Eosinophilic cystitis | |
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Other names | EoC, EC |
Eosinophilic cystitis showing edematous and chronically inflamed lamina propria with numerous eosinophils. | |
Specialty | Urology |
Eosinophilic cystitis is a rare type of interstitial cystitis first reported in 1960 by Edwin Brown. [1] Eosinophilic cystitis has been linked to a number of etiological factors, including allergies, bladder tumors, trauma to the bladder, parasitic infections, and chemotherapy drugs, though the exact cause of the condition is still unknown. The antigen-antibody response is most likely the cause of eosinophilic cystitis. This results in the generation of different immunoglobulins, which activate eosinophils and start the inflammatory process. [2]
The most typical symptom complex includes dysuria, hematuria, frequency, and suprapubic pain. For diagnosis, cystoscopy and biopsy are considered the gold standard. Peripheral eosinophilia, which is rare in patients, microscopic hematuria, proteinuria, and other laboratory findings corroborate the diagnosis. [2]
Treatment for eosinophilic cystitis is still up for debate. Antihistamines, antispasmodics, leukotriene antagonists, immunosuppressives, oral and intravesical corticosteroids, and in extreme situations, surgery have all been used in the treatment of symptoms. [3]
The most prevalent symptoms of eosinophilic cystitis include frequency, dysuria, hematuria, and suprapubic pain. Other less typical signs and symptoms are retention of urine and nocturia. [4] Uncommon presentations documented in the literature consist of skin rash in a patient treated with mitomycin-C, [5] pneumaturia resulting from an enterovesical fistula in a patient experiencing chromic catgut suture allergy, [6] and gastrointestinal symptoms due to eosinophilic gastroenteritis. [7]
It has been noted that 2% of patients with superficial transitional cell carcinoma have bladder eosinophilia. In comparison to people without bladder eosinophilia, this has been shown to be predictive of a statistically lower tumor recurrence rate. [8] This phenomenon could be the reason for patients who have had intravesical chemotherapy or transitional cell carcinoma in the past and have underlying eosinophilic infiltration. [9]
Patients with eosinophilic cystitis typically have unremarkable physical examination results. Palpation of the abdomen typically reveals tenderness in patients who are in pain. [10] However, there have been a number of case reports of individuals who arrived with a mass in their lower abdomen. [11] [12]
According to one study, the most frequent complication—which affected 27% of the patients—is dilation of the upper urinary tract. [4] The site of stenosis was the ureterovesical junction in most of the patients with eosinophilic ureteritis. [13]
Fibrosis of the bladder wall and reduced bladder compliance can compress the intramural portion of the ureter in eosinophilic cystitis. [14] When bilateral, this blockage may result in renal insufficiency [15] or a (temporary) non-functioning kidney. [16]
Patients may need blood transfusions if hematuria is severe [17] or complicated by a bleeding disorder. [18]
Numerous etiologies and connections to other illnesses have been postulated. These include various drugs, [14] intestinal damage, [19] persistent intestinal irritation, wound healing after intestinal surgery, parasitosis, [20] food and drug allergies, [21] UTIs, [22] urothelial cancer, [23] autoimmune diseases, and eosinophilic enteritis. [7]
The disease's pathogenesis and cause are not entirely understood. It is hypothesized that when the bladder is exposed to antigens, an antigen-antibody complex forms. This IgE-mediated reaction triggers mast cell degranulation, which draws eosinophils and results in an inflammatory reaction that damages tissue. [24]
Microscopic hematuria and proteinuria are frequently seen on urinalysis. Typically, urine cultures are negative. [4] About 50% of patients with a history of allergies or atopy also have peripheral eosinophilia. Giemsa and Wright's stain can be used to identify eosinophils in urine. [2] In clinical settings, eosinophiluria linked to eosinophilic cystitis is uncommon because mucosal shedding from the urothelium occurs sparingly or eosinophils are broken down quickly. [9]
Tests for renal function are typically normal, but ureteric obstruction from intramural ureter fibrosis may cause impairment. [14] An intravenous urogram may reveal hydroureteronephrosis, bilateral or unilateral, [15] and abnormalities in the bladder and ureters, or it may be normal. [25]
