scholarly journals Life-Threatening Subglottic Stenosis of Granulomatosis with Polyangiitis: A Case Report

Medicina ◽  
2021 ◽  
Vol 57 (5) ◽  
pp. 423
Author(s):  
Jin An ◽  
Jae-Won Song
Keyword(s):  
Immunosuppressive Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Granulomatous Inflammation ◽  
Subglottic Stenosis ◽  
Emergency Tracheostomy ◽  
Intralesional Steroid ◽  
Intralesional Steroid Injection ◽  
Life Threatening ◽  
Carbon Dioxide Co2 ◽  
Laryngotracheal Resection

Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by necrotizing granulomatous inflammation. Subglottic stenosis, which is defined as narrowing of the airway below the vocal cords, has a frequency of 16–23% in GPA. Herein, we present the case of a 39-year-old woman with subglottic stenosis manifesting as life-threatening GPA, which was recurrent under systemic immunosuppressive therapy. The patient underwent an emergency tracheostomy, intratracheal intervention, such as carbon dioxide (CO2) laser surgery and intralesional steroid injection via laryngomicroscopic surgery, and laryngotracheal resection with remodeling. Severe subglottic stenosis treatment requires active intratracheal intervention, surgery, and systemic immunosuppressive therapy.

scholarly journals Intralesional Steroid Injection for Primary Chalazion in Adults

2020 ◽  
Vol 5 (3) ◽  
Keyword(s):  
Triamcinolone Acetonide ◽  
Treatment Option ◽  
Granulomatous Inflammation ◽  
Meibomian Gland ◽  
Alternative Treatment Option ◽  
Vertical Incision ◽  
Intralesional Steroid ◽  
Intralesional Steroid Injection ◽  
Upper Lid ◽  
Gland Duct

Background: Chalazion (Meibomian cyst) a chronic lipo-granulomatous inflammation seconday to retention of sebum caused by obstruction of Meibomian gland duct of unknown cause. It is manifested by localized painless swelling in upper or lower lid usually points towards conjunctival side of lid (to be differentiated from stye). Vary in presentation from a small painless nodule self-limiting to a painful lid swelling complicated by corneal astigmatism and mechanical ptosis. Treatment option in case 2ry infection is hot compresses and topical antibiotic and if large surgery excision (vertical incision into the tarsal gland from conjunctiva surface). Injection of steroid (Triamcinolone) inside Chalazion considered as alternative treatment option with high success rate particularly in patients where incision and curette is difficult to perform due to poor patient’s cooperation. Or risk of lacrimal passage injury when the lesion located near to it... The aim of this study is to assess the effectiveness of intralesional triamcinolone acetonide (TA) injection for the treatment of primary chalazion in adults. Methods: 24 adult Patients mean age 38.6 ± 13.2 years with primary chalazion subjected to intralesional triamcinolone injection as management of primary chalazion. 0.05 to 0.15mL of triamcinolone injected into the center of the chalazion transconjunctivally in three patients the eversion of the upper lid were difficult and the injection was given transcutaneous. Result: 15 females and 9 males were included in this study. right eye was more affected than the left side14/10 respectively in all cases chalazion locate in the upper lid, mean chalazion size 0.74 ± 0.4 mm², time for complete resolution of the chalazion17.5 ± 12.0 days. There were no significant complications reported from this procedure Conclusion: Intralesional triamcinolone acetonide (TA) injection for the treatment of primary chalazion in adult was effective and without any significant complications.

scholarly journals Subglottic Stenosis in Granulomatosis With Polyangiitis: The Role of Laryngotracheal Resection

2018 ◽  
Vol 105 (1) ◽  
pp. 249-253 ◽  
Author(s):  
Christina L. Costantino ◽  
John L. Niles ◽  
Cameron D. Wright ◽  
Douglas J. Mathisen ◽  
Ashok Muniappan
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Subglottic Stenosis ◽  
Laryngotracheal Resection

