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Idiopathic pulmonary fibrosis (IPF) is a chronic disease with an unknown etiology that causes deterioration of the structure of the lung parenchyma, resulting in a severe and progressive decline in respiratory function and early mortality. IPF is essentially an incurable disease, with a mean overall survival of 5 years in approximately 20% of patients without treatment. The combination of a poor prognosis, uncertainty about the disease's progression, and the severity of symptoms has a significant impact on the quality of life of patients and their families. New antifibrotic drugs have been shown to slow disease progression, but their impact on health-related quality of life (HRQoL) has to be proven yet. To date, studies have shown that palliative care can improve symptom management, HRQoL, and end-of-life care (EoL) in patients with IPF, reducing critical events, hospitalization, and health costs. As a result, it is essential for proper health planning and patient management to establish palliative care early and in conjunction with other therapies, beginning with the initial diagnosis of the disease.

Chronic interstitial lung disease (ILD) is considered a heterogeneous group of pulmonary diseases usually classified on the basis of clinical, radiological and histological aspects, characterized by a variable degree of inflammation and fibrosis. The most diagnosed ILD are idiopathic pulmonary fibrosis (IPF), characterized by the presence of serious changes in the normal architecture of the pulmonary parenchyma, resulting in a progressive and often rapid respiratory functional decline. Although considered a rare disease, the IPF has seen a considerable increase in prevalence and mortality, over the last 10 years. Moreover, it has a worse outcome than most malignant tumours resulting in an average. IPF should be considered in all adult patients with unexplained chronic exertional dyspnoea, and commonly presents with cough, bibasilar inspiratory crackles and finger clubbing. The diagnosis of IPF requires exclusion of other known causes of interstitial lung disease (ILD) and the presence of a usual interstitial pneumoniae (UIP) pattern on high-resolution computed tomography (HRCT) in patients not subjected to surgical lung biopsy. The functional respiratory pattern is characterized by restrictive type of ventilatory compromise, reduction of the DLCO diffusion capacity, hypoxaemia during exercise and, then, at rest too. Despite the efforts made in scientific research