the last few years there has been increasing activity to resolve those issues. In this chapter, symptoms, findings, and treatment options are outlined.

scholarly journals Optimising the management of bladder pain syndrome

2020 ◽  
pp. 205141582095473
Author(s):  
Waleed Al-Singary ◽  
Reena Patel ◽  
Ujjal Sarkar ◽  
Hiten RH Patel
Keyword(s):  
Pain Control ◽  
Symptomatic Relief ◽  
Pain Syndrome ◽  
Skin Lesions ◽  
Bladder Pain Syndrome ◽  
Pain Clinic ◽  
Level Of Evidence ◽  
Bladder Pain ◽  
Irritable Bowel ◽  
Good Pain Control

Objective: Clinicians have shown variable practice in the diagnosis and management of bladder pain syndrome (BPS). This study assessed pain localisation sites, common co-morbidities, investigations and treatment patterns in clinical practice. Patients and methods: We performed a retrospective analysis of 412 patients attending our pelvic pain clinic between 2004 and 2016. Frequency counts were used to summarise findings. Results: Pain in women ( N=388) was localised to the lower abdomen (92.0%), lower back (71.1%) and vagina (60.8%). Men ( N=24) typically presented with testicular pain with painful ejaculation (70.8%). Nearly all (95.4%) patients reported sexual dysfunction. Visceral neuropathic pain and autoimmune co-morbidities, such as irritable bowel syndrome, chronic headaches or migraines and skin lesions, were more prevalent in our cohort than in the general population. All patients had urine culture and sensitivities and flexible cystoscopy. Laparoscopy, urodynamic studies and bladder biopsies were mostly normal, but were essential in excluding other pathologies. Good pain control was achieved on amitriptyline (83.0%). Hydro-distension and benign bladder ulcer cauterisation provided temporary symptomatic relief. Conclusion: This study demonstrates that patient expectation management and education is essential in BPS, with most achieving good pain control with conservative measures and amitriptyline. Those receiving intravesical treatments will most likely require subsequent revisions. Level of evidence: Not applicable for this single-centre audit.

Chronic pelvic pain in benign and functional urological conditions: A review and comparison of the UK and European guidelines

2018 ◽  
Vol 11 (2) ◽  
pp. 115-121 ◽  
Author(s):  
Sophia Cashman ◽  
Suzanne Biers
Keyword(s):  
Pelvic Pain ◽  
Chronic Pelvic Pain ◽  
Chronic Pelvic Pain Syndrome ◽  
Pain Syndrome ◽  
Bladder Pain Syndrome ◽  
Current Evidence ◽  
Pelvic Pain Syndrome ◽  
Bladder Pain ◽  
European Guidelines ◽  
Relative Paucity

We aim to provide a general overview of the available UK and European guidelines on non-oncological causes of chronic pelvic pain, and highlight any differences in practice. We have reviewed the current guidelines on chronic pelvic pain syndrome (defined as chronic pelvic pain with no identified underlying cause and/or the pain is non-specific or involves more than one organ) and other specific organ pain syndromes particularly relevant to urological clinical practice, including prostate pain syndrome, bladder pain syndrome and gynaecological causes of chronic pelvic pain. We have identified a relative paucity of UK guidelines, and accept that the European Association of Urology provides a comprehensive and current evidence based reference and guide which is utilised and regarded by most urologists as the ‘gold standard’ in UK practice.

Challenges and current evidence on the management of bladder pain syndrome

2013 ◽  
Vol 33 (8) ◽  
pp. 1193-1201 ◽  
Author(s):  
Eleftheria L. Chrysanthopoulou ◽  
Stergios K. Doumouchtsis
Keyword(s):  
Pain Syndrome ◽  
Bladder Pain Syndrome ◽  
Current Evidence ◽  
Bladder Pain

Clinical and histological findings in bladder pain syndrome/interstitial cystitis: The implication of time of symptoms