Intratracheal Dilation-injection Technique in the Treatment of Granulomatosis with Polyangiitis Patients with Subglottic Stenosis

2016 ◽  
Vol 43 (11) ◽  
pp. 2042-2048 ◽  
Author(s):  
Justyna Fijolek ◽  
Elzbieta Wiatr ◽  
Dariusz Gawryluk ◽  
Magdalena Maria Martusewicz-Boros ◽  
Tadeusz Maria Orlowski ◽  
...  
Keyword(s):  
Disease Activity ◽  
Immunosuppressive Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Treatment Effectiveness ◽  
Clinical Symptoms ◽  
Subglottic Stenosis ◽  
Injection Technique ◽  
Effective Technique ◽  
Multiorgan Disease ◽  
Median Interval

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.

scholarly journals Systemic safety of serial intralesional steroid injection for subglottic stenosis

2018 ◽  
Vol 129 (7) ◽  
pp. 1634-1639 ◽  
Author(s):  
Jonathan Woliansky ◽  
Debra Phyland ◽  
Paul Paddle
Keyword(s):  
Steroid Injection ◽  
Subglottic Stenosis ◽  
Intralesional Steroid ◽  
Intralesional Steroid Injection

scholarly journals Wegener’s granulomatosis: surgical repair of severe subglottic stenosis

2020 ◽  
Vol 12 (4) ◽  
pp. 119-120
Author(s):  
Isabel Costa ◽  
António Lim ◽  
Cátia Azevedo ◽  
Fernando Mar ◽  
Berta Rodrigues ◽  
...  
Keyword(s):  
Surgical Procedures ◽  
Wegener’S Granulomatosis ◽  
Granulomatosis With Polyangiitis ◽  
Surgical Repair ◽  
Balloon Dilation ◽  
Wegener's Granulomatosis ◽  
Subglottic Stenosis ◽  
Transoral Laser Microsurgery ◽  
Atypical Form ◽  
Life Threatening

Laryngeal and subglottic stenosis are present in about 20% of patients with granulomatosis with polyangiitis. This atypical form of the disease can be severe and life-threatening. In what concerns to treatment, subglottic lesions are less responsive to conventional systemic therapy and interventional and surgical procedures should be considered. The authors present a case of a 30-year-old woman with diagnosis of Wegener’s granulomatosis who presented to our department with dyspnea and stridor due to a severe subglottic stenosis, requiring an emergent tracheostomy. The obstructive defect was electively and effectively repaired through a transoral laser microsurgery using local application prednisolone and mitomycin C followed by balloon dilation.

scholarly journals SAT0267 ROLE OF AGGRESSIVE IMMUNOSUPPRESSION ON SUBGLOTTIC STENOSIS IN GRANULOMATOSIS WITH POLYANGIITIS: RETROSPECTIVE ANALYSIS OF A MONOCENTRIC COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1076.1-1077
Author(s):  
L. Moroni ◽  
L. Giudice ◽  
G. A. Ramirez ◽  
S. Sartorelli ◽  
A. Cariddi ◽  
...  
Keyword(s):  
Medical Treatment ◽  
Clinical Characteristics ◽  
Granulomatosis With Polyangiitis ◽  
Balloon Catheter ◽  
Subglottic Stenosis ◽  
Damage Accrual ◽  
The Mean ◽  
The One

