Background/Purpose: Ear chondritis is often considered the pathognomonic feature of relapsing polychondritis (RP).  Although painful redness and swelling of the pinna and a resultant cauliflower ear are universally recognized as chondritis, the complete spectrum of symptoms associated with ear chondritis have not been well described.  The study objective was to seek patient input to help characterize ear chondritis.

Methods: An online survey was administered in English or Spanish to participants with self-reported RP. Participants were asked questions about their ear pain, including quality, location, duration, aggravating/alleviating factors, timing of onset and duration. Participants were included who reported age ≥ 18 years, a diagnosis of RP confirmed by a physician, and sufficient symptoms to meet McAdams or Damiani’s diagnostic criteria. Participants were categorized as having “typical ear chondritis” if they reported ear pain localized to the pinna with associated redness and swelling. Atypical presentations of ear chondritis were also considered.

Results: A total of 685 participants from five continents completed the survey. Among them, 659 met inclusion criteria for subsequent analysis. Most participants were female (n=574; 87%), white (n=548; 83%) and from the United States (n=484;74%). The median age was 50 years (interquartile range = 41-58). In total, 593 (90%) patients reported ear pain, 227 (38%) had “typical ear chondritis”, and 98 (16%) had cauliflower ear.

Ear pain was most commonly described as burning (n=334, 56%) or throbbing (n=295, 50%).  The most common location of pain was the pinna (n=373, 63%). Participants reported ear redness (n=454, 76%) and swelling (n=349, 53%). Some patients experienced isolated redness (n=130, 22%) or swelling (n=25, 4%). The most common aggravating factors were minor trauma (n=371, 62%) and stress (n=358, 60%).  The most common alleviating factor was avoidance of touching the ear (n=374, 63%). Pain was most frequently reported during the daytime (n=355, 60%) and most likely to occur in either ear at different times (n=310, 52%). Onset could be gradual (n=198, 33%) or sudden (n=155, 26%).  Pain typically lasted a few hours (n=175, 30%) or 2-3 days (n=130, 22%). The majority of patients who had pinna pain also had pain in other parts of the ear (e.g. mastoid process, inner ear, whole ear) at some point (n=394, 67%). In patients with cauliflower ear, the most common location of pain was the pinna (n=57, 58%) followed by pain inside the ear (n=53, 54%). Most participants reported at least two different types of pain (n=420, 64%).

Conclusion: Ear chondritis in patients with RP has a wide range of clinical presentations and characteristics beyond the typical triad of redness, swelling, and pain localized to the pinna. The description of pain often significantly varies within the same patient. Knowledge of the various distinct characteristics of ear involvement in RP may help physicians recognize and monitor the disease more effectively.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/defining-ear-chondritis-data-from-685-patients-with-relapsing-polychondritis/