Justyna Fijolek1,2, Elzbieta Wiatr3,4, Dariusz Gawryluk3,4, Magdalena Maria Martusewicz-Boros3,4, Tadeusz Maria Orlowski3,4, Dariusz Dziedzic3,4, Malgorzata Polubiec-Kownacka3,4, Karina Oniszh3,4, Renata Langfort3,4, Kazimierz Roszkowski-Sliz3,4. 1. 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. jfijolek@op.pl. 2. 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. jfijolek@op.pl. 3. 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. 4. 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.
Abstract
OBJECTIVE: 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). METHODS: Review and treatment with IDIT of 34 patients with SGS associated with GPA. RESULTS: 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. CONCLUSION: 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.
OBJECTIVE: 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). METHODS: Review and treatment with IDIT of 34 patients with SGS associated with GPA. RESULTS: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. CONCLUSION: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.
Entities:
Keywords:
GRANULOMATOSIS WITH POLYANGIITIS; SUBGLOTTIC STENOSIS; TREATMENT
Authors: Justyna Fijolek; E Wiatr; V Petroniec; E Augustynowicz-Kopec; M Bednarek; D Gawryluk; K Roszkowski-Sliz Journal: Clin Rheumatol Date: 2019-07-23 Impact factor: 2.980
Authors: Thomas Kofler; Thomas Daikeler; Spasenija Savic Prince; Yvonne Holzmann; Jens Bremerich; Michael Tamm; Kathleen Jahn Journal: Respir Med Case Rep Date: 2018-05-19