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Anastomotic airway complications, including the dehiscence of the bronchial anastomosis, are a severe cause of morbidity after lung transplantation. We present a case of dehiscence treated by placing an uncovered metal stent into the main bronchus. We usually use this procedure for bronchial stenosis, but in this case, the stent favored the growth of granulation tissue and so the closure of the dehiscence. This procedure was minimally invasive and may be an alternative to an open repair, without precluding open repair in case of failure. [ABSTRACT FROM AUTHOR]

Abstract: In September 2005, the 3rd OPBG/Mayo Clinic Joint Conference was held in Rome to highlight current views in paediatric medicine. After an overview of the various pathologies, the Otolaryngology Unit of the Bambino Gesù Children''s Hospital of Rome described their own experience with endoscopic surgery for the treatment of upper airway diseases between 2002 and 2004. During this period, 1500 paediatric endoscopies were performed for obstructive upper airway disease. A total of 80% of patients were under 2 years of age. The aetiological causes of upper airway obstruction most frequently cited were: foreign bodies (the most frequent reason for urgent endoscopic treatment), choanal atresia, severe laryngomalacia, laryngeal cysts, neurological lesions (vocal fold paralysis), laryngeal clefts, laryngeal haemangiomas, recurrent respiratory papilloma, tracheoesophageal fistula, tracheal granuloma and tubercular adenobronchial fistula. In all the patients, stridor disappeared following surgery. No complications, such as aspiration or supraglottic stenosis, were identified and no revision surgeries were required. Stenting was necessary in only three cases. This study described the OPBG management of paediatric airway obstruction and outcomes during a 3-year period and was the starting point for reflection on this topic. [Copyright &y& Elsevier]

Objective: Previous studies suggested that distinct phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) could be determined by the presence or absence of antineutrophil cytoplasmic antibodies (ANCA), reflecting predominant vasculitic or eosinophilic processes, respectively. This study explored whether ANCA-based clusters or other clusters can be identified in EGPA., Methods: This study used standardized data of 15 European centers for patients with EGPA fulfilling widely accepted classification criteria. We used multiple correspondence analysis, hierarchical cluster analysis, and a decision tree model. The main model included 10 clinical variables (musculoskeletal [MSK], mucocutaneous, ophthalmological, ENT, cardiovascular, pulmonary, gastrointestinal, renal, central, or peripheral neurological involvement); a second model also included ANCA results., Results: The analyses included 489 patients diagnosed between 1984 and 2015. ANCA were detected in 37.2% of patients, mostly perinuclear ANCA (85.4%) and/or antimyeloperoxidase (87%). Compared with ANCA-negative patients, those with ANCA had more renal ( P < 0.001) and peripheral neurological involvement ( P = 0.04), fewer cardiovascular signs ( P < 0.001), and fewer biopsies with eosinophilic tissue infiltrates ( P = 0.001). The cluster analyses generated 4 (model without ANCA) and 5 clusters (model with ANCA). Both models identified 3 identical clusters of 34, 39, and 40 patients according to the presence or absence of ENT, central nervous system, and ophthalmological involvement. Peripheral neurological and cardiovascular involvement were not predictive characteristics., Conclusion: Although reinforcing the known association of ANCA status with clinical manifestations, cluster analysis does not support a complete separation of EGPA in ANCA-positive and -negative subsets. Collectively, these data indicate that EGPA should be regarded as a phenotypic spectrum rather than a dichotomous disease., (Copyright © 2023 by the Journal of Rheumatology.)

In this work, we investigate the correlation between ragweed pollen concentration and conjunctival, nasal, and asthma symptom severity in patients allergic to ragweed pollen using ambient pollen exposure in the Milan area during the 2014 ragweed season We calculate the pollen/symptom thresholds and we assess the effectiveness of ragweed allergen immunotherapy (AIT). A total of 66 participants allergic to ragweed (Amb a 1) were enrolled in the study and divided into two groups: AIT treated (24) and no AIT treated (42). Pollen counts and daily symptom/medication patient diaries were kept. Autoregressive distributed lag models were used to develop predictive models of daily symptoms and evaluate the short-term effects of temporal variations in pollen concentration on the onset of symptoms. We found significant correlations between ragweed pollen load and the intensity of symptoms for all three symptom categories, both in no AIT treated (τ = 0.341, 0.352, and 0.721; and ρ = 0.48, 0.432, and 0.881; p-value < 0.001) and in AIT treated patients ([Formula: see text]= 0.46, 0.610, and 0.66; and ρ = 0.692, 0.805, and 0.824; p-value < 0.001). In both groups, we observed a positive correlation between the number of symptoms reported and drug use. Mean symptom levels were significantly higher in no AIT treated than in AIT treated patients (p-value < 0.001) for all symptom categories. Pollen concentration thresholds for the four symptom severity levels (low, medium-low, medium-high and high) were calculated. Ragweed pollen concentration is predictive of symptom severity in patients with a ragweed (Amb a 1) allergy. Patients treated with AIT had significantly reduced mean symptom levels compared to those without AIT., (© 2022. The Author(s).)

