Amyloidosis

This disease damages tissues by the accumulation of abnormal fibrillar substance. Amyloidosis is an idiopathic disease that may be either primary or secondary to multiple myeloma. The abnormal depositions may be local or diffuse, and may involve more than one organ system. The peak incidence is from 60 to 65 years, but it occurs as early as the third decade. Systemic disease is equally common in men and women, but there is a slight male predominance for disease localized in the head and neck. Amyloidosis can occur in practically any organ and commonly affects the kidneys, heart, nerves, gastrointestinal tract, skin, and lungs. Death is usually caused by kidney or heart failure.

Laryngeal amyloid is almost always localized and not systemic. Amyloid may be most frequently in the supraglottis, followed by glottis, and subglottis (6). One-third of patients may also have tracheal involvement. Symptoms of laryngeal involvement include hoarseness, stridor, globus, and dysphagia. Hemoptysis is rare. Laryngeal amyloidosis accounts for less than 1% of all benign laryngeal lesions.

On endoscopic exam, laryngeal lesions have a waxy, gray to yellow-orange appearance, with rubbery or firm texture. Sometimes the epiglottis is massively enlarged (3). Figure 3 depicts massive enlargement of the false vocal fold. Subglottic involvement can also project into the lumen and restrict the airway. Diagnosis can only be established by biopsy. With hematoxylin-eosin stain, amyloid is an extracellular, amorphous, pink material. Classic apple-green birefringence is seen with Congo red stain under polarized light. Electron microscopy shows linear, nonbranching fibrils in p-pleated sheets.

There is no specific treatment for amyloidosis. When it is secondary to multiple myeloma, the primary disease is managed pharmacologically by melphalan and prednisone. Systemic amyloidosis, however, can progress to death, despite control of the myeloma.

FIGURE 3 Laryngeal sarcoidosis involving epiglottis and left false vocal fold. Tip of laryngoscope is elevating the epiglottis.

Local symptoms of laryngeal amyloidosis can be managed by endoscopic excision, or less commonly, by open surgery. Total removal is often not possible and the disease typically recurs, requiring frequent surgery, or ultimately tracheotomy to relieve obstruction. Scarring from surgical excision can cause permanent airway or voice impairment.

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