Rare Nephrology News

Disease Profile

Eosinophilic cystitis

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

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Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Summary

Eosinophilic cystitis (EC) is a rare inflammatory bladder condition caused by the build up of eosinophils in the bladder. The exact cause of this condition is not known. However, EC has been found in those with allergies and asthma, and in those with a history of bladder trauma or infection, open bladder surgery, or surgery for a bladder tumor. EC has also been found in those who take certain medications.[1][2]

Symptoms

Common symptoms include urinary frequency, painful urination, blood in the urine, and abdominal/pelvic pain. Other less common symptoms include excessive urination during the night and urinary retention. Many of the symptoms commonly seen in EC mimic symptoms seen in other conditions. Rare features include gastrointestinal symptoms because of eosinophilic gastroenteritis, skin rash, and the passage of air in the urine (pneumaturia). EC has been observed in 2% of patients with superficial transitional cell carcinoma (bladder cancer).[1]

Cause

The cause of eosinophilic cystitis remains unclear, although it has been associated with allergies, reactions to certain medications (antibiotics, chemotherapy), bladder trauma, bladder tumors, and parasitic infections.[1][2]

Treatment

Any possible causative factors, should be avoided or discontinued, such as certain medications (tranilast, mitomycin C). Non-steroidal anti-inflammatory drugs (NSAIDs), such as aspirin or ibuprofen, and antihistamines have been used as the primary management of choice with good results. Antibiotics are helpful, especially in those individuals with EC associated with urinary tract infections. Those who do not respond to NSAIDs and antihistamines have been treated with corticosteroids. Corticosteroids and antihistamines are used because they suppress the inflammatory reaction in the bladder.[3] However, symptoms of EC may persist, despite steroid therapy. If symptoms persist after steroid therapy, azathioprine or cyclosporine can be tried.[3] Other treatments include intravesical dimethylsulfoxide, cyclosporin-A (immunosuppressant drug), and silver nitrate.[1]

In those with bladder lesions, surgery has been successful. Few patients with progressive EC not responding to medical therapy or surgery may be considered for more radical procedures, such as partial/total cystectomy (surgical removal of all or part of the bladder).[1]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      In-Depth Information

      • PubMed is a searchable database of medical literature and lists journal articles that discuss Eosinophilic cystitis. Click on the link to view a sample search on this topic.

        References

        1. Teegavarapu PS, Sahai A, Chandra A, Dasgupta P, Khan MS. Eosinophilic cystitis and its management. Int J Clin Pract. 2005; 59(3):356-60. https://www.ncbi.nlm.nih.gov/pubmed/15857336. Accessed 10/31/2014.
        2. Eosinophilic Cystitis. American Partnership for Eosinophilic Disorders. September 8, 2011; https://apfed.org/drupal/drupal/eosinophilic_cystitis. Accessed 8/20/2014.
        3. van den Ouden D. Diagnosis and management of eosinophilic cystitis: a pooled analysis of 135 cases. Eur Urol. 2000; 37(4):386-94. https://www.ncbi.nlm.nih.gov/pubmed/10765067. Accessed 10/31/2014.

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