Objective: To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA., Methods: Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease., Results: The study population included 157 patients (mean age 49.4±14.1), with a follow-up of 7.4±6.4years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p=0.005) and with the presence of ANCA (p<0.001). Overall, 59% of patients had polyangiitis as defined by definite vasculitis, strong surrogate of vasculitis, mononeuritis multiplex, and/or ANCA with at least one systemic manifestation other than ENT or respiratory. Patients with polyangiitis had more systemic manifestations including arthralgias (p=0.02) and renal disease (p=0.024), had higher peripheral eosinophilia (p=0.027), and a trend towards less myocarditis (p=0.057). Using predefined criteria of vasculitis and surrogates of vasculitis, ANCA alone were found to be insufficient to categorise patients with vasculitis features., Conclusion: We suggest a revised nomenclature and definition for EGPA and a new proposed entity referred to as hypereosinophilic asthma with systemic (non vasculitic) manifestations., (Copyright © 2016 Elsevier B.V. All rights reserved.)

The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study population included 157 patients (mean±sd age 49.4±14.1 years), with a mean±sd blood eosinophil count of 7.4±6.4×10 9  L -1 at diagnosis. There was a mean±sd of 11.8±18.2 years from the onset of asthma to the diagnosis of EGPA, of 1.4±8.4 years from the first onset of peripheral eosinophilia to the diagnosis of EGPA, and of 7.4±6.4 years from EGPA diagnosis to the final visit. Despite inhaled and oral corticosteroid treatment, the severity of asthma increased 3-6 months before the onset of the systemic manifestations. Asthma was severe in 57%, 48%, and 56% of patients at diagnosis, at 3 years, and at the final visit, respectively. Persistent airflow obstruction was present in 38%, 30%, and 46% at diagnosis, at 3 years, and at the final visit, respectively.In EGPA, asthma is severe, antedates systemic manifestations by a mean of 12 years, and progresses to long-term persistent airflow obstruction despite corticosteroids in a large proportion of patients, which affects long-term management and morbidity., (Copyright ©ERS 2016.)

Objective: To develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (EGPA)., Methods: The EGPA Consensus Task Force experts comprised 8 pulmonologists, 6 internists, 4 rheumatologists, 3 nephrologists, 1 pathologist and 1 allergist from 5 European countries and the USA. Using a modified Delphi process, a list of 40 questions was elaborated by 2 members and sent to all participants prior to the meeting. Concurrently, an extensive literature search was undertaken with publications assigned with a level of evidence according to accepted criteria. Drafts of the recommendations were circulated for review to all members until final consensus was reached., Results: Twenty-two recommendations concerning the diagnosis, initial evaluation, treatment and monitoring of EGPA patients were established. The relevant published information on EGPA, antineutrophil-cytoplasm antibody-associated vasculitides, hypereosinophilic syndromes and eosinophilic asthma supporting these recommendations was also reviewed., Discussion: These recommendations aim to give physicians tools for effective and individual management of EGPA patients, and to provide guidance for further targeted research., (Copyright © 2015 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.)

