Relapsing Polychondritis With Airway Involvement:Report Of A Case And Review Of Literature

Objective:Through a retrospective case analysis and literature review.to improve the understanding of the variety of clinical featur es and the importance of early diagnosis and treatment of relapsing polychondritis(RP) With the cases Involvement of the respiratory.Methods:one patient diagnosed as relpsing polychondritis(RP) with airway involvement in the First Affiliated Hospital of Guangxi Medical University.we retrospectively analyze the clinical materials of general situation, clinical manifestation, laboratory studies, image ology, pulmonary function, bronchofibroscopy and treatment.Results:1.the male patients was 21 yeas old,with recurrent fe ver, chest pain, shortness of breath as the main clinical symptoms, in the course of hoarseness, hearing loss, saddle nose, eyes blurred vision, skin rashes, White blood cells, c-reactive protein and ESR was elevated, lung CT revealed Trachea and bronchi calcification,1 ung function prompts obstructive ventilation dysfunction, fiber bronc hoscope on trachea stenosis, symptoms improved after hormone com bination immunosuppressant treatment.2. The result of literature revi ew, from the PUBMED and other multiple database retrieval from J anuary 2005 and January 2005 cases involving the respiratory tract in patients with RP case reported at home and abroad, a preliminar y retrieval with 74 references, according to the criteria for the diag nosis of article 39, a total of 50 cases had been reported have a c omplete medical history and clinical data.29 cases of men, women, 21 cases, the youngest 20 years old, biggest 83 years old, average 50.7 years.Respiratory system main clinical manifestation of cough, sputum, dyspnea, hearing and lung wheezing sound.48 routine pulm onary CT examination, more performance for tracheal bronchial wal 1 thickening, luminal stenosis.28 routine lung function, give priority to with obstructive ventilation dysfunction.Only 10 routine bronchial mucosal biopsy.17 cases were misdiagnosed as asthma, bronchitis,1 2 cases, COPD5 cases.Simple to hormone therapy, a total of 14 bet ter, to 22 cases with immune inhibitors, and need to intervene or n oninvasive mechanical ventilation, a total of 10 cases.Only part of t he follow-up, patients with 1 year follow-up in 2 cases,2 years fol low-up in 2 cases,5 years follow-up,1 case had no recurrence.But 1 patient died of respiratory failure after half a year.Conclusions:Merger of respiratory tract involvement is commo n in patients with relapsing polychondritis is a sign of poor progno sis, and easily missed diagnosis and misdiagnosis, lung imaging, pul monary function, and fiber bronchoscope examination is helpful to diagnosis, treatment mainly with the hormone and immune inhibitor s or dapsone, biological agents, the surgical intervention or mechani cal assisted ventilation will helpful when respiratory tract involving.