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Background:Nintedanib (NTD) has been shown to reduce the rate of decline in Interstitial Lung Disease (ILD) in Systemic Sclerosis (SSc) following the positive results of the SENSCIS trial and sustained effects have been shown in the SENSCIS-ON study.Objectives:To analyse the predictors of response and NTD-related side effects at 1-year and efficacy and safety of NTD at 2-year in SSc-ILD in a real-life setting.Methods:The clinical data of SSc-ILD patients treated with NTD from 11 Italian SSc centres were retrospectively evaluated at 12 prior to NTD introduction; at baseline, and at 12 and 24 months after NTD introduction. The following parameters were recorded: SSc clinical features, concomitant therapies, NTD tolerability, pulmonary function tests (PFTs) and modified Rodnan skin score (mRSS). Progression was defined according to ATS definition (drop in predicted% FVC≥5% or DLCO≥10% or high-resolution chest tomography (HRCT) evolution and worsening of respiratory symptoms). Logistic regression analyses were performed to assess predictors of response at 1-year and predictors of NTD reduction/suspension.Results:136 SSc-ILD patients treated with NTD were identified, disease features are summarized in Table 1. A progressive phenotype, 12 months prior to NTD introduction, was observed in 92 (67%) of patients. In those who achieved 1-year follow-up (84 (61%) patients), the percentage of 1-year progressors was significantly lower compared to the previous 12 months (64% versus 30%, p<0.001). No significant change was observed for the mRSS at 12 months in dcSSc patients (7.5 ± 6.4 versus 6.1 ± 4.9, p=0.205). After a median time of 4 (2 – 8) months, NTD dose was reduced to 200 mg daily and maintained in 51 (37.5%) patients. In 19 (13.9%) patients, NTD was stopped after a median time of 3 (1-9) months. At logistic regression analysis only the presence of a diffuse cutaneous subset (OR 0.201 (0.001– 0.401)) was associated with 12-month progression-free response whereas the use of high-dose proton pump inhibitors (PPI) (OR 3.522 (1.057 – 11.0729)) was associated with NTD reduction/suspension. At 2 years, the percentage of progressors did not change compared to the first year (37%, p=0.465). The prevalence of NTD-related side-effects and causes of reduction and suspension are summarized in Figure 1. During the follow-up, 6 patients died after a median time of 10 (10 – 24) months.Table 1.Clinical and demographic characteristics of our multicenter cohort of SSc-ILD patients.Clinical and demographic characteristics136 patientsFemales, n (%)96 (70)Age (years), mean ± SD59.8 ± 12.0Disease duration (years), mean ± SD9.7 ± 6.8Cutaneous subset Diffuse, n (%) Limited, n (%) Sine scleroderma, n (%)Autoantibodies70 (51.1)51 (37.2)16 (11.7) Anti-topoisomerase I Ab, n (%)99 (72.3) Anti-RNA-polymerase III Ab, n (%)4 (2.9) Anti-centromere Ab, n (%) Anti-U1-RNP Ab, n (%) Anti-Th/T0, n (%) Anti-Ku, n (%)8 (5.8)5 (3.6)3 (2.4)4 (2.9)Organ involvement GI involvement, n (%) Myositis, n (%) Myocarditis, n (%) Scleroderma Renal Crisis, n (%) Digital Ulcer, n (%) Pulmonary Arterial Hypertension, n(%)108 (78.8)11 (8.0)16 (11.7)0 (0)50 (36.5)10 (7.3)Previous Therapies99 patients Mycophenolate mofetil, n (%)79 (79.8) Methotrexate, n (%)14 (14.1) Cyclophosphamide, n (%)29 (29.3) Azathioprine, n (%) Tocilizumab, n(%) Rituximab, n (%)23 (23.2)2 (2.0)31 (31.3)Concomitant Therapies122 patients (89.7%) Corticosteroids, n (%) Mycophenolate mofetil, n (%)76 (62.3)91 (74.6) Methotrexate, n (%)6 (4.9) Cyclophosphamide, n (%) Azathioprine, n (%) Tocilizumab, n (%) Rituximab, n (%)Baseline HRCT NSIP, n (%) UIP, n (%)0 (0)1 (0.8)10 (8.2)26 (21.3)118 patients80 (67.8)38 (32.2)N= number; SD= standard deviation; Ab= antibodies; GI= gastro-intestinal; NSIP= non-specific interstitial pneumonia; UIP= usual interstitial pneumonia.Figure 1.Nintedanib-related side effects.Conclusion:In a real-life clinical scenario, NTD, in combination with immunosuppressants, reduced the rate of progressors and this effect ins maintained at 2 years. A diffuse cutaneous subset seems a predictor of poorer response whereas the baseline use of high-dose PPI is a risk factor for poor drug tolerance.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:Corrado Campochiaro BI, BI, Jannsen, Novartis, Giacomo De Luca: None declared, Maria Grazia Lazzaroni: None declared, Giuseppe Armentaro: None declared, Barbara Ruaro: None declared, Anna Stanziola: None declared, Devis Benfaremo: None declared, Enrico De Lorenzis: None declared, Beatrice Moccaldi: None declared, Francesco Bonomi: None declared, Lorenzo Mattia Bianchessi: None declared, Florenzo Iannone: None declared, Fabio Cacciapaglia: None declared, Amelia Spinella: None declared, Serena Guiducci: None declared, Veronica Codullo: None declared, Silvia Laura Bosello: None declared, Giovanna Cuomo: None declared, Elisabetta Zanatta: None declared, Paola Confalonieri: None declared, Nicoletta Del Papa: None declared, Paolo Airo’: None declared, Gianluca Moroncini: None declared, Dilia Giuggioli: None declared, Lorenzo Dagna: None declared, Marco Matucci-Cerinic: None declared.

