Fig. 6.17 Relapsing polychondritis: note the loss of nasal cartilage and the resultant nasal deformity

6.1.7.2 Systemic Manifestations

Inßammation of auricular pinnae is the most common feature of the disease, occurring in 88.6% of patients [281]. Recurrent pain, swelling, and tenderness of one or both pinnae lead to resorption of cartilage and soft, pliable, drooping ears. Inßammation of the nasal cartilage occurs in about 50% of patients and may result in a saddle nose deformity (Fig. 6.17). Audiovestibular involvement occurs in about 50% of patients and includes conductive or sensorineural hearing loss with or without vestibular damage. Impaired conductive hearing may be caused by involvement of the eustachian tube, external auditory meatus, or serous otitis media. Impaired sensorineural hearing or vestibular dysfunction may be caused by vasculitis of the cochlear or vestibular branch of the internal auditory artery [281]. Deafness or dizziness, vertigo, tinnitus, ataxia, nausea, and vomiting are the characteristic complaints. Laryngotracheal inßammation may be present in over 50% of patients and produces cough, hoarseness, dyspnea, pain over the anterior tracheal cartilage, and wheezing. Collapse of the upper airway through inßammation of the glottis, larynx, or

subglottic tissue may be the cause of death in 10% of patients. Bronchial disease also can occur, leading to pulmonary infections and respiratory failure. A seronegative, nondeforming, nonerosive oligoarthritis or polyarthritis may appear in 50Ð80% of patients. Costochondral joints are frequently involved. Aortitis due to large-vessel vasculitis is the cardinal cardiovascular manifestation. It may lead to thoracic or abdominal aortic aneurysms that are silent until dissection or rupture occurs. Aortitis also may produce aortic regurgitation with secondary aortic valve insufÞciency, and electrocardiographic abnormalities due to the proximity of the aortic root and the cardiac conduction system.

Manifestations due to smalland mediumsized vessel vasculitis include skin lesions, renal involvement, and neurologic abnormalities. Skin lesions include purpura, urticaria, erythema nodosum, livedo reticularis, angioedema, and migratory thrombophlebitis [282]. Renal involvement presenting with proteinuria or microhematuria may be caused by focal proliferative glomerulonephritis with crescent formation [283]. Neurologic abnormalities include cranial nerve involvement (second, sixth, seventh, and eighth), seizures, hemiplegia, ataxia, and sensorimotor neuropathy [282, 284].

6.1.7.3 Ocular Manifestations

Ocular manifestations may be the presenting symptom of RP in 20% of patients and will eventually occur in about 60% of patients [282]. Scleritis and episcleritis are the most common Þndings, but anterior uveitis, retinitis, extraocular muscle palsies, optic neuritis, conjunctivitis, keratitis, and exophthalmos also may occur [242, 282, 284Ð287].

Scleritis

The reported incidence of RP in patients with scleritis ranges from 0.96 to 6.39% [122Ð124]. Conversely, about 14% of patients with RP have scleritis [282]. Scleritis may be diffuse anterior, nodular anterior, necrotizing anterior, or posterior [122, 288], and tends to occur with other manifestations of disease activity, such as auricular and nasal chondritis and arthritis. Scleritis is