Head and Neck Manifestations

Auricular chondritis is the most common presenting feature (40-50%) and eventually occurs in 83% to 95% of patients. The inflammation of the external ear occurs suddenly and can be unilateral or bilateral. Pain and tenderness are frequently severe. The cartilaginous portion of the pinna becomes red/violaceous and swollen while the noncartilaginous earlobe is spared. This helps to separate it from cellulitis. The auricular inflammation lasts a few days or can persist for weeks before subsiding spontaneously or with therapy. With sustained or recurrent episodes, the cartilage of the pinna is destroyed, and the ear can become soft and floppy or knobby and cauliflower-like in appearance (Fig. 2). Other areas of the ear can be affected, including the external auditory meatus. Audio and/or vestibular involvement from Eustachian tube obstruction with serous otitis, middle ear inflammation, or vasculitis of the internal auditory artery usually occurs later in the disease course, resulting in reduced hearing (17-46%) or vestibular dysfunction (13-53%).

Nasal chondritis is present at disease onset in 25% to 30% of patients, and occurs during the course of the disease in 48% to 72% of patients. Symptoms include rhinorrhea, crusting, epistaxis, and decreased olfaction. With sustained or recurrent episodes of inflammation, patients can develop a saddle nose deformity (17-29%) from destruction and collapse of the nasal bridge (Fig. 3).

TABLE 2 Clinical Manifestations of Relapsing Polychondritis

Manifestations

Disease onset (%)

Course of disease (%)

Auricular chondritis

40-50

83-95

Internal ear

Reduced hearing

17-46

Vestibular dysfunction

13-53

Nasal chondritis

25-30

48-72

Saddle nose

17-29

Laryngotracheobronchial

20-25

30-67

Tracheal narrowing

14

Ocular inflammation

20-25

50-65

Arthritis

35-40

50-85

Cardiovascular

5-10

Valvular disease

2-8

Aneurysms

5

Vasculitis

15

Skin involvement

12-16

17-36

Kidney disease

5-10

Neurologic involvement

5-10

Death (8 yr)

6

Source: Adapted from Refs. 3,18-20.

Source: Adapted from Refs. 3,18-20.

FIGURE 2 (Left) The ear of a patient with early relapsing polychondritis. The localized redness, swelling, tenderness, and warmth may be confused with an acute infection. (Right) Relapsing polychondritis that repeatedly recurs or progresses leads to cartilage destruction. The pinna thickens and collapses, resulting in a floppy ear. Source: American College of Rheumatology.

FIGURE 2 (Left) The ear of a patient with early relapsing polychondritis. The localized redness, swelling, tenderness, and warmth may be confused with an acute infection. (Right) Relapsing polychondritis that repeatedly recurs or progresses leads to cartilage destruction. The pinna thickens and collapses, resulting in a floppy ear. Source: American College of Rheumatology.

Laryngotracheobronchial involvement occurs in 20% to 25% of patients at disease onset and in 30% to 67% at some time during the disease (21,22). Notably, airway involvement may be the only manifestation, or it may be asymptomatic early in the disease course. Symptoms depend on the site of involvement and include hoarseness, nonproductive cough, and dyspnea. Luminal narrowing from inflammation or fibrosis can cause wheezing and inspiratory stridor. Tracheal tenderness may be present. With cartilage destruction, laryngeal collapse can occur during inspiration and tracheal collapse during expiration. Airway obstruction or collapse can lead to secondary infections.

Ocular inflammation affects 20% to 25% at disease onset and 50% to 65% during the disease course. Conjunctivitis, episcleritis, and scleritis are the most common manifestations (23). Nongranulomatous uveitis and keratitis can also occur and parallel other disease activities. Other eye manifestations include periorbital edema, chemosis, tarsitis, and proptosis from posterior choroiditis or a mucosa-associated lymphoid tissue (MALT) type lymphoma. Rarely, retinal vasculitis, retinal detachment, retinal artery or vein occlusion,

FIGURE 3 Patient with relapsing polychondritis with saddle-nose deformity caused by collapse of cartilaginous structures. Source: American College of Rheumatology.

optic neuritis/ischemic optic neuropathy, or corneoscleral perforation can cause diminished vision or blindness.

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