| Objective:To summarize the clinical features of laryngotracheal stenosis caused by granulomatosis with polyangiitis,and to understandthe clinic characteristic of the disease.Method:Analysisretrospectivelytheclinicalsymptom,laboratory examination,imaging data,pathological manifestation,treatment and follow-up of a case,who was suffered from laryngotracheal stenosis as the initial manifestation caused by granulomatosis with polyangiitis,in the first affiliated hospital of Guangxi Medical University.Review the related literatures and discuss the characteristics of the disease.Result:（1）The case in our hospital was a 36 year-old female,who mainly showed the clinical features below:1)Laryngotracheal stenosis was the main manifestation,withmultiplesystems（nose,lung,skin,joint,oralcavity）involved.2)The main laboratory examination was c-ANCA and anti-PR3positive,chest CT showed multiple nodules with cavity formation,fiberoptic bronchoscopy suggested subglottic tracheal stenosis.3)Cellulose degeneration of vascular wall was found in superficial and deep dermis,and infiltration of lymphocytes and neutrophils around blood vessels could be seen.So she was diagnosedwithGPA.Aftertreatmentwithcorticosteroidsand immunosuppressive,she wasimproved in nasal,lung,skin,joint-related and mouth symptoms and the pulmonary nodules became smaller,but dyspnea symptoms remained.The therapeutic effect of surgical treatment endotracheal tumor removal and laryngeal mould insertion is poor.Tracheotomy and indwelling tracheal intubation were conducted.Dyspnea disappeared after after high frequency electrotomy,balloon dilation and CO2 cryosurgery under fiberoptic bronchoscope.Endotracheal intubation was removed 10 months later.The laryngotracheal stenosis was stable during 22 months of follow-up.（2）Results of literature review:A total of 19 cases of literature were collected,including 9 males and 10 females.The average age of onset is29.Besides the laryngotracheal,other organs including nose and sinuses（89.5%）,lungs（78.9%）,kidneys（47.4%）,ears（15.8%）,oral（15.8%）,eyes（5.3%）,skin（10.5%）,joint and muscle（26.3%）,and digestive system（10.5%）were also suffered from GPA.ANCA was performed in 13 cases,of which 12（92.3%）were c-ANCA positive,and 1（7.7%）were negative for both c-ANCA and p-ANCA.Fiberoptic bronchoscopy or laryngoscope suggested that 18 cases of airway stenosis were located under the glottis,and only 1 case was located in the supraglottic region of the larynx.Pathological biopsies were performed in 17patients,and the main positive manifestations were necrotic granuloma and vasculitis.All the 19 cases of laryngotracheal stenosis due to GPA were treated with glucocorticoid,with 16 cases treated with cyclophosphamide,2 cases treated with rituximab,1 case treated with azathioprine,and 3 cases treated with sulfamethoxazole.Most patients gained little positive effectsand required one or more local treatments,such as dilation,local injection of steroid hormones or mitomycin-C,CO2 laser resection,and surgery.Some of them had to undergo tracheotomy.All the 19 cases were treated with immunosuppressive therapy.At the beginning of diagnosis,14 cases were treated with immunosuppressive therapy alone,only 5 cases（35.7%）were relieved after treatment,and 3 cases（60.0%）had recurrence during the follow-up period.On the basis of immunosuppressive therapy,14 patients were treated with local therapy.The number of patients involved in local treatment was 18（4 patients were treated with 2 kind of local treatments at the same time）.After treatment,dyspnea was relieved in 13 cases（92.9%）,in whom 1 case（7.1%）had recurrence after laser treatment during the follow-up period.No obvious relief was found in 1 case after surgical airway reconstruction,who had to wear a tracheotomy cannula during the 6-year follow-up period.Tracheotomy was performed in 10 patients（52.6%）.Conclusion:The involvement of laryngotracheal is a rare clinical manifestation of GPA,in which granuloma and vasculitis proliferating leads to stenosis.Immunosuppressive therapy alone can not significantly alleviate dyspnea,so it is necessary to implement surgery and respiratory endoscopic interventional therapy at the same time. |
