[Diagnosis and treatment of relapsing polychondritis].
OBJECTIVE
To explore the diagnosis and treatment of relapsing polychondritis (RP).


METHODS
The clinical manifestations, diagnosis, and treatment of 24 patients with RP were retrospectively analyzed. Twenty-four RP patients were examined for autoimmune antibody. Laryngoscopy was performed in 4 out of 6 RP patients with laryngeal involvement. Bronchoscopy was performed in 12 out of 19 RP patients with lower respiratory tract involvement. Biopsies were taken from the cartilage involved in 16 out of 24 patients.


RESULTS
Five patients were ANA positive at present, 2 patients were SSA antibody positive, 2 patients were anti-RNP antibody positive, and 2 patients were anti-Sm antibody positive. Laryngoscopy revealed vocal fold edema and laryngeal stenosis. Bronchoscopy showed stenosis of trachea and both main bronchi and destruction of tracheobronchial cartilage. Histopathology revealed chronic inflammation of cartilage. Treatment included immunosuppressants (cyclophosphamide, methotrexate) together with prednisone. Eleven patients with respiratory tract involvement had laryngotracheostomy or airway stenting. Twenty-three patients alleviated after treatment, and 1 patients died.


CONCLUSIONS
RP involves cartilage and connective tissue. Laryngotracheobronchial complications are the most severe manifestations of this disease. Corticosteroids and immunosuppressive drugs are effective treatment options. Patients with laryngotracheal stenosis and collapsed tracheobronchial wall should receive laryngostomy, tracheostomy, or airway stenting to improve airway obstruction symptoms.
Xu
Ke-Jian
KJ
Department of Dermatology, PUMC Hospital, CAMS and PUMC, Beijing 100730, China.
Liu
Yue-Hua
YH
Jiang
Ming
M
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008727:Methotrexate / Q000627:therapeutic use; D011081:Polychondritis, Relapsing / Q000175:diagnosis* / Q000188:drug therapy*; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone; D008727:Methotrexate