Background:Subglottic stenosis (SGS) is defined as airway narrowing below the vocal cords and is a common and potentially life-threatening manifestation of Granulomatosis with Polyangiitis (GPA), with an estimated prevalence of 16-23% (1). Balloon catheter dilation is effective in GPA-related SGS, but relapses are frequent. Little is known about the role of immunosuppression in this setting.Objectives:to analyse the clinical characteristics of a monocentric GPA cohort, describe phenotype differences among patients with and without SGS and investigate the role of surgical and medical treatments on relapse risk and general outcome.Methods:Biopsy-proven patients with SGS were identified by review of medical charts among a cohort of patients with GPA, classified according to the algorithm of the European Medicine Agency (2). The clinical characteristics of patients with SGS were retrospectively collected over a median follow-up time of 15.9 years and compared to those of patients without SGS.Results:Fourteen patients with SGS-GPA were identified, with a female to male ratio of 1:1 and a prevalence of 29.2% among the cohort. The mean ± SD age at GPA onset was 30.8 ± 14.4 years, with a mean time from GPA diagnosis to SGS onset of 4.7 ± 4.2 years. ANCA were positive in 78.6% (54.0% anti-PR3, 18.1% anti-MPO and 27.9% IFI only). The mean Birmingham Vasculitis Activity Score (BVAS) at onset was 10.0 ± 5.6. The main clinical manifestations associated with SGS were crusty rhinitis (100%), sinusitis (78%), pulmonary disease (72.7%), otitis/mastoiditis (50%), glomerulonephritis (42.9%), orbital pseudotumor (28.6%). Six patients (42.9%) received medical treatment only, other six (42.9%) had one to three balloon dilations and two (14.2%) underwent four or more procedures. Eight patients had no SGS relapse (maximum one dilation) and they all received immunosuppression with rituximab (RTX), cyclophosphamide (CYC) or azathioprine (AZA). All patients who received no immunosuppression, methotrexate (MTX) or mycophenolate (MMF) had at least one relapse. Patients treated with MTX or MMF had a mean relapse-free survival of 13.1 months, which was comparable to the one of patients not receiving medical treatment (40.2 months; p=NS) and shorter than the one of patients receiving CYC or RTX (153.2 months; p=0.032). CYC use also inversely correlated with the number of surgical procedures (r=-0.691, p=0.006). Compared to patients without SGS (31 consecutive patients with at least 4 years of follow-up), patients with SGS-GPA had an earlier disease onset (mean age 30.8 vs 50.4 years; p<0.001), but with lower BVAS (mean 10.0 vs 15.3; p=0.013) and showed a higher prevalence of crusty rhinitis (100% vs 67.7%; p=0.019). No difference was observed in damage accrual over time between the two groups.Conclusion:Subglottic stenosis is highly prevalent in patients with GPA and may define a milder disease subset occurring more frequently in younger patients. MTX and MMF might be insufficient to prevent SGS relapses requiring balloon dilation. Aggressive immunosuppression (CYC or RTX) might have a non-redundant role in this setting and reduce the risk of relapses.References:[1]Quinn KA, et al. Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis. Rheumatology 2019; 58 (12), 2203-2211.[2]Watts R, et al. Development and validation of a consensus methodology for the classification of the ANCA associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66: 222-7.Disclosure of Interests:Luca Moroni: None declared, Laura Giudice: None declared, Giuseppe Alvise Ramirez: None declared, Silvia Sartorelli: None declared, adriana cariddi: None declared, Angelo Carretta: None declared, Enrica Bozzolo: None declared, Lorenzo Dagna Grant/research support from: The Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) received unresctricted research/educational grants from Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merk Sharp & Dohme, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI., Consultant of: Prof Lorenzo Dagna received consultation honoraria from Abbvie, Amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI.

scholarly journals OP0054 NEW ROLE FOR PROTEINASE 3 IN IL-16 BIOACTIVITY CONTROL IN GRANULOMATOSIS WITH POLYANGIITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 28.1-29
Author(s):  
A. Kerstein-Staehle ◽  
C. Alarcin ◽  
J. Luo ◽  
G. Riemekasten ◽  
P. Lamprecht ◽  
...  
Keyword(s):  
Cell Death ◽  
Western Blot ◽  
Disease Activity ◽  
Chronic Inflammation ◽  
Immune Cells ◽  
Peripheral Blood ◽  
Granulomatosis With Polyangiitis ◽  
Granulomatous Inflammation ◽  
Proteinase 3 ◽  
Sds Page

