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Introduction:Urothelial amyloidosis is a disease of proteinaceous deposits in the urinary tract, which can present with localized or systemic disease. Bladder disease typically presents with hematuria or irritative voiding symptoms. Our goal was to characterize the demographics and disease characteristics of patients diagnosed with urothelial amyloidsis at a major amyloid center.
Materials & Methods: We performed an IRB-approved, retrospective chart review of 34 patients with urothelial amyloidosis. Patient data was evaluated for age, presenting symptoms, ethnicity, disease location, localized versus systemic disease, and treatments.
Results: Our cohort included 34 patients [21 males (61.7%) and 13 females (38.2%)], with an average age of 62.7 years (range 42-84 years). 26 patients were Caucasian (76.4%), 2 were African-American (5.8%), 3 were Asian (8.8%), and 3 had no ethnicity documented. 26 patients had amyloid deposits only in the bladder (76.4%), 6 only in the ureter (17.6%), and 2 had both bladder and ureteral involvement (5.8%). 11 patients had systemic disease (32.3%) while 23 had only urothelial involvement (76.4%). 18 patients underwent transurethral resection/fulguration (TUR) (52.9%), while 2 underwent radiation (5.8%) and 3 had DMSO (8.8%). Importantly, 27 patients (79.4%) presented initially with urinary symptoms, leading to the diagnosis of amyloidosis.
Conclusions: Though hematuria and urinary symptoms are common presentations to urologists, they may be the initial symptoms of either localized or systemic amyloidosis. Patients diagnosed with urothelial amyloidosis should be further worked up for systemic disease. In addition, patients diagnosed with amyloid presenting with hematuria or urinary symptoms should be evaluated by a urologist.