Relapsing polychondritis in an adolescent.
Relapsing polychondritis (RP) is an uncommon systemic disorder with a highly variable course. A 17-year-old woman recently presented with a 1-month history of depression, weight loss, chest wall tenderness, hoarseness, and dysphagia. Physical examination revealed cachexia, low-grade fever, pharyngeal erythema, and tenderness of the right auricle, anterior chest, cricothyroid cartilage, and both knees. Laboratory studies included a hematocrit of 34% and a sedimentation rate of 50 mm/hr. Initial improvement on oral corticosteroids was followed by respiratory distress. At that time calcified tracheal cartilage, subglottic stricture, and a saddle nose deformity were present. Despite therapy with steroids, dapsone, and pulse cyclophosphamide, the respiratory distress reoccurred, eventually necessitating tracheostomy. Tracheal cartilage biopsy confirmed the presumptive diagnosis of RP. Bilateral auricular chondritis developed after initial presentation, as did acute vertigo. Although seen in all age groups, less than 10% of cases of RP are seen in children and adolescents. Auricular chondritis (89% of all cases), inflammatory asymetric arthritis (81% of all cases), nasal chondritis (72% of all cases), respiratory tract chondritis (56% of all cases), and audiovestibular abnormalities (46% of all cases) were present in our patient. Relapsing polychondritis may follow a slowly evolving or rapidly progressive course. Appropriate diagnosis and aggressive therapy are recommended to lessen the morbidity and mortality.
Trepel
R J
RJ
Division of Adolescent and Young Adult Medicine, Children's Hospital National Medical Center, Washington, D.C.
Lipnick
R N
RN
D'Angelo
L
L
D000293:Adolescent; D005260:Female; D006801:Humans; D011081:Polychondritis, Relapsing / Q000175:diagnosis*