Background:The immunomodulatory cytokine IL-16 is increased in several inflammatory and autoimmune diseases1. IL-16 recruits and activates CD4+ immune cells such as T cells, dendritic cells, or monocytes. IL-16 is produced by various immune and non-immune cells, but synthesis and storage of IL-16 is regulated differentially depending on the cell type and stimulation. For its biological activity, IL-16 cleavage by caspase-3 is required1. Necrotizing granulomatous inflammation is a hallmark of granulomatosis with polyangiitis (GPA) with neutrophil dysregulation as a central driver of chronic inflammation and autoimmunity2. Earlier studies showed a correlation between increased serum IL-16 and disease parameters in AAV, including GPA3, but functional evidence for a direct link between IL-16 and neutrophils in granulomatous inflammation is missing so far.Objectives:In this study we aim to identify a functional link between increased IL-16, neutrophils, and the autoantigen proteinase 3 (PR3) with regard to chronic inflammation and autoimmunity in GPA.Methods:IL-16 was measured in sera of GPA patients (n = 40) and healthy controls (HC, n = 50) by ELISA and correlated with clinical features, such as disease activity (BVAS), creatinine, GFR, VDI and PR3-ANCA status. IL-16 protein expression was analyzed in peripheral blood mononuclear cells (PBMC) and polymorphonuclear cells (PMN) from GPA patients and HC (n = 5, each) by SDS-PAGE and western blot. Binding affinity of recombinant pro-IL-16 to native human PR3 was assessed by microscale thermophoresis. Cleavage of pro-IL-16 by active human PR3 was performed at various time points at 37°C. Cleavage products were analyzed by SDS-PAGE and western blot.Results:Circulating IL-16 was significantly increased in GPA patients compared to HC. Elevated IL-16 positively correlated with BVAS, creatinine, VDI and PR3-ANCA status and negatively correlated with GFR. In PMBC and PMN from GPA and HC we identified different expression patters of precursor and active forms of IL-16. In healthy PBMC we found high amounts of precursor (80kD), pro-IL-16 (55kD) and active IL-16 (17kD). In contrast, PBMC from GPA patients had lower amounts of pro-IL-16 and no active IL-16, indicating activation and secretion of IL-16 due to inflammatory stimulation, as shown earlier5. In GPA PMN we detected no precursor IL-16, but pro-IL-16 and its active form, in contrast to very low amounts of all IL-16 forms in healthy PMN. Processing and release of IL-16 in neutrophils has been linked to apoptosis and secondary necrosis5. By interaction studies we demonstrated direct binding of pro-IL-16 to PR3 with a Kd of 10 nM. In a subsequent cleavage assay we confirmed IL-16 processing by PR3 in a time-dependent manner.Conclusion:Correlation of serum IL-16 with clinical features of GPA suggests that IL-16 is associated with markers of disease activity, tissue damage and autoreactivity. We showed that PBMC and PMN represent a source of IL-16 in GPA. By the identification of PR3 as an additional IL-16-activating enzyme we could demonstrate a potential link between excessive PR3 expression, cell death and IL-16-dependent mechanisms, contributing to chronic granulomatous inflammation and autoimmunity in GPA.References:[1]Glass, W. G. et al. Not-so-sweet sixteen: The role of IL-16 in infectious and immune-mediated inflammatory diseases. J. Interf. Cytokine Res. 26, 511–520 (2006).[2]Millet, A. et al. Proteinase 3 on apoptotic cells disrupts immune silencing in autoimmune vasculitis. J. Clin. Invest. 125, 4107–4121 (2015).[3]Yoon, T. et al. Serum interleukin-16 significantly correlates with the Vasculitis Damage Index in antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Res. Ther. 22, 1–6 (2020).[4]Elssner, A. et al. IL-16 Is Constitutively Present in Peripheral Blood Monocytes and Spontaneously Released During Apoptosis. J. Immunol. 172, 7721–7725 (2004).[5]Roth, S. et al. Secondary necrotic neutrophils release interleukin-16C and macrophage migration inhibitory factor from stores in the cytosol. Cell Death Discov. 1, 15056 (2015).Disclosure of Interests:None declared

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