Background FIBRONET was an observational, multicentre, prospective cohort study investigating the baseline characteristics, clinical course of disease and use of antifibrotic treatment in Italian patients with idiopathic pulmonary fibrosis (IPF). Methods Patients aged ≥ 40 years diagnosed with IPF within the previous 3 months at 20 Italian centres were consecutively enrolled and followed up for 12 months, with evaluations at 3, 6, 9 and 12 months. The primary objective was to describe the clinical course of IPF over 12 months of follow-up, including changes in lung function measured by % predicted forced vital capacity (FVC% predicted). Results 209 patients (82.3% male, mean age 69.54 ± 7.43 years) were enrolled. Mean FVC% predicted was relatively preserved at baseline (80.01%). The mean time between IPF diagnosis and initiation of antifibrotic therapy was 6.38 weeks; 72.3% of patients received antifibrotic therapy within the first 3 months of follow-up, and 83.9% within 12 months of follow-up. Mean FVC% predicted was 80.0% at baseline and 82.2% at 12 months, and 47.4% of patients remained stable (i.e. had no disease progression) in terms of FVC% predicted during the study. Conclusions FIBRONET is the first prospective, real-life, observational study of patients with IPF in Italy. The short time between diagnosis and initiation of antifibrotic therapy, and the stable lung function between baseline and 12 months, suggest that early diagnosis and prompt initiation of antifibrotic therapy may preserve lung function in patients with IPF. Trial registration: NCT02803580

Background The query “are there animals at home?” is usually administered for collecting information on anamnesis. This modality to consider exposure to pet allergens constitutes a potential bias in epidemiological studies and in clinical practice. The aim of our study was to evaluate/quantify different modalities of exposure to cat/dog in inducing allergic sensitization. Methods Thirty Italian Allergy units participated in this study. Each centre was required to collect the data of at least 20 consecutive outpatients sensitized to cat/dog allergens. A standardized form reported all demographic data and a particular attention was paid in relieving possible modalities of exposure to cat/dog. Results A total 723 patients sensitized to cat/dog were recorded, 359 (49.65%) reported direct pet contact, 213 patients (29.46%) were pet owners, and 146 subjects (20.19%) were exposed to pets in other settings. Other patients were sensitized by previous pet ownership (150–20.75%) or indirect contact (103–14.25%), in 111 subjects (15.35%) any contact was reported. Conclusions Only 213 patients (29.46%) would be classified as “exposed to animals” and 510 (70.54%) as “not exposed” according to usual query. Our classification has shown that many “not-exposed” subjects (399–55.19%) were “really exposed”. The magnitude of exposure to pet allergens at home is not related exclusively to pet ownership. These considerations should be taken into account during the planning of epidemiological studies and in clinical practice for the management of pet allergic individuals.

