疑难病杂志
疑難病雜誌
의난병잡지
JOURNAL OF DIFFICULT AND COMPLICATED CASES
2015年
2期
137-140
,共4页
白雪%胡红%许菡苡%韩国敬%苏宪灵%解立新
白雪%鬍紅%許菡苡%韓國敬%囌憲靈%解立新
백설%호홍%허함이%한국경%소헌령%해립신
多发性软骨炎,复发性%气道受累%诊断%治疗
多髮性軟骨炎,複髮性%氣道受纍%診斷%治療
다발성연골염,복발성%기도수루%진단%치료
Polychondritis,recurrent%Airway involvement%Diagnosis%Treatment
目的:分析复发性多软骨炎的临床特征及治疗效果。方法回顾性分析2000年9月—2014年11月在解放军总医院住院并确诊的复发性多软骨炎患者48例的临床资料。结果48例患者中,男22例,女26例,确诊时年龄15~79(46.7±13.9)岁。从首次就诊到确诊中位时间为5个月。受累器官包括关节33例(68.8%),耳部31例(64.6%),呼吸系统29例(60.4%),眼部26例(54.2%),鼻部20例(41.7%),皮肤9例(18.8%),血液系统9例(18.8%),肾脏5例(10.4%)。在呼吸系统受累的29例患者中,行肺CT检查22例,表现为气管及主支气管壁增厚15例(68.2%),气道狭窄14例(63.6%),气道壁钙化8例(36.4%),气道塌陷2例(9.1%);行喉CT检查6例,喉部软组织增厚2例,声门下气管狭窄、管壁增厚2例,喉腔闭塞1例。48例全部接受糖皮质激素治疗,其中40例同时加用了免疫抑制剂,4例因疗效不佳行气管切开,余44例治疗后病情好转。随访30例,其中4例因喘息、气短及肺炎多次住院治疗,1例因呼吸困难加重行气管支架治疗,术后出现呼吸衰竭,其余25例病情平稳。结论复发性多软骨炎易累及全身多个系统,误诊率较高,应用糖皮质激素联合免疫抑制剂治疗效果较佳,但呼吸系统受累导致气道严重狭窄者可能需要气管切开及气管支架治疗。
目的:分析複髮性多軟骨炎的臨床特徵及治療效果。方法迴顧性分析2000年9月—2014年11月在解放軍總醫院住院併確診的複髮性多軟骨炎患者48例的臨床資料。結果48例患者中,男22例,女26例,確診時年齡15~79(46.7±13.9)歲。從首次就診到確診中位時間為5箇月。受纍器官包括關節33例(68.8%),耳部31例(64.6%),呼吸繫統29例(60.4%),眼部26例(54.2%),鼻部20例(41.7%),皮膚9例(18.8%),血液繫統9例(18.8%),腎髒5例(10.4%)。在呼吸繫統受纍的29例患者中,行肺CT檢查22例,錶現為氣管及主支氣管壁增厚15例(68.2%),氣道狹窄14例(63.6%),氣道壁鈣化8例(36.4%),氣道塌陷2例(9.1%);行喉CT檢查6例,喉部軟組織增厚2例,聲門下氣管狹窄、管壁增厚2例,喉腔閉塞1例。48例全部接受糖皮質激素治療,其中40例同時加用瞭免疫抑製劑,4例因療效不佳行氣管切開,餘44例治療後病情好轉。隨訪30例,其中4例因喘息、氣短及肺炎多次住院治療,1例因呼吸睏難加重行氣管支架治療,術後齣現呼吸衰竭,其餘25例病情平穩。結論複髮性多軟骨炎易纍及全身多箇繫統,誤診率較高,應用糖皮質激素聯閤免疫抑製劑治療效果較佳,但呼吸繫統受纍導緻氣道嚴重狹窄者可能需要氣管切開及氣管支架治療。
목적:분석복발성다연골염적림상특정급치료효과。방법회고성분석2000년9월—2014년11월재해방군총의원주원병학진적복발성다연골염환자48례적림상자료。결과48례환자중,남22례,녀26례,학진시년령15~79(46.7±13.9)세。종수차취진도학진중위시간위5개월。수루기관포괄관절33례(68.8%),이부31례(64.6%),호흡계통29례(60.4%),안부26례(54.2%),비부20례(41.7%),피부9례(18.8%),혈액계통9례(18.8%),신장5례(10.4%)。재호흡계통수루적29례환자중,행폐CT검사22례,표현위기관급주지기관벽증후15례(68.2%),기도협착14례(63.6%),기도벽개화8례(36.4%),기도탑함2례(9.1%);행후CT검사6례,후부연조직증후2례,성문하기관협착、관벽증후2례,후강폐새1례。48례전부접수당피질격소치료,기중40례동시가용료면역억제제,4례인료효불가행기관절개,여44례치료후병정호전。수방30례,기중4례인천식、기단급폐염다차주원치료,1례인호흡곤난가중행기관지가치료,술후출현호흡쇠갈,기여25례병정평은。결론복발성다연골염역루급전신다개계통,오진솔교고,응용당피질격소연합면역억제제치료효과교가,단호흡계통수루도치기도엄중협착자가능수요기관절개급기관지가치료。
Objective To analyze the clinical characteristics and therapeutic effect of relapsing polychondritis. Met-hods Retrospective analysis from 2000 September to 2014 November clinical data of 48 patients with relapsing polychondritis in PLA General Hospital. Results In the 48 patients, male were 22 cases, female were 26 cases, age at diagnosis was 15-79 (46. 7 ± 13. 9) years old. From the first visits to the diagnosis with a median of 5 months. The involved organs including the joint in 33 cases (68. 8%), ears in 31 cases (64. 6%), 29 cases of respiratory tract (60. 4%), 26 cases of eyes (54. 2%), 20 cases of nasal (41. 7%), 9 cases (18. 8%) in skin, 9 cases of blood system (18. 8%), and kidney in 5 ca-ses (10. 4%). In 29 cases of respiratory system involvement, 22 cases received lung CT scan, 15 cases revealed the tracheal and bronchial wall thickening (68. 2%), airway stenosis in 14 cases (63. 6%), airway wall calcification in 8 cases (36. 4%), upper airway collapse in 2 cases (9. 1%);6 cases received laryngeal CT examination, throat soft tissue thicken-ing in 2 cases,subglottic and tracheal stenosis, wall thickening in 2 cases,laryngeal cavity occlusion in 1 cases. All 48 cases received corticosteroid therapy, of which 40 cases combined with immune inhibitor, tracheotomy was performed in 4 cases be-cause of poor efficacy, the remaining 44 cases after treatment, the condition was improved. Follow up of 30 cases, 4 cases re-admitted to hospital several times because of wheezing, shortness of breath and pneumonia, in 1 cases received tracheal stent treatment because of dyspnea, respiratory failure occurred after operation, the other 25 cases with stable condition during fol-low up. Conclusion The relapsing polychondritis involves multiple system with a high rate of misdiagnosis, treatment effect of glucocorticoid combined with the application of immunosuppressant are good, but the respiratory system involvement leads to severe airway stenosis may need tracheotomy and tracheal stent.