scholarly journals The Liverpool Experience: The Role of Immunosuppression in treating Vasculitic Subglottic Stenosis

2021 ◽  
Author(s):  
Matthew Zammit ◽  
Vedika Dhunnoo ◽  
Andrew Kinshuck ◽  
Sarah Hardy ◽  
Janice Harper ◽  
...  
Keyword(s):  
Management Strategy ◽  
Granulomatosis With Polyangiitis ◽  
Early Recognition ◽  
Subglottic Stenosis ◽  
Systemic Immunosuppression ◽  
Free Interval ◽  
Key Points ◽  
Surgical Manipulation ◽  
Multi Disciplinary Team

Five Key Points • Subglottic stenosis (SGS) is the commonest tracheobronchial manifestation of granulomatosis with polyangiitis (GPA), with early recognition and treatment key to preventing its vasculitic progression and fibrosis. • Previous studies have shown SGS to be the first feature of GPA in 4% of cases. It is not uncommon to see negative biochemical (10% ANCA negative) and negative histological biopsies (77% of head and neck specimens are negative). • Our management strategy emphasized rapid SGS-GPA treatment with limited surgical manipulation of the airway and systemic immunosuppression (IS) to prevent evolution of SGS & concurrent systemic vasculitic relapse. • In our study early multi-disciplinary team involvement to deliver induction IS in the presence of active SGS-GPA led to a procedure free interval (PFI) of 31.3 months. This is a significant increase compared to other published studies. • Nineteen percent (4/21) of the cohort did not require any surgical input following induction IS.

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.

The role of intratracheal dilation – Injection technique in treatment of granulomatosis with polyangiitis (GPA) patients with subglottic stenosis

2016 ◽  
Author(s):  
Justyna Fijolek ◽  
Elzbieta Wiatr ◽  
Dariusz Gawryluk ◽  
Magdalena Maria Martusewicz-Boros ◽  
Tadeusz Maria Orlowski ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Subglottic Stenosis ◽  
Injection Technique

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

Role of Serum β-HCG doubling in Diagnosis of Early Pregnancy and its Management Strategy

2020 ◽  
Vol 16 ◽  
Author(s):  
Divya Mirji ◽  
Shubha Rao ◽  
Akhila Vasudeva ◽  
Roopa P.S
Keyword(s):  
Ectopic Pregnancy ◽  
Early Pregnancy ◽  
Management Strategy ◽  
Transvaginal Ultrasound ◽  
Expectant Management ◽  
First Trimester ◽  
Intrauterine Pregnancy ◽  
Β Hcg ◽  
Pregnancy Of Unknown Location

Background: Pregnancy of unknown location (PUL) is defined as the absence of intrauterine or extrauterine sac and Beta Human Chorionic Gonadotropin levels (β-HCG) above the discriminatory zone of 1500 mIU/ml. It should be noted that PUL is not always an ectopic; however, by measuring the trends of serum β-HCG, we can determine the outcome of a PUL. Objective: This study aims to identify the various trends β-HCG levels in early pregnancy and evaluate the role of β-HCG in the management strategy. Methods: We conducted a prospective observational study of pregnant women suspected with early pregnancy. Cases were classified as having a pregnancy of unknown location (PUL) by transvaginal ultrasound and ß-HCG greater than 1000 mIU/ml. Expectant management was done until there was a definite outcome. All the collected data were analyzed by employing the chi-square test using SPSS version 20. Results: Among 1200 women who had early first trimester scans, 70 women who fulfilled our criteria of PUL and ß-HCG > 1000 mIU/ml were recruited in this study. In our study, the mean age of the participants was 30±5.6yrs, and the overall mean serum ß-HCG was 3030±522 mIU/ml. The most common outcome observed was an ectopic pregnancy, 47% in our study. We also found the rate of failing pregnancy was 27%, and that of intrauterine pregnancy (IUP) was 25%. Overall, in PUL patients diagnosed with ectopic pregnancy, 9% behaved like IUP, and 4% had an atypical trend in their ß-HCG. Those who had an IUP, 11% had a suboptimal increase in ß-HCG. Conclusion: PUL rate in our unit was 6%. Majority of the outcome of PUL was ectopic in our study. Every case of PUL should be managed based on the initial ß-HCG values, clinical assessments and upon the consent of the patient.

scholarly journals Gut microbiota profiles and characterization of cultivable fungal isolates in IBS patients

2021 ◽  
Author(s):  
Piero Sciavilla ◽  
Francesco Strati ◽  
Monica Di Paola ◽  
Monica Modesto ◽  
Francesco Vitali ◽  
...  
Keyword(s):  
Candida Albicans ◽  
Gut Microbiota ◽  
Healthy Subjects ◽  
Fungal Isolates ◽  
Key Points ◽  
Health Promoting ◽  
Irritable Bowel

Abstract Studies so far conducted on irritable bowel syndrome (IBS) have been focused mainly on the role of gut bacterial dysbiosis in modulating the intestinal permeability, inflammation, and motility, with consequences on the quality of life. Limited evidences showed a potential involvement of gut fungal communities. Here, the gut bacterial and fungal microbiota of a cohort of IBS patients have been characterized and compared with that of healthy subjects (HS). The IBS microbial community structure differed significantly compared to HS. In particular, we observed an enrichment of bacterial taxa involved in gut inflammation, such as Enterobacteriaceae, Streptococcus, Fusobacteria, Gemella, and Rothia, as well as depletion of health-promoting bacterial genera, such as Roseburia and Faecalibacterium. Gut microbial profiles in IBS patients differed also in accordance with constipation. Sequence analysis of the gut mycobiota showed enrichment of Saccharomycetes in IBS. Culturomics analysis of fungal isolates from feces showed enrichment of Candida spp. displaying from IBS a clonal expansion and a distinct genotypic profiles and different phenotypical features when compared to HS of Candida albicans isolates. Alongside the well-characterized gut bacterial dysbiosis in IBS, this study shed light on a yet poorly explored fungal component of the intestinal ecosystem, the gut mycobiota. Our results showed a differential fungal community in IBS compared to HS, suggesting potential for new insights on the involvement of the gut mycobiota in IBS. Key points • Comparison of gut microbiota and mycobiota between IBS and healthy subjects • Investigation of cultivable fungi in IBS and healthy subjects • Candida albicans isolates result more virulent in IBS subjects compared to healthy subjects

