Effect of influenza vaccinations on immune response and serum eotaxin level in patients with allergic bronchial asthma.

BACKGROUND: One of the most promising markers of allergic inflammation is eotaxin, which has a selective influence on the migration of eosinophils. Its serum content significantly correlates with the intensity of allergic symptoms, so it might be interesting to know whether vaccination has any influence on serum expression of this chemokine. AIMS: Comparison of the humoral response to influenza vaccine and post-vaccination changes in the serum eotaxin level in patients with allergic bronchial asthma and healthy controls. METHODS: Forty-two asthmatics and 45 healthy individuals were vaccinated with a single dose of influenza subunit vaccine (Influvac). The serum eotaxin level and the antibody response to haemagglutinin (HI) and neuraminidase (NI) glycoproteins were measured before and after vaccination. RESULTS: A significant increase of geometric mean titres of HI and NI was observed in both groups. There were no significant differences between the groups in meanfold increase of HI and NI titres, response rate and protective level of HI. After vaccination, a significant decrease of the mean serum eotaxin value was observed in patients with asthma (149.4 +/- 71.0 versus 125.1 +/- 67.0, p= 0.0017), while no similar effect was present in healthy individuals (153.4 +/- 56.9 versus 159.3 +/- 54.4, p= 0.5). CONCLUSIONS: The results indicate that in patients with allergic bronchial asthma influenza vaccinations assure efficient protective antibody level and modulate the serum level of eotaxin

Development and Implementation of the AIDA International Registry for Patients With Undifferentiated Systemic AutoInflammatory Diseases

Objective: This paper points out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients affected by Undifferentiated Systemic AutoInflammatory Diseases (USAIDs). Methods: This is an electronic registry employed for real-world data collection about demographics, clinical, laboratory, instrumental and socioeconomic data of USAIDs patients. Data recruitment, based on the Research Electronic Data Capture (REDCap) tool, is designed to obtain standardized information for real-life research. The instrument is endowed with flexibility, and it could change over time according to the scientific acquisitions and potentially communicate with other similar tools; this platform ensures security, data quality and data governance. Results: The focus of the AIDA project is connecting physicians and researchers from all over the world to shed a new light on heterogeneous rare diseases. Since its birth, 110 centers from 23 countries and 4 continents have joined the AIDA project. Fifty-four centers have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 179 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry is collecting baseline and follow-up data using 3,769 fields organized into 23 instruments, which include demographics, history, symptoms, trigger/risk factors, therapies, and healthcare information access for USAIDs patients. Conclusions: The development of the AIDA International Registry for USAIDs patients will facilitate the online collection of real standardized data, connecting a worldwide group of researchers: the Registry constitutes an international multicentre observational groundwork aimed at increasing the patient cohort of USAIDs in order to improve our knowledge of this peculiar cluster of autoinflammatory diseases

Data_Sheet_1_Case report: VEXAS as an example of autoinflammatory syndrome in pulmonology clinical practice.docx

Lung involvement is not widely recognized as a complication of auto-inflammatory diseases. We present a broad approach to diagnose a severe form of autoinflammatory syndrome in an adult male patient. A 63-year-old Caucasian male presented with recurrent episodes of high fever, interstitial lung infiltration, and pleural effusion. Laboratory tests performed during the flares revealed lymphopenia and increased levels of C-reactive protein and ferritin. Broad diagnostic research on infections, connective tissue diseases, and malignancies yielded negative results. The patient’s symptoms promptly resolved upon the administration of glucocorticoids; however, they reappeared when the prednisone dose was reduced. All attempts to administer immunomodulatory and immunosuppressive medications were ineffective. During follow-up, autoinflammatory syndrome was suspected; however, no pathological variants of monogenic autoinflammatory diseases were identified by genome-exome sequencing. The patient did not respond to interleukin 1 blockade with anakinra. He died due to multi-organ failure, and his condition remained unresolved until the first reported description of vacuole, E1 enzyme, X-linked, autoinflammatory, and somatic syndrome (VEXAS). We describe the diagnostic traps and reasoning process involved in establishing that the patient’s symptoms were autoinflammatory in nature based on clinical symptoms, in addition to the proof of concept gained from genetic reevaluation and identification of pathogenic variants in the UBA1 gene. The aim of this review is to increase the awareness of VEXAS among pulmonologists. Genetic screening for UBA1 should be considered in patients with recurrent pneumonitis of unknown origin with elevated inflammatory markers and signs of cytopenia, especially if they require chronic steroids to control the disease. Respiratory manifestations are part of VEXAS; these may be dominant in the course of the disease and severe at presentation.</p

Development and implementation of the AIDA international registry for patients with Schnitzler's syndrome

The present paper describes the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry specifically dedicated to patients with Schnitzler's syndrome

Development and Implementation of the AIDA International Registry for Patients With VEXAS Syndrome

The aim of this paper is to present the AutoInflammatory Disease Alliance (AIDA) international Registry dedicated to Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome, describing its design, construction, and modalities of dissemination