2018 ◽  
Vol 11 (4) ◽  
pp. 248-253
Author(s):  
Daniele Porru ◽  
Valentina Bobbi ◽  
Carmelo Di Franco ◽  
Alessandra Viglio ◽  
Mattia Novario ◽  
...  
Keyword(s):  
Interstitial Cystitis ◽  
Symptom Onset ◽  
Bladder Wall ◽  
Pain Syndrome ◽  
Bladder Pain Syndrome ◽  
Detrusor Muscle ◽  
Level Of Evidence ◽  
Bladder Pain ◽  
Bladder Biopsy ◽  
The Impact

Objective: To find out whether a correlation exists between denudation of urothelium and time of symptom onset in patients with bladder pain syndrome/interstitial cystitis (BPS/IC), and to search for a correlation between the impact of symptoms. Patients and methods: Fifty-seven consecutive patients underwent cystoscopy under anaesthesia to classify those cases suspected of being affected with BPS/IC. The time elapsed between onset of symptoms and diagnosis at the time of bladder biopsy was also defined as BPS/IC duration. Bladder biopsies were taken including detrusor muscle, three deep cold biopsies of posterior, anterior and lateral bladder wall. Results: Statistical analysis showed significant correlation between BPS/IC duration and the presence of Hunner’s lesions ( P<0.023). Hunner’s lesion with cystoscopy and histological evidence of urothelial denudation with bladder biopsy appear to be related to BPS/IC duration. Thus an early diagnosis allows an appropriate therapeutic approach to be started to prevent a more severe evolution of this multifaceted painful syndrome. Conclusions: Our study shows a correlation between the time of symptom onset and evidence of urothelial denudation and with detrusor mast cell count in the whole group of patients. BPS/IC duration did not seem to correlate with the severity of symptoms, but rather with the presence of associated diseases. Level of evidence: Not applicable for this multicentre audit.

scholarly journals Clinical Remission Using Personalized Low-Dose Intravenous Infusions of N-acetylcysteine with Minimal Toxicities for Interstitial Cystitis/Bladder Pain Syndrome

2021 ◽  
Vol 11 (5) ◽  
pp. 342
Author(s):  
Dipnarine Maharaj ◽  
Gayathri Srinivasan ◽  
Sarah Makepeace ◽  
Christopher J. Hickey ◽  
Jacqueline Gouvea
Keyword(s):  
Adverse Events ◽  
Interstitial Cystitis ◽  
Clinical Remission ◽  
Low Dose ◽  
Treatment Options ◽  
Pain Syndrome ◽  
Bladder Pain Syndrome ◽  
Infusion Therapy ◽  
Blurred Vision ◽  
Bladder Pain

Interstitial Cystitis or Bladder Pain Syndrome (IC/BPS) is a heterogeneous condition characterized by elevated levels of inflammatory cytokines, IL-1β, IL-6, IL-8, IL-10, TNF-α, and is associated with debilitating symptoms of pelvic pain and frequent urination. A standard of care for IC/BPS has not been established, and most patients must undergo a series of different treatment options, with potential for severe adverse events. Here, we report a patient with a 26-year history of IC/BPS following treatment with multiple therapies, including low doses of etodolac, amitriptyline and gabapentin, which she was unable to tolerate because of adverse effects, including headaches, blurred vision and cognitive impairment. The patient achieved a complete clinical remission with minimal adverse events after 16 cycles of N-acetylcysteine (NAC) intravenous (IV) infusions over a period of 5 months, and pro-inflammatory cytokine levels were reduced when compared to measurements taken at presentation. Personalized low dose NAC IV infusion therapy represents an effective, safe, anti-inflammatory therapy administered in the outpatient setting for IC/BPS, and warrants further investigation.

scholarly journals Biomarkers in the Light of the Etiopathology of IC/BPS

Diagnostics ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 2231
Author(s):  
Jochen Neuhaus ◽  
Mandy Berndt-Paetz ◽  
Andreas Gonsior
Keyword(s):  
Treatment Options ◽  
Pain Syndrome ◽  
Genetic Alterations ◽  
Bladder Pain Syndrome ◽  
Virus Infections ◽  
Bladder Pain ◽  
Chronic Urinary Tract Infection ◽  
New Findings ◽  
Close Relationship ◽  
Urinary Microbiome

