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Recurrent Eye and Ear Pain in an Older Patient

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 67-year-old man presented to his primary care physician with new-onset pain and erythema of his left eye and left ear, cough for 1 month, and a 9-kg weight loss over the past 3 months. Twelve months prior, he experienced a self-limited episode of right eye pain and redness, and 2 months later, a similar episode occurred in his left eye. He reported no fevers, chills, headaches, visual changes, hearing loss, hoarseness, shortness of breath, wheezing, chest pain, arthralgias, or rash. Physical examination revealed normal vital signs, and no abnormalities on nasal, cardiopulmonary, joint, and skin examination. His left eye had scleral erythema, and the cartilaginous portion of his left ear was swollen and erythematous (Figure).

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Relapsing polychondritis

C. Prescribe prednisone, 0.25 to 1 mg/kg orally daily

The key to the correct diagnosis is recognizing that auricular inflammation and scleritis are characteristic of relapsing polychondritis. Although chest radiography and PFT (choice A) are recommended when evaluating patients for relapsing polychondritis, these tests should not delay treatment. A biopsy of the left ear (choice B) is unnecessary if the diagnosis of relapsing polychondritis can be made based on clinical presentation. Ibuprofen (choice D) is incorrect because relapsing polychondritis affecting the eyes should be treated with steroids.

Relapsing polychondritis is a rare, progressive, relapsing-remitting autoimmune disease that affects cartilaginous and proteoglycan-rich tissues throughout the body, and it results in recurrent episodes of inflammation that can destroy tissue. The incidence of relapsing polychondritis is approximately 0.7 to 3.5 cases per million per year.1,2 Disease onset typically occurs in middle age, and women appear slightly more affected than men.1,2 Approximately one-third of patients have another systemic illness, such as vasculitis, connective tissue disease, or myelodysplastic syndrome.1,3 Due to the frequent spontaneous resolution of flares and variability of presentation, the diagnosis of relapsing polychondritis is often delayed for years after initial symptom onset.3,4

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Article Information

Corresponding Author: Monica Yang, MD, Division of Rheumatology, Department of Medicine, University of California, San Francisco, 400 Parnassus Ave, San Francisco, CA 94143 (monica.yang@ucsf.edu).

Published Online: May 27, 2022. doi:10.1001/jama.2022.8313

Conflict of Interest Disclosures: None reported.

Funding/Support: Dr Yang’s work was supported by NIH grant T32 AR079068-01.

Role of the Funder/Sponsor: The NIH had no role in the preparation, review, or approval of the manuscript or in the decision to submit the manuscript for publication.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Mathian  A , Miyara  M , Cohen-Aubart  F ,  et al.  Relapsing polychondritis: a 2016 update on clinical features, diagnostic tools, treatment and biological drug use.   Best Pract Res Clin Rheumatol. 2016;30(2):316-333. doi:10.1016/j.berh.2016.08.001PubMedGoogle ScholarCrossref
2.
de Montmollin  N , Dusser  D , Lorut  C ,  et al.  Tracheobronchial involvement of relapsing polychondritis.   Autoimmun Rev. 2019;18(9):102353. doi:10.1016/j.autrev.2019.102353PubMedGoogle ScholarCrossref
3.
Lekpa  FK , Chevalier  X .  Refractory relapsing polychondritis: challenges and solutions.   Open Access Rheumatol. 2018;10:1-11. doi:10.2147/OARRR.S142892PubMedGoogle ScholarCrossref
4.
Ferrada  MA , Grayson  PC , Banerjee  S ,  et al.  Patient perception of disease-related symptoms and complications in relapsing polychondritis.   Arthritis Care Res (Hoboken). 2018;70(8):1124-1131. doi:10.1002/acr.23492PubMedGoogle ScholarCrossref
5.
Rednic  S , Damian  L , Talarico  R ,  et al.  Relapsing polychondritis: state of the art on clinical practice guidelines.   RMD Open. 2018;4(suppl 1):e000788. doi:10.1136/rmdopen-2018-000788PubMedGoogle ScholarCrossref
6.
Kingdon  J , Roscamp  J , Sangle  S , D’Cruz  D .  Relapsing polychondritis: a clinical review for rheumatologists.   Rheumatology (Oxford). 2018;57(9):1525-1532. doi:10.1093/rheumatology/kex406PubMedGoogle ScholarCrossref
7.
Fukuda  K , Mizobuchi  T , Nakajima  I ,  et al.  Ocular involvement in relapsing polychondritis.   J Clin Med. 2021;10(21):4970. doi:10.3390/jcm10214970PubMedGoogle ScholarCrossref
8.
Catano  J , Uzunhan  Y , Paule  R ,  et al.  Presentation, diagnosis, and management of subglottic and tracheal stenosis during systemic inflammatory diseases.   Chest. 2022;161(1):257-265. doi:10.1016/j.chest.2021.07.037PubMedGoogle ScholarCrossref
9.
Petitdemange  A , Sztejkowski  C , Damian  L ,  et al.  Treatment of relapsing polychondritis: a systematic review.   Clin Exp Rheumatol. Published online May 18, 2022. doi:10.55563/clinexprheumatol/h9gq1oPubMedGoogle ScholarCrossref
10.
Padoan  R , Campaniello  D , Iorio  L ,  et al.  Biologic therapy in relapsing polychondritis: navigating between options.   Expert Opin Biol Ther. 2022;22(5):661-671. doi:10.1080/14712598.2022.2048647PubMedGoogle ScholarCrossref
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