CasesBackground

A previously healthy 42-year-old woman with a history significant for only hypertension presents with a 1-week history of pain and swelling in her left ear. She denies trauma and dizziness and does not have any difficulty hearing. She also denies buzzing, discharge, and a sensation of fullness in the affected ear. Use of over-the-counter medications, such as ibuprofen, and the application of cold packs relieve the pain. She had similar pain in both ears nearly 2 years ago. At that time, her primary care physician told her that she had an infection of the canal of the ear and treated her with antibiotic eardrops, which did not help. On review of systems, the patient reports that, over the last several months, she has had joint pains that seem to move from 1 joint to another, intermittent fevers, and a generalized decrease in exercise tolerance because of fatigue and shortness of breath. She also unintentionally lost about 10 lb. Physical examination demonstrates a temperature of 37°C, a heart rate of 62 beats per minute, and a blood pressure of 112/67 mm Hg. The left external ear is diffusely edematous with mild erythema and tenderness to palpation, sparing the ear lobe. Examination of the external auditory canal reveals edema, but no discharge or evidence of infection is noted. The tympanic membrane is intact, and a normal light reflex is observed. Hearing is grossly intact. The patient has no cervical or axillary lymphadenopathy. Findings on cardiac and respiratory examination are unremarkable. She has no deformity or limitation in range of motion of any of her joints.

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***** HINT *****
Biopsy of the auricle reveals perichondrial lymphatic T-cell infiltration.
***** ANSWER *****

Relapsing polychondritis: After a lengthy workup, which included several consultations and a biopsy, relapsing polychondritis (RP) was eventually diagnosed. RP is an uncommon and severe systemic and episodic inflammatory disease that compromises the structural integrity of cartilage. This condition typically affects the cartilaginous structures of the ear, nose, and/or laryngotracheobronchial tree. When the ear is affected, the patient usually presents with unilateral or bilateral external-ear inflammation with pain, tenderness, and patchy erythema. The ear lobes are often spared, as in this patient. In severe cases, specifically those involving the ear, the opening to the external auditory canal, as well as the canal itself, may remain edematous for weeks. RP usually affects other cartilaginous structures in addition to the ear, including the eyes (where it leads to scleritis, episcleritis, and blindness), cardiovascular system, peripheral joints (where it leads to deformity and arthritis), nervous system, nose (saddle-nose deformity), and airway (laryngotracheobronchial stricture, epiglottitis, need for permanent tracheotomy). Complications include systemic vasculitis, anemia, severe pulmonary infection, aortic dissection, and glomerulonephritis-associated renal failure. The pathogenesis of the disease is most likely autoimmune, as evidenced by pathology findings that demonstrate infiltrating T cells and antigen-antibody complexes in the affected cartilage. In addition, patients with RP have concurrent autoimmune disorders 25-35% of the time. The condition most commonly occurs in the fifth decade of life and affects 3 times as many women as men. Several criteria can be used to diagnose RP. One set, the McAdam criteria, requires at least 3 of the following 6 clinical features: bilateral auricular chondritis, nonerosive seronegative polyarthritis, nasal chondritis, ocular inflammation, chondritis of the respiratory tract, and audiovestibular damage. Other diagnostic criteria include positive histology, chondritis of 2 or more separate anatomic locations, and therapeutic response to steroids. The differential diagnosis of RP is extensive and includes inflammatory arthritis, vasculitides, localizing chondritis (eg, infectious etiology including bacteria, tuberculosis, syphilis, leprosy), sarcoidosis, and trauma. Results of laboratory tests are mostly unremarkable, but nonspecific elevations of the erythrocyte sedimentation rate with eosinophilia (10%) and levels of anti-type II collagen antibodies (>50%) can suggest the disease. Biopsy of the cartilage can be performed if histologic confirmation of RP is necessary. Samples demonstrate chondrolysis, chondritis, and perichondritis with a mixed inflammatory infiltrate of lymphocytes, neutrophils, and plasma cells in the perichondrium. Immune complexes (immunoglobulin G [IgG] and complement components) may be present at the chondrofibrous junction, with destruction of the cartilage matrix that eventually leads to its replacement by fibrous connective tissue. Despite appearing erythematous on clinical observation, the overlying skin is usually normal. Treatment guidelines are based on data from case studies and the objective of therapy is to relieve symptoms and maintain the integrity of the cartilage. In mild cases, nonsteroidal antiinflammatory drugs (NSAIDs) may be effective, but many cases require high-dose steroids given either intermittently or (most often) continuously. Doses may be as high as 80-100 mg/d. Other medications reported to control symptoms include dapsone, methotrexate, cyclosporine, azathioprine, cyclophosphamide, or anti-tumor necrosis factor antibody (infliximab). Surgical intervention may include tracheostomy, aortic-aneurysm repair, and cardiac valve replacement. The benefits must be weighed against the risk of infection, regardless of the administration of corticosteroids. A team approach involving specialists to manage specific manifestations of the disease is recommended. The 5-year survival rate for RP had originally been reported to be 66-74% (or 45% if RP occurs with systemic vasculitis). More recently, the 5-year survival rate has been 94%. The most common causes of death are respiratory compromise (10-28% of deaths result from airway chondritis) and infection related to immunosuppressive therapy

Author

John Cho, MD, UCLA – Olive View Medical Center Residency, Department of Internal Medicine, Olive View – UCLA Medical Center Rick Kulkarni, MD, Attending Physician, Director of Informatics, Department of Emergency Medicine, Olive View – UCLA Medical Center, Assistant Professor of Medici

ne, David Geffen School of Medicine at UCLA

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