Fijolek Justyna, Wiatr Elzbieta, Gawryluk Dariusz, Martusewicz-Boros Magdalena Maria, Orlowski Tadeusz Maria, Dziedzic Dariusz, Polubiec-Kownacka Malgorzata, Oniszh Karina, Langfort Renata, Roszkowski-Sliz Kazimierz
From the Third Department of Pneumonology, and Department of Thoracic Surgery, and Department of Radiology, and Department of Pathology, National Research Institute of Tuberculosis and Lung Diseases, Warsaw, Poland.
J. Fijolek, PhD, Third Department of Pneumonology, National Research Institute of Tuberculosis and Lung Diseases; E. Wiatr, Professor, Third Department of Pneumonology, National Research Institute of Tuberculosis and Lung Diseases; D. Gawryluk, PhD, Third Department of Pneumonology, National Research Institute of Tuberculosis and Lung Diseases; M.M. Martusewicz-Boros, PhD, Third Department of Pneumonology, National Research Institute of Tuberculosis and Lung Diseases; T.M. Orlowski, Professor, Department of Thoracic Surgery, National Research Institute of Tuberculosis and Lung Diseases; D. Dziedzic, PhD, Department of Thoracic Surgery, National Research Institute of Tuberculosis and Lung Diseases; M. Polubiec-Kownacka, PhD, Department of Thoracic Surgery, National Research Institute of Tuberculosis and Lung Diseases; K. Oniszh, PhD, Department of Radiology, National Research Institute of Tuberculosis and Lung Diseases; R. Langfort, Professor, Department of Pathology, National Research Institute of Tuberculosis and Lung Diseases; K. Roszkowski-Sliz, Professor, Third Department of Pneumonology, National Research Institute of Tuberculosis and Lung Diseases.
J Rheumatol. 2016 Nov;43(11):2042-2048. doi: 10.3899/jrheum.151355. Epub 2016 Sep 15.
An analysis of subglottic stenosis (SGS) occurrence frequency in patients with granulomatosis with polyangiitis (GPA) based on the time of appearance of clinical symptoms, and an assessment of treatment effectiveness, in particular with the intratracheal dilation-injection technique (IDIT).
Review and treatment with IDIT of 34 patients with SGS associated with GPA.
SGS developed in 34 of 250 patients with GPA (13.6%) and was not reflective of disease activity in the organs in 15 of 34 patients (44%): 11 cases after and 4 cases during immunosuppressive therapy (IST) when patients did not have organ symptoms. All patients underwent IDIT and in total, the treatment resulted in immediate improvement. In addition, in 21 cases, IST was applied because of other organ involvement or of the lack of longterm efficacy of IDIT. The median time of response was 37 months and the median interval between sessions was 5 months. None of the patients required tracheostomy after beginning IDIT in our hospital.
SGS often occurs independently of other features of active GPA. IDIT is a safe and effective technique in the treatment of GPA-related SGS. It should be performed in all patients with GPA who develop significant SGS and in those with multiorgan disease concomitantly with IST. In patients with isolated SGS, IDIT also makes IST and tracheostomy unnecessary.
基于临床症状出现时间分析肉芽肿性多血管炎(GPA)患者声门下狭窄(SGS)的发生频率,并评估治疗效果,尤其是气管内扩张注射技术(IDIT)的治疗效果。
回顾性分析34例与GPA相关的SGS患者并采用IDIT进行治疗。
250例GPA患者中有34例发生SGS(13.6%),34例患者中有15例(44%)的SGS与器官疾病活动无关:11例发生在免疫抑制治疗(IST)后,4例发生在IST期间,此时患者无器官症状。所有患者均接受了IDIT治疗,总体而言,治疗后症状立即改善。此外,21例患者因其他器官受累或IDIT缺乏长期疗效而接受IST治疗。反应的中位时间为37个月,治疗间隔的中位时间为5个月。在我院开始IDIT治疗后,无一例患者需要气管造口术。
SGS常独立于活动性GPA的其他特征出现。IDIT是治疗GPA相关SGS的一种安全有效的技术。所有发生严重SGS的GPA患者以及伴有多器官疾病的患者均应在接受IST的同时进行IDIT治疗。对于孤立性SGS患者,IDIT也可避免IST和气管造口术。