A 56-year-old man presented to the emergency department complaining of bilateral ear pain and eye redness. Ten days ago, he noticed right ear swelling and pain and treated this at home with cold compresses. These symptoms seemed to improve, but over the next few days his left ear began to swell. Both eyes subsequently became red and extremely painful with tearing and photophobia associated with an intense retrobulbar headache. He was seen at another hospital three days ago and treated with antibiotic eye drops for conjunctivitis. He admitted to using more than the prescribed dose and used the entire bottle of medication with no relief of his eye symptoms. He denied any prior similar episodes, trauma, sick contacts or new exposures.
His past medical history was significant for treated hypertension and schizophrenia. He reported an allergy to ibuprofen and acetaminophen, but is able to tolerate Percocet without difficulty. He is a smoker and denied alcohol or drug use.
On exam, vital signs were normal with no fever. He was sitting in a darkened room in obvious discomfort. The initial eye exam was limited due to intense photophobia. However, his vision was intact. His ear exam was notable for significant bilateral swelling to the cartilage, slightly worse on the left. The ears were minimally tender without drainage. The tympanic membranes were normal as was his hearing. Nasal exam was unremarkable. Cervical adenopathy was present. The remainder of the exam was unremarkable.
The patient’s pain was controlled, and he was admitted to the hospital where he was evaluated by ophthalmology, ENT and rheumatology.
What are your concerns as you review the history and images of this patient?
Dx: Relapsing Polychondritis
This patient had relapsing polychondritis, a rare autoimmune disease that affects the cartilaginous structures of the body. Structures at risk include the nose, ears, joints and the tracheobronchial tree. Also at risk are the eyes, heart and blood vessels.
Almost all patients have auricular involvement at some point in their course. Eye symptoms, including scleritis, episcleritis, conjunctivitis, keratitis or uveitis, occur in half of the patients. Relapsing polychondritis is a clinical diagnosis. The differential varies based on which structures are affected. Wegener’s granulomatosis may present with nasal destruction. Auricular swelling may also be caused by trauma or infection.
Treatment for relapsing polychondritis varies based on the severity and location of inflammation. Chondritis localized to the ears or nose may be treated with nonsteroidal anti-inflammatory drugs. More serious organ involvement is treated with steroids, other immunosuppressive drugs and recently with emerging biological therapies. Maintenance therapy has been attempted with cochicine, indomethacin, or diaminodiphenylsulfone (dapsone) with some reports of success. Surgical intervention for airway and cardiac involvement may be necessary.
Patients with relapsing polychondritis have a reduced life expectancy. Symptoms fluctuate but typically progress. Destruction of respiratory and cardiac structures lead to the significant morbidity and mortality associated with the disease.
Ernst A. Relapsing polychondritis and airway
involvement. Chest. 2009 Apr; 135(4):1024-30.
Gergely P. Relapsing polychondritis. Best Pract Res Clin
Rheumatol. 2004 Oct. 18(5):723–38.
Trentham DE. Relapsing polychondritis. Ann Intern
Med. 1998 Jul; 129(2):114-22.