Laryngotracheal involvement as the initial manifestation of relapsing polychondritis.
Relapsing polychondritis (RP) is a rare multisystemic disease characterized by recurrent inflammation of cartilaginous and noncartilaginous tissues. When laryngotracheal or bronchial cartilages are involved, the disease can be life-threatening and needs aggressive treatment. Upper airway complaints are rare as initial presentations of RP. Here, however, we present a case of RP, with initial manifestations of cough and dyspnea that were treated as bronchial asthma for 6 months. Subglottic stenosis was found in April 2003, during a bronchoscopic examination, and emergency tracheostomy was performed. Auricular and nasal chondritis and bilateral scleritis developed 3 months after tracheostomy. High doses of methylprednisolone and immunosuppressive agents were used, and active inflammation in the eyes and ears was controlled, but the patient's upper airway was completely collapsed. This case is reported with the hope of increasing awareness about the potential for early upper airway involvement in RP.
Chang
Shu-Jen
SJ
Section of Allergy, Immunology, and Rheumatology, Department of Medicine, Cheng Hsin Rehabilitation Medical Center, Taipei Veterans General Hospital, Taipei, Taiwan, R.O.C.
Lu
Chong-Chen
CC
Chung
Yu-Mei
YM
Lee
Shinn-Shing
SS
Chou
Chung-Tei
CT
Huang
De-Feng
DF
D000402:Airway Obstruction / Q000209:etiology*; D003371:Cough / Q000209:etiology; D004417:Dyspnea / Q000209:etiology*; D005260:Female; D006801:Humans; D007818:Laryngeal Diseases / Q000150:complications*; D008875:Middle Aged; D011081:Polychondritis, Relapsing / Q000150:complications*; D014133:Tracheal Diseases / Q000150:complications*; D014139:Tracheostomy