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What caused sudden painful swelling and redness to develop in this older man's left eye and ear? That's the question facing rheumatologists in a recent case report.

A 67-year-old patient presented with unilateral new-onset pain and erythema of the eye and ear. Further questioning revealed that he had had a cough for 1 month, and had lost 9 kg over the past 3 months, noted Monica Yang, MD, and Mary Margaretten, MD, MAS, both of the University of California San Francisco, in .

The patient's medical history included an episode of right eye pain and redness about 12 months previously; this resolved on its own, only to recur in his left eye 2 months later. He denied having any fevers, chills, joint pain, hoarseness, headaches, or changes in his sight or hearing, nor had he experienced dyspnea, wheezing, chest pain, or rash.

A physical examination showed that the patient's vital signs were normal, and there was no evidence of abnormalities on nasal, cardiopulmonary, joint, and skin examinations. Examination of his affected left eye and ear revealed an erythematous and edematous patch on his left sclera, and swelling and redness affecting the cartilage of the external aspect of his left ear, with sparing of the earlobe.

Laboratory test results included:

• White blood cell count of 4,200/μL
• Hemoglobin level of 12.9 mg/dL
• Platelet count of 142,000/μL
• Erythrocyte sedimentation rate (ESR) of 53 mm/h (reference range 0-15 mm/h)
• C-reactive protein level of 135 mg/L (reference range 0-8 mg/L)
• Negative results for antineutrophil cytoplasmic antibodies and HLA-B27

Clinicians diagnosed the patient with relapsing polychondritis, and prescribed treatment with prednisone 20 mg orally daily. He also underwent ophthalmologic evaluation.

Within 1 week of starting steroid treatment, his ear symptoms and scleritis had resolved. After 1 month, his C-reactive protein and ESR levels returned to normal; clinicians reduced his prednisone dose to 15 mg. However, his auricular inflammation and eye scleritis recurred on his left side.

Clinicians increased his prednisone dose to 20 mg, and added oral methotrexate 25 mg weekly. Within 1 week, his ear and eye symptoms had resolved. A follow-up workup including chest x-ray, pulmonary function testing (PFT), chest CT, and otolaryngological evaluation revealed no abnormalities. At the most recent follow-up, 6 months after presentation, the patient was asymptomatic and was taking prednisone 10 mg daily and methotrexate 25 mg weekly.

Discussion

Yang and Margaretten noted that "the key to the correct diagnosis is recognizing that auricular inflammation and scleritis are characteristic of relapsing polychondritis." They pointed out that while recommended diagnostic measures for relapsing polychondritis include chest radiography and PFT, treatment should not be delayed by these procedures. A biopsy of the affected ear is not needed when the condition can be identified based on a patient's clinical presentation, and would be performed only rarely for atypical presentations, they noted.

In the absence of validated diagnostic criteria, based mainly on symptoms and physical examination findings suggestive of chondritis. Although there are no specific diagnostic laboratory tests, the authors said, about 60% of patients have elevated levels of C-reactive protein and ESR.

As in this patient, "testing for antineutrophil cytoplasmic antibodies is recommended to exclude granulomatosis with polyangiitis, which may present similarly," Yang and Margaretten wrote. Newly should be supported by "a baseline assessment of organ involvement, including otolaryngological and ophthalmologic evaluation, PFTs, chest radiography, chest CT, electrocardiography, and echocardiography."

This rare, progressive, relapsing-remitting autoimmune disease -- with an annual incidence of 0.7 to 3.5 cases per million -- is characterized by repeated inflammatory episodes with the potential to damage "cartilaginous and proteoglycan-rich tissues throughout the body," the authors noted. It generally develops during middle age, and may affect women slightly more than men.

About one in three individuals who develop relapsing polychondritis have a co-existing systemic illness, such as vasculitis, connective tissue disease, or myelodysplastic syndrome. Its variable presentation and episodic nature can contribute to significant delays in diagnosis, often lasting years after symptoms initially present.

The condition frequently involves the ears, nose, and eyes. Approximately 90% of patients with relapsing polychondritis have auricular chondritis, which can damage the ear cartilage, leading to a cauliflower appearance and causing hearing loss in about 40% of those affected. In addition, "nasal chondritis can cause saddle nose deformity," the authors wrote.

Relapsing polychondritis affects the eyes in about 50% of patients, and carries a risk of blindness. Patients often present with scleritis/episcleritis (the most common ocular complications associated with relapsing polychondritis), followed by uveitis and conjunctivitis, while keratitis, retinopathy, optic neuropathy, muscle palsy, and orbital inflammation are seen less frequently.

The condition also affects the larynx or in 20% to 50% of patients, with the potential to "cause laryngeal or tracheal stenosis, tracheomalacia, or tracheobronchial obstruction," Yang and Margaretten wrote. Notably, sudden death has been reported in patients with laryngeal or tracheal spasm or acute airway edema. Patients with cardiovascular involvement may experience "aortic regurgitation, mitral regurgitation, and ascending aortic aneurysms. Costochondritis causes chest pain and if severe, may impair breathing," they noted.

Additionally, about 50% of patients develop "asymmetric, nonerosive arthritis of large and small joints," and "many experience systemic symptoms such as fatigue and weight loss," the authors added.

Noting the paucity of data or standardized approaches to treating relapsing polychondritis, they said that patients with chondritis affecting the ears, nose, and joints are generally managed with nonsteroidal anti-inflammatory drugs, such as ibuprofen 800 mg 3 times daily, as well as low-dose oral glucocorticoids, colchicine, or dapsone in cases of . When the eyes or cardiopulmonary system are involved, patients should receive prednisone 0.25 to 1 mg/kg, dosed according to disease severity, they added.

Recurrent or persistent symptoms when steroids are tapered should be treated with methotrexate, azathioprine, or mycophenolate mofetil, Yang and Margaretten noted. When disease is life-threatening, "pulse-dose solumedrol (500-1,000 mg intravenously for 3 to 5 days) is recommended and cyclophosphamide may be considered."

Biologics such as infliximab (Remicade), abatacept (Orencia), or tocilizumab (Actemra) have been used in patients with treatment-refractory relapsing polychondritis. Treatment should be tapered slowly over months after remission is achieved, the authors added, although long-term immunosuppression may be required in some cases.

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