Background/Purpose:

Recently several case reports of serous otitis media (SOM), hypertrophic pachymeningitis (HP) and cranial neuropathy (CN) have been reported in connection with MPO-ANCA associated vasculitis (MPO-AAV). However, there are few clinical studies on the lesions. Herein, we address the frequency and the characteristics of these manifestations in patients with MPO-AAV

Methods:

This retrospective study focused on consecutive patients in whom MPO-AAV was diagnosed between 2003 and 2014 at Tokyo Metropolitan Tama Medical Center. We investigated their clinical and radiological profile by reviewing the medical records.

Results:

A total of 111 patients with MPO-AAV were seen at the hospital in this period. There were 19 patients (17%) with at least one of the manifestations: SOM, HP, or CN. SOM was observed in 16 cases (14%), and constituted the first manifestation of the disease in 11 cases. HP and CN were seen in eight (7%) and seven (6%) patients, respectively. Of the patients presenting with SOM, seven patients had HP and/or CN (4 with HP and CN, 2 with CN, 1 with HP). Of three HP patients unassociated with SOM, CN was detected in one. There were no patients with isolated CN. Of the 11 patients examined by MRI, six patients demonstrated intense inflammation in the epipharynx. There was a significant difference in the rate of glomerulonephritis between the patients with the manifestations and those without them (32% vs. 73%; P = 0.0006). Furthermore, none of the patients with HP exhibited glomerulonephritis.

Conclusion:

SOM is not a rare manifestation in MPO-AAV and often precedes other manifestations. There is a close relationship among SOM, HP and CN. Inflammation in the epipharynx might play a role as a pre-condition for the development of SOM and HP.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/otolaryngologic-lesions-are-not-rare-and-closely-related-with-pachymeningitis-and-cranial-neuropathy-in-mpo-anca-associated-vasculitis/