In this review, we focused on putatively interesting biomarkers of interstitial cystitis/bladder pain syndrome (IC/BPS) in relation to the etiopathology of this disease. Since its etiopathology is still under discussion, the development of novel biomarkers is critical for the correct classification of the patients in order to open personalized treatment options, on the one hand, and to separate true IC/BPS from the numerous confusable diseases with comparable symptom spectra on the other hand. There is growing evidence supporting the notion that the classical or Hunner-type IC (HIC) and the non-Hunner-type IC (NHIC) are different diseases with different etiopathologies and different pathophysiology at the full-blown state. While genetic alterations indicate close relationship to allergic and autoimmune diseases, at present, the genetic origin of IC/BPS could be identified. Disturbed angiogenesis and impairment of the microvessels could be linked to altered humoral signaling cascades leading to enhanced VEGF levels which in turn could enhance leucocyte and mast cell invasion. Recurrent or chronic urinary tract infection has been speculated to promote IC/BPS. New findings show that occult virus infections occurred in most IC/BPS patients and that the urinary microbiome was altered, supporting the hypothesis of infections as major players in IC/BPS. Environmental and nutritional factors may also influence IC/BPS, at least at a late state (e.g., cigarette smoking can enhance IC/BPS symptoms). The damage of the urothelial barrier could possibly be the result of many different causality chains and mark the final state of IC/BPS, the causes of this development having been introduced years ago. We conclude that the etiopathology of IC/BPS is complex, involving regulatory mechanisms at various levels. However, using novel molecular biologic techniques promise more sophisticated analysis of this pathophysiological network, resulting in a constantly improvement of our understanding of IC/BPS and related diseases.

scholarly journals Mirabegron as adjuvant treatment for patients with interstitial cystitis/bladder pain syndrome

2017 ◽  
Vol 12 (3) ◽  
pp. E100-4 ◽  
Author(s):  
Michael Di Lena ◽  
Victoria Tolls ◽  
Kerri-Lynn Kelly ◽  
J. Curtis Nickel
Keyword(s):  
Interstitial Cystitis ◽  
Adjuvant Treatment ◽  
Treatment Options ◽  
Pain Syndrome ◽  
Bladder Pain Syndrome ◽  
Post Treatment ◽  
Symptom Scale ◽  
Symptom Index ◽  
Bladder Pain ◽  
Significant Difference

Introduction: Interstitial cystitis/bladder pain syndrome (IC/BPS) patients represent a heterogeneous group with pain and urinary storage symptoms and varying responses to current treatment options. The novel beta-3 agonist, mirabegron, has been shown to improve storage symptoms of patients with bladder overactivity; however, its effect on symptoms in the IC/BPS population has yet to be studied.Methods: Patients diagnosed at a single IC centre with IC/BPS undergoing standard therapy were treated with additional daily mirabegron 25 mg and seen in followup post-treatment. Patients completed the Interstitial Cystitis Symptom Index and Problem Index (ICSI/ICPI), and the Pelvic Pain and Urgency/Frequency Patient Symptom Scale (PUF) prior to and following mirabegron treatment. Global (NRS) and symptom-specific outcomes were assessed by comparing the pre- and post-treatment mean scores using tailed-t test (p<0.05 considered statistically significant).Results: A total of 23 patients were available for review pre- and post-mirabegron treatment. There was no significant difference in ICSI (p=0.448), ICPI (p=0.352), or PUF (p=0.869) pre- and post-treatment. Analysis of symptom-specific outcomes show statistically significant improvements in urgency (p=0.048); however, no statistically significant improvements in frequency (p=0.951) or pain (p=0.952) were observed with mirabegron therapy.Conclusions: IC/BPS patients treated with mirabegron had improvement of urinary urgency, but no significant benefit in terms of pain or urinary frequency. This data suggests that mirabegron’s role in the IC/BPS patient should be that of adjuvant treatment to ameliorate urgency.

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