Primary localized amyloidosis of the urinary bladder: a case series of 31 patients.

OBJECTIVE: To evaluate the presentation and prognosis of primary localized amyloidosis of the urinary bladder. PATIENTS AND METHODS: The medical records of 31 patients with primary localized amyloidosis of the urinary bladder were reviewed. Immunohistochemical amyloid typing was performed on bladder biopsy specimens from 27 patients. RESULTS: The median age of the 22 men and 9 women was 55 years. Twenty-four patients (77%) presented with gross hematuria (associated with irritative urinary tract symptoms in 6 patients), and 7 (23%) had only irritative lower urinary tract symptoms. Multiple bladder areas were involved in 20 patients (65%), a single area was involved in 8 (26%), and diffuse involvement was present in 3 (10%). Twenty-four patients had immunoglobulin light chain, and 3 had transthyretin-related amyloid. Local recurrences were common. None of the patients developed systemic amyloidosis. CONCLUSION: Primary localized amyloidosis of the urinary bladder can be easily confused with a neoplasm. Immunohistochemical amyloid typing is important. Transthyretin-related amyloid of the bladder requires no further work-up. Repeated work-ups for systemic amyloidosis are unnecessary for patients with light chain-related amyloidosis of the urinary bladder. Early eradication with fulguration or laser therapy is indicated. Cystoscopic follow-up is necessary.

Use of methotrexate for autoimmune hearing loss.

To assess the efficacy of low-dose methotrexate (MTX) given long-term for the treatment of autoimmune hearing loss, we performed a prospective open-label study of 11 patients with treatment-refractory autoimmune hearing loss. All patients had ongoing episodic worsening of hearing in 1 or both ears before enrollment despite traditional medical therapy. The MTX dose was 7.5 to 17.5 mg/wk. Hearing loss and vertigo were evaluated at baseline and at completion of the study. Hearing improvement was defined as an improvement in the pure tone threshold (PT) average of >10 dB or an increase in speech discrimination (SD) of >15%, whereas worsening was defined as a worsening of >10 dB in PT or a decrease of >15% in SD in at least 1 ear. The MTX was well tolerated. Among the 6 patients with Meniere's disease. 4 had improvement or resolution of vertigo, while 2 had no improvement. Disequilibrium improved in all 3 patients with Cogan's syndrome. According to the parameters defined above, hearing improved in 9 patients (82%), was unchanged in 1 patient (9%), and worsened in 1 patient (9%). Long-term low-dose MTX therapy may be a useful therapy for some patients who have hearing loss with a presumptively autoimmune-mediated component that is refractory to traditional therapies.