An ultrasonography examination may reveal tumor-like masses and irregular thickening of the bladder wall, or it may be normal. Patients with eosinophilic cystitis who have bladder masses have been reported to have undergone other tests, such as cystograms, CT scans, and MRIs, but they do not exhibit any characteristic features. [4]
Cystoscopy typically reveals large mucosal edema along with erythematous, polypoid, velvety red lesions. It can be mistaken for vesical rhabdomyosarcoma in children. [25] Eosinophilic cystitis is hard to differentiate from other cystitis types (like interstitial and tuberculous cystitis), neoplastic lesions (like carcinoma in situ), and other bladder cancers. Therefore, in order to confirm the diagnosis of eosinophilic cystitis, biopsies are required. [2] Transmural inflammation is present histologically, primarily with eosinophils. The lamina propria has more severe edema and inflammation. A contracted bladder may result from focal muscle necrosis and varying degrees of detrusor muscle fibrosis. [26]
Mostly conservative measures are used to treat eosinophilic cystitis. Due to the condition's variable natural history, chronicity, and recurrence risk, patients with eosinophilic cystitis require ongoing monitoring that includes pertinent blood tests, urine examinations, appropriate imaging, and cystoscopies. [27]
Removing the causing factor has a good cure rate in patients with eosinophilic cystitis secondary to allergic reactions, tranilast, or mitomycin-C. Non-steroidal anti-inflammatory drugs (NSAID) and antihistamines have been utilized as the primary management with good results when no apparent cause of eosinophilic cystitis is found in patients. [28]
Particularly for patients suffering from eosinophilic cystitis linked to UTIs, antibiotics are beneficial. Corticosteroids have proven to be an effective treatment for patients who have not responded to NSAIDs and antihistamines. By improving the lysosomal membrane's stability, these lessen bladder inflammation. Even with steroid treatment, eosinophilic cystitis symptoms may not go away. [29]
Additional therapies mentioned include silver nitrate, cyclosporin-A, [30] and intravesical dimethylsulfoxide. [31] The majority of patients have had the bladder lesion excised transurethrally, with good results. A small number of patients with progressive eosinophilic cystitis who are not improving with medication or transurethral resection may be candidates for more invasive surgeries like augmentation ileocystoplasty [32] or partial or total cystectomy. [17]
Interstitial cystitis (IC), a type of bladder pain syndrome (BPS), is chronic pain in the bladder and pelvic floor of unknown cause. It is the urologic chronic pelvic pain syndrome of women. Symptoms include feeling the need to urinate right away, needing to urinate often, and pain with sex. IC/BPS is associated with depression and lower quality of life. Many of those affected also have irritable bowel syndrome and fibromyalgia.
Eosinophils, sometimes called eosinophiles or, less commonly, acidophils, are a variety of white blood cells and one of the immune system components responsible for combating multicellular parasites and certain infections in vertebrates. Along with mast cells and basophils, they also control mechanisms associated with allergy and asthma. They are granulocytes that develop during hematopoiesis in the bone marrow before migrating into blood, after which they are terminally differentiated and do not multiply. They form about 2 to 3% of white blood cells in the body.
Eosinophilia is a condition in which the eosinophil count in the peripheral blood exceeds 5×108/L (500/μL). Hypereosinophilia is an elevation in an individual's circulating blood eosinophil count above 1.5 × 109/L (i.e. 1,500/μL). The hypereosinophilic syndrome is a sustained elevation in this count above 1.5 × 109/L (i.e. 1,500/μL) that is also associated with evidence of eosinophil-based tissue injury.
Glomerulation refers to bladder hemorrhages which are thought to be associated with some types of interstitial cystitis (IC).
Mycoplasmataceae is a family of bacteria in the order Mycoplasmatales. This family consists of the genera Mycoplasma and Ureaplasma.
Bladder exstrophy is a congenital anomaly that exists along the spectrum of the exstrophy-epispadias complex, and most notably involves protrusion of the urinary bladder through a defect in the abdominal wall. Its presentation is variable, often including abnormalities of the bony pelvis, pelvic floor, and genitalia. The underlying embryologic mechanism leading to bladder exstrophy is unknown, though it is thought to be in part due to failed reinforcement of the cloacal membrane by underlying mesoderm. Exstrophy means the inversion of a hollow organ.