Pulmonary hypertension (PH) is a serious disorder that worsens the course of chronic heart, lung or systemic diseases. However the need to discrimi- nate PH, a pathophysiological condition, from pul- monary arterial hypertension [...]

Adherence to medical therapies is a growing issue, so much so that the World Health Organization defined it as “a new pharmacological problem”. The main factors affecting compliance are: frequency of administration, rapid onset of action, role of device. The most severe consequence of non-adherence is the increased risk of poor clinical outcome, associated with worsening of the quality of life and increase in health-care expenditure. It appears crucial to identify those COPD patients who are “poorly or not at all compliant with their treatment”. In order to evaluate adherence to the medical therapy, several methods were proposed, the most effective of which turned out to be self-reports, i.e. simple, brief questionnaires (e.g. Morisky test). To increase the likelihood of quickly identifying non-compliant patients, it may be useful to administer a simple questionnaire to naïve subjects (for example, in the waiting room before an examination) including six specific items allowing to identify the patient’s key characteristics. Depending on the answers, patients who do not comply with their pharmacological treatment may be classified as belonging to 6 phenotypes. For patients who are already under treatment it might be useful to administer another short questionnaire during follow up examination. Once the risk of non-compliance is identified, four possible types of measures can be taken: prescription-related, educational, behavioral and complex combined measures (combination of two or more actions). Therefore, while it is clear that adherence in COPD is a critical issue, it is also obvious that raising awareness on the disease and improving cooperation among specialists, general practitioners, health-care professionals, and patients is the starting point at which this evolution should immediately begin. Each medication is able to foster good compliance with the therapy, and consequently to maximize the efficacy, by virtue of its specific inhaler and its own active ingredient.

Niemann-Pick disease type B is caused by a deficiency in acid sphingomyelise activity; among the six variants of Niemann-Pick disease known to date, it is the most frequently associated with lung involvement, a major cause of morbidity and mortality in this subtype in patients of all ages. Nevertheless, the vast majority of reports in the literature concern infantile forms, while less reported is, for several reasons, the onset in adults being consequently still poorly understood and characterized its clinical, radiographic and functiol manifestations. We report a case of a 37 years-old female patient affected by subtype B since she was an infant, operated for aortic valve replacement two years before and came to our attention for the onset of a worsening exertiol dyspnoea which proved, through a series of functiol tests and radiological exams, to be a consequence of the diffuse lung involvement by the metabolic disorder; we performed a review on this topic through a Medline search of all the available €œadult-onset€ case reports published since the first description in 1964, also considering the possible association between NPDB and, more generally lysosomal storage disorders, and the valvular disease, already suggested by several Authors in previous works.

Adherence to medical therapies is a growing issue, so much so that the World Health Organization defined it as “a new pharmacological problem”. The main factors affecting compliance are: frequency of administration, rapid onset of action, role of device. The most severe consequence of non-adherence is the increased risk of poor clinical outcome, associated with worsening of the quality of life and increase in health-care expenditure. It appears crucial to identify those COPD patients who are “poorly or not at all compliant with their treatment”. In order to evaluate adherence to the medical therapy, several methods were proposed, the most effective of which turned out to be self-reports, i.e. simple, brief questionnaires (e.g. Morisky test). To increase the likelihood of quickly identifying non-compliant patients, it may be useful to administer a simple questionnaire to naïve subjects (for example, in the waiting room before an examination) including six specific items allowing to identify the patient’s key characteristics. Depending on the answers, patients who do not comply with their pharmacological treatment may be classified as belonging to 6 phenotypes. For patients who are already under treatment it might be useful to administer another short questionnaire during follow up examination. Once the risk of non-compliance is identified, four possible types of measures can be taken: prescription-related, educational, behavioral and complex combined measures (combination of two or more actions). Therefore, while it is clear that adherence in COPD is a critical issue, it is also obvious that raising awareness on the disease and improving cooperation among specialists, general practitioners, health-care professionals, and patients is the starting point at which this evolution should immediately begin. Each medication is able to foster good compliance with the therapy, and consequently to maximize the efficacy, by virtue of its specific inhaler and its own active ingredient.