Dysphonia: differential diagnosis amyloidosis

Introduction:

Dysphonia is one of the most frequent causes for ENT consultation. Organic causes like voice overload, inflammation and tumors as well as functional disorders have to be considered. Isolated amyloidosis of the larynx and trachea is a rare organic cause of dysphonia.

Case report:

A 66-years old female patient visits a phoniatrics specialist complaining about progressive dysphonia with interspersed aphonia for 30 years. A chronic laryngitis is assumed and treated conservatively without any improvements. Under further ENT investigation, a swelling of the posterior trachea is found during microlaryngoscopy and a biopsy is performed. AL-amyloidosis (type kappa light chain) is diagnosed after the histological examination of the tissue samples. Systemic amyloidosis is ruled out after fatty tissue aspiration in a specialized centre.

Results/Conclusions:

Local AL-amyloidosis should be considered as a possibility in cases with unclear dysphonia. The origin is unknown. Since the faulty light chains are produced by local clonal plasma cells, recurrent and chronic infections can be part of the pathomechanism and can also cause a progress of the disease.

Biopsy is the diagnostic of choice. MRI-imaging can be used for progress control. Systemic amyloidosis as well as it's related diseases like monoclonal gammopathy of unknown significance (MGUS) and myeloma have to be ruled out.

In addition to treating respiratory infections early and consistently, symptomatic masses can be treated by CO2 laser resection. The effect of steroids is disputed, since it does not affect the amyloid plaques

Publication History

Publication Date:
23 April 2019 (online)