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 French recommendations for the management of Behçet’s disease

2021 ◽  
Vol 16 (S1) ◽  
Author(s):  
Isabelle Kone-Paut ◽  
◽  
Stéphane Barete ◽  
Bahram Bodaghi ◽  
Kumaran Deiva ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Initial Assessment ◽  
Diagnostic Process ◽  
Behcet's Disease ◽  
Therapeutic Modalities ◽  
Clinical Presentations ◽  
Key Points

AbstractBehçet’s disease (BD) is a systemic variable vessel vasculitis that involves the skin, mucosa, joints, eyes, arteries, veins, nervous system and gastrointestinal system, presenting with remissions and exacerbations. It is a multifactorial disease, and several triggering factors including oral cavity infections and viruses may induce inflammatory attacks in genetically susceptible individuals. BD vasculitis involves different vessel types and sizes of the vascular tree with mixed-cellular perivascular infiltrates and is often complicated by recurrent thrombosis, particularly in the venous compartment. Several new therapeutic modalities with different mechanisms of action have been studied in patients with BD. A substantial amount of new data have been published on the management of BD, especially with biologics, over the last years. These important therapeutic advances in BD have led us to propose French recommendations for the management of Behçet’s disease [Protocole National de Diagnostic et de Soins de la maladie de Behçet (PNDS)]. These recommendations are divided into two parts: (1) the diagnostic process and initial assessment; (2) the therapeutic management. Thirty key points summarize the essence of the recommendations. We highlighted the main differential diagnosis of BD according to the type of clinical involvement; the role of genetics is also discussed, and we indicate the clinical presentations that must lead to the search for a genetic cause.

The Role of Angioembolization in Hepatic Trauma

2020 ◽  
pp. 000313482097372
Author(s):  
Ali Cadili ◽  
Jonathan Gates
Keyword(s):  
Gold Standard ◽  
Management Strategy ◽  
Operative Management ◽  
Hepatic Trauma ◽  
Anatomy And Physiology ◽  
Liver Injuries ◽  
Hepatic Injuries ◽  
Non Operative Management ◽  
Type Of Injury

The liver is one of the most commonly injured solid organs in blunt abdominal trauma. Non-operative management is considered to be the gold standard for the care of most blunt liver injuries. Angioembolization has emerged as an important adjunct that is vital to the success of the non-operative management strategy for blunt hepatic injuries. This procedure, however, is fraught with some possible serious complications. The success, as well as rate of complications of this procedure, is determined by degree and type of injury, hepatic anatomy and physiology, and embolization strategy among other factors. In this review, we discuss these important considerations to help shed further light on the contribution and impact of angioembolization with regards to complex hepatic injuries.

scholarly journals The cypsela (achene) of Echinacea purpurea as a diffusion unit of a community of microorganisms

2021 ◽  
Vol 105 (7) ◽  
pp. 2951-2965
Author(s):  
Massimiliano Cardinale ◽  
Marian Viola ◽  
Elisangela Miceli ◽  
Teresa Faddetta ◽  
Anna Maria Puglia ◽  
...  
Keyword(s):  
Respiratory Infections ◽  
Active Role ◽  
Echinacea Purpurea ◽  
Endophytic Microorganisms ◽  
Host Membrane ◽  
Key Points ◽  
Endophytic Bacterial Community ◽  
Pharmaceutical Properties ◽  
Stimulation Of

AbstractEchinacea purpurea is a plant cultivated worldwide for its pharmaceutical properties, mainly related to the stimulation of the immune system in the treatment of respiratory infections. The cypselas (fruits) of E. purpurea were examined in order to investigate the presence, localization and potential function(s) of endophytic microorganisms. Electron and confocal microscopy observations showed that three different components of microorganisms were associated to cypselas of E. purpurea: (i) one endocellular bacterial component in the cotyledons, enclosed within the host membrane; (ii) another more generic bacterial component adhering to the external side of the perianth; and (iii) a fungal component inside the porous layer of the perianth, the woody and porous modified residual of the flower, in the form of numerous hyphae able to cross the wall between adjacent cells. Isolated bacteria were affiliated to the genera Paenibacillus, Pantoea, and Sanguibacter. Plate tests showed a general resistance to six different antibiotics and also to an antimicrobial-producing Rheinheimera sp. test strain. Finally, microbiome-deprived E. purpurea seeds showed a reduced ability to germinate, suggesting an active role of the microbiome in the plant vitality. Our results suggest that the endophytic bacterial community of E. purpurea, previously found in roots and stem/leaves, might be already carried at the seed stage, hosted by the cotyledons. A further microbial fungal component is transported together with the seed in the perianth of the cypsela, whose remarkable structure may be considered as an adaptation for fungal transportation, and could influence the capability of the seed to germinate in the soil.Key Points• The fruit of Echinacea purpurea contains fungi not causing any damage to the plant.• The seed cotyledons contain endocellular bacteria.• Seed/fruit deprived of the microbiome showed a reduced ability to germinate.

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