Adrenal function recovery after durable oral corticosteroid sparing with benralizumab in the PONENTE study

Background Oral corticosteroid (OCS) dependence among patients with severe eosinophilic asthma can cause adverse outcomes, including adrenal insufficiency. PONENTE's OCS reduction phase showed that, following benralizumab initiation, 91.5% of patients eliminated corticosteroids or achieved a final dosage ≤5 mg·day-1 (median (range) 0.0 (0.0-40.0) mg). Methods The maintenance phase assessed the durability of corticosteroid reduction and further adrenal function recovery. For ~6 months, patients continued benralizumab 30 mg every 8 weeks without corticosteroids or with the final dosage achieved during the reduction phase. Investigators could prescribe corticosteroids for asthma exacerbations or increase daily dosages for asthma control deteriorations. Outcomes included changes in daily OCS dosage, Asthma Control Questionnaire (ACQ)-6 and St George's Respiratory Questionnaire (SGRQ), as well as adrenal status, asthma exacerbations and adverse events. Results 598 patients entered PONENTE; 563 (94.1%) completed the reduction phase and entered the maintenance phase. From the end of reduction to the end of maintenance, the median (range) OCS dosage was unchanged (0.0 (0.0-40.0) mg), 3.2% (n=18/563) of patients experienced daily dosage increases, the mean ACQ-6 score decreased from 1.26 to 1.18 and 84.5% (n=476/563) of patients were exacerbation free. The mean SGRQ improvement (-19.65 points) from baseline to the end of maintenance indicated substantial quality-of-life improvements. Of patients entering the maintenance phase with adrenal insufficiency, 32.4% (n=104/321) demonstrated an improvement in adrenal function. Adverse events were consistent with previous reports. Conclusions Most patients successfully maintained maximal OCS reduction while achieving improved asthma control with few exacerbations and maintaining or recovering adrenal function

Clinical phenotypes of Italian and Spanish patients with α1-antitrypsin deficiency.

With the aim of providing better clinical characterisation of patients with α1-antitrypsin deficiency (AATD), we analysed the data of adult patients with severe AATD enrolled in the Spanish and Italian national registries. We assessed 745 subjects, 416 of whom were enrolled in the Spanish registry and 329 in the Italian registry. 57.2\% were male and 64.9\% were smokers or former smokers with a mean±sd age of 49.9±13.8 years. Most (81.2\%) were index cases, mainly having the PI*ZZ genotype (73.4\%), and the mean±sd diagnostic delay was 9.0±12.1 years. Patients with chronic bronchitis were younger, had better preserved lung function and lower tobacco consumption. Overlap patients (chronic obstructive pulmonary disease with asthma) were mainly females, more frequently never-smokers and received respiratory medications more often. 48\% of emphysema, 27.5\% of chronic bronchitis and 44.8\% of overlap subjects were receiving augmentation therapy. Compared with PI*ZZ patients (n=547), the PI*SZ (n=124) subjects were older at diagnosis and had more preserved lung function, despite a higher mean smoking consumption. Early diagnosis of AATD is still an unmet need. Augmentation therapy is administered to similar proportions of patients with different clinical phenotypes. PI*ZZ patients in both registries had more severe respiratory disease than those with PI*SZ, despite lower smoking levels

Clinical course of IPF in Italian patients during 12 months of observation: results from the FIBRONET observational study

none26nononePoletti, V., Vancheri, C., Albera, C., Harari, S., Pesci, A., Metella, R.R., Campolo, B., Crespi, G., Rizzoli, S., Vancheri, C., Tomassetti, S., Rottoli, P., Bocchino, M., Stanziola, A.A., Luppi, F., Sebastiani, A., Lacedonia, D., Vitulo, P., Tavanti, L., Vianello, A., Saetta, M., Marinari, S., Pirina, P., Valente, S., Oggionni, T., Gasparini, S.Poletti, V.; Vancheri, C.; Albera, C.; Harari, S.; Pesci, A.; Metella, R. R.; Campolo, B.; Crespi, G.; Rizzoli, S.; Vancheri, C.; Tomassetti, S.; Rottoli, P.; Bocchino, M.; Stanziola, A. A.; Luppi, F.; Sebastiani, A.; Lacedonia, D.; Vitulo, P.; Tavanti, L.; Vianello, A.; Saetta, M.; Marinari, S.; Pirina, P.; Valente, S.; Oggionni, T.; Gasparini, S

Effects of a structured educational intervention in moderate-to-severe elderly asthmatic subjects

Background: Adherence to inhaled drugs is linked to patients' satisfaction with their device, and an incorrect use can negatively affect the outcomes of asthma treatment. We speculated that this is particularly true in elderly asthmatic subjects.Aim: We performed a national pre-post interventional multicentre study, enrolling moderate-to-severe asthmatic subjects aged >= 65 years treated with fixed inhaled combination drugs by dry powder inhaler (DPI) or pressurized metered dose inhaler (pMDI). Adherence and critical errors were evaluated by means of validated questionnaires at first visit (V1) and after 3-6 months (V2). At V1, subjects underwent intensive training on the correct use of their device by physical demonstration.Results: A total of 411 asthmatics (F/M: 238/173, mean age +/- SD: 72 +/- 5 years) participated to the study. At V1, 50% of the study subjects showed an Asthma Control Test (ACT) score <= 19 despite GINA step 3 and 4 treatment, and 40% had experienced at least one severe asthma exacerbation in the previous year. Poor adherence to treatment was recorded in 43% of subjects, and at least one error in using the device was registered in 56% of subjects. At V2, available for 318 patients, both the percentage of individuals with poor adherence and with at least one critical error significantly decreased (from 46% to 25%, and from 49% to 25%, respectively; p < 0.001 for both comparisons) with a significant increase of the ACT score (from 19 +/- 4.9 to 20 +/- 4.0, p < 0.001).Conclusions: Asthma in the elderly is characterized by low levels of symptom control. Educational interventions are strongly advocated in this age group in order to increase adherence to treatment and inhaler techniques

Anterior Callosal Angle: A New Marker of Idiopathic Normal Pressure Hydrocephalus?

Objective: Diagnosing idiopathic normal pressure hydrocephalus (iNPH) still remains a clinical challenge. The callosal angle (CA) is a widely used neuroradiologic marker for iNPH. However, the relationship of the CA to clinical features has not been well investigated. We hypothesize that iNPH symptoms might better correlate with a variant of the CA (anterior callosal angle [ACA]). We aim to establish the validity of the ACA measurement for the diagnosis of iNPH and compare it with current radiologic parameters. Methods: The multidisciplinary BOLOGNA PRO-HYDRO Study Group performed a retrospective review of consecutive iNPH patients. Magnetic resonance imaging studies for these patients were collected, as well as magnetic resonance imaging studies from Alzheimer disease and healthy control patients. The CA, ACA, and Evans Index were measured by 2 blinded members of the study team based on magnetic resonance images for each of these populations. Results: The ACA shows high accuracy, sensitivity, and specificity in distinguishing iNPH patients from healthy control and Alzheimer disease patients. The optimal pathologic diagnostic cut-off value for the ACA is 119 degrees. The diagnostic accuracy of the ACA is not significantly different from the CA. Conclusions: The ACA could be a valid radiologic parameter in the diagnostic armamentarium for iNPH