HLA-DRB4 gene is associated with Churg-Strauss syndrome (CSS), a systemic eosinophilic vasculitis with a prodromal phase characterized by severe asthma, eosinophilia, nasal polyposis, and sinusitis. Aim of this study was to evaluate if the presence of HLA-DRB4 in asthmatic patients is associated with a clinical picture resembling that of the prodromal phase of CSS. HLA-DRB1 was determined in a cohort of 159 asthmatic patients and its frequency was compared with that of 1808 blood donors. HLA-DRB4 presence/absence was correlated with clinical features, including sinusitis, nasal polyposis, eosinophils, antiasthmatic drugs, asthma severity, and pulmonary function tests. HLA-DRB4 gene was associated with severe persistent asthma before treatment (P < 0.02), near fatal or severe hypoxemic asthma (P < 0.01), sinusitis (P < 0.01), nasal polyposis (P < 0.01), number of patients with eosinophils >1000/ μ l: (P < 0.05), need of beclomethasone >1000-2000  μ g/daily (P < 0.001), use of a third controller (P < 0.05), and oral prednisone (P < 0.02). HLA-DRB4 gene is associated in asthmatic patients with a clinical picture characterized by asthma severity, sinusitis, nasal polyposis, and eosinophilia closely resembling that of the prodromal phase of CSS and might be useful to suspect corticosteroids-masked cases of CSS.

Background: The asthmatic-prodromal phase of Churg-Strauss syndrome (CSS) is usually considered allergic, but data about the involved allergens are scarce. The aim of our work was to examine the prevalence of allergy in a group of CSS patients and in two control groups of persistent asthmatic subjects selected for eosinophilia >10% [first control group patients (CGP1)] and eosinophils <6% [second control group patients (CGP2)]., Methods: The respiratory symptoms, and the results of prick test and/or RAST for the common allergens, performed before the vasculitic phase in 51 CSS, were retrospectively evaluated and compared with those of 46 CGP1 and 50 CGP2., Results: 31.4% of CSS vs 67.4% of CGP1 (P = 0.0004) and vs 58.0% CGP2 (P = 0.007) were allergic. The number of subjects with seasonal allergies was lower in CSS vs CGP1 (P = 0.0069) and vs CGP2 (P = 0.0002). The number of perennial allergies was significantly higher in CSS than in both control groups (CSS vs CGP1, P = 0.0108; CSS vs CGP2, P = 0.0079). The subjects allergic to Dermatophagoides were prevalent in CSS vs CGP1 (P = 0.0045) but not vs CGP2., Conclusions: The evidence of allergy, considered as the demonstration of specific IgE consistent with the clinical history, is present in less than one-third of CSS and the higher prevalence of seasonal allergies in the controls disagrees with persistent asthma. Allergy may be only one of several mechanisms triggering exacerbation of asthma or supporting chronic airway inflammation as in asthma in general. Alternatively, unidentified allergens may play a role.

Objective: To explore the association between HLA alleles and Churg-Strauss syndrome (CSS), and to investigate the potential influence of HLA alleles on the clinical spectrum of the disease., Methods: Low-resolution genotyping of HLA-A, HLA-B, and HLA-DR loci and genotyping of TNFA -238A/G and TNFA -308A/G single-nucleotide polymorphisms were performed in 48 consecutive CSS patients and 350 healthy controls., Results: The frequency of the HLA-DRB1*07 allele was higher in the CSS patients than in controls (27.1% versus 13.3%; chi(2) = 12.64, P = 0.0003, corrected P [P(corr)] = 0.0042, odds ratio [OR] 2.42, 95% confidence interval [95% CI] 1.47-3.99). The HLA-DRB4 gene, present in subjects carrying either HLA-DRB1*04, HLA-DRB1*07, or HLA-DRB1*09 alleles, was also far more frequent in patients than in controls (38.5% versus 20.1%; chi(2) = 16.46, P = 0.000058, P(corr) = 0.000232, OR 2.49, 95% CI 1.58-3.09). Conversely, the frequency of the HLA-DRB3 gene was lower in patients than in controls (35.4% versus 50.4%; chi(2) = 7.62, P = 0.0057, P(corr) = 0.0228, OR 0.54, 95% CI 0.35-0.84). CSS has 2 major clinical subsets, antineutrophil cytoplasmic antibody (ANCA)-positive, with features of small-vessel vasculitis, and ANCA-negative, in which organ damage is mainly mediated by tissue eosinophilic infiltration; analysis of HLA-DRB4 in patients categorized by different numbers of vasculitic manifestations (purpura, alveolar hemorrhage, mononeuritis multiplex, rapidly progressive glomerulonephritis, and constitutional symptoms) showed that its frequency strongly correlated with the number of vasculitis symptoms (P for trend = 0.001)., Conclusion: These findings indicate that HLA-DRB4 is a genetic risk factor for the development of CSS and increases the likelihood of development of vasculitic manifestations of the disease.