Xu Ke-Jian, Liu Yue-Hua, Jiang Ming
Department of Dermatology, PUMC Hospital, CAMS and PUMC, Beijing 100730, China.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2007 Apr;29(2):171-3.
To explore the diagnosis and treatment of relapsing polychondritis (RP).
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.
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.
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.
探讨复发性多软骨炎(RP)的诊断与治疗。
回顾性分析24例RP患者的临床表现、诊断及治疗情况。对24例RP患者进行自身免疫抗体检测。6例有喉部受累的RP患者中4例行喉镜检查。19例有下呼吸道受累的RP患者中12例行支气管镜检查。24例患者中有16例对受累软骨进行活检。
目前5例患者ANA阳性,2例患者SSA抗体阳性,2例患者抗RNP抗体阳性,2例患者抗Sm抗体阳性。喉镜检查显示声带水肿及喉狭窄。支气管镜检查显示气管及双侧主支气管狭窄,气管支气管软骨破坏。组织病理学显示软骨慢性炎症。治疗包括免疫抑制剂(环磷酰胺、甲氨蝶呤)联合泼尼松。11例有呼吸道受累的患者行喉气管造口术或气道支架置入术。23例患者治疗后病情缓解,1例患者死亡。
RP累及软骨和结缔组织。喉气管支气管并发症是该病最严重的表现。糖皮质激素和免疫抑制药物是有效的治疗选择。有喉气管狭窄及气管支气管壁塌陷的患者应行喉造口术、气管造口术或气道支架置入术以改善气道梗阻症状。