Significant Rate of Multiorgan Involvement in Patients with Laryngeal Amyloidosis

Comment: This manuscript out of Stanford evaluates all 865 patients treated for amyloidosis at the institution over 20 years, and identified a cohort of 22 patients with biopsy-proven laryngeal amyloidosis. The researchers compared this cohort with an age- and sex-matched set of amyloidosis patients with negative laryngoscopy, and show much higher rates of systemic amyloidosis involvement than has previously been shown. The article is of interest due to the relatively higher number of patients in this study compared with prior studies. —Jonathan Bock, MD

What are the differences in clinical presentation and outcomes of laryngeal amyloidosis versus systemic amyloidosis?

Bottom Line
There is a significant rate (18%) of multiorgan involvement in patients with laryngeal amyloidosis, which contradicts conventional concepts that this is an isolated disorder. This finding could have a significant impact on the evaluation and management of patients with laryngeal amyloidosis.

Background: Amyloidosis represents a heterogeneous group of disorders marked by abnormal protein formation and deposition, which can affect multiple organs throughout the body. Despite the heterogeneity of this disease, all forms of amyloid are characterized by a signature abnormality in protein synthesis, namely the formation of β-pleated sheets from extracellular fibrils. Laryngeal amyloidosis is rare and classically thought to remain isolated with little risk of systemic involvement or associated malignancy. This study sought to further characterize differences in clinical characteristics between patients with laryngeal and nonlaryngeal amyloidosis.

Study design: Retrospective case-control study prospectively collected data.

Setting: Stanford University School of Medicine, Calif.

Synopsis: Of 865 patients treated for amyloidosis between 1996 and 2016, 22 (2.5%) patients with biopsy-proven laryngeal amyloidosis were identified. An additional 22 patients with amyloidosis of a different organ and negative laryngoscopy—and therefore without laryngeal amyloidosis—were identified as controls. Compared to these controls with nonlaryngeal amyloidosis, patients with laryngeal amyloidosis were younger, and 18% had additional organ involvement. Immunoglobulin light-chain amyloidosis was the most common subtype in both groups of patients. Eighty-six percent of patients with laryngeal amyloidosis required surgical excision, and of these patients, more than 30% required multiple excisions.

Citation: Rudy SF, Jeffery CC, Damrose EJ. Clinical characteristics of laryngeal versus nonlaryngeal amyloidosis. Laryngoscope. 2018;128:670–674.