Eosinophilic esophagitis (EoE) is an allergic inflammatory condition of the esophagus that involves eosinophils, a type of white blood cell. In healthy individuals, the esophagus is typically devoid of eosinophils. In EoE, eosinophils migrate to the esophagus in large numbers. When a trigger food is eaten, the eosinophils contribute to tissue damage and inflammation. Symptoms include swallowing difficulty, food impaction, vomiting, and heartburn.
Eosinopenia is a condition where the number of eosinophils, a type of white blood cell, in circulating blood is lower than normal. Eosinophils are a type of granulocyte and consequently from the same cellular lineage as neutrophils, basophils, and mast cells. Along with the other granulocytes, eosinophils are part of the innate immune system and contribute to the defense of the body from pathogens. The most widely understood function of eosinophils is in association with allergy and parasitic disease processes, though their functions in other pathologies are the subject of ongoing research. The opposite phenomenon, in which the number of eosinophils present in the blood is higher than normal, is known as eosinophilia.
In male anatomy, the lacuna magna is the largest of several recesses in the roof of the navicular fossa of the male urethra.
Urachal cancer is a very rare type of cancer arising from the urachus or its remnants. The disease might arise from metaplastic glandular epithelium or embryonic epithelial remnants originating from the cloaca region.
Amelanotic melanoma is a type of skin cancer in which the cells do not make any melanin. They can be pink, red, purple or of normal skin color, and are therefore difficult to diagnose correctly. They can occur anywhere on the body, just as a typical melanoma can.
Chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS), previously known as chronic nonbacterial prostatitis, is long-term pelvic pain and lower urinary tract symptoms (LUTS) without evidence of a bacterial infection. It affects about 2–6% of men. Together with IC/BPS, it makes up urologic chronic pelvic pain syndrome (UCPPS).
Urologic diseases or conditions include urinary tract infections, kidney stones, bladder control problems, and prostate problems, among others. Some urologic conditions do not affect a person for that long and some are lifetime conditions. Kidney diseases are normally investigated and treated by nephrologists, while the specialty of urology deals with problems in the other organs. Gynecologists may deal with problems of incontinence in women.
Allopurinol hypersensitivity syndrome(AHS) typically occurs in persons with preexisting kidney failure. Weeks to months after allopurinol is begun, the patient develops a morbilliform eruption or, less commonly, develops one of the far more serious and potentially lethal severe cutaneous adverse reactions viz., the DRESS syndrome, Stevens Johnson syndrome, or toxic epidermal necrolysis. About 1 in 1000 patients receiving allopurinol are affected, and mortality rates have been reported to be between 20% and 25%.
Cystitis cystica is an uncommon chronic reactive inflammatory disease that is believed to be brought on by a tumor, calculi, infection, or obstruction of the urothelium. Cystitis glandularis is a proliferative progression of cystitis cystica that is distinguished by urothelial glandular metaplasia.
Ectopic testis is used to describe the testis leaving the inguinal canal and entering a site other than the scrotum. Usually, it results from obstruction of the scrotal entrance or from overdevelopment and lengthening of a segment of the gubernaculum.
Lupus vasculitis is one of the secondary vasculitides that occurs in approximately 50% of patients with systemic lupus erythematosus (SLE).
Gaetano Ciancio is an Italian American surgeon at the University of Miami who specializes in kidney transplant. He is the chief medical and academic officer of the Miami Transplant Institute and the director of its Kidney & Kidney-Pancreas Programs. His most significant contributions to medicine are related to surgically treating kidney cancer once it has spread to the inferior vena cava and in optimizing the immunosuppression protocol after kidney transplant.
Urologic chronic pelvic pain syndrome (UCPPS) is ongoing bladder pain in either sex, chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS) in men and interstitial cystitis or painful bladder syndrome (IC/PBS) in women.
Reginald Wyndham Lloyd-Davies was a British urologist and head of clinical urology at St Thomas' Hospital, London, who completed early studies of the bladder epithelium with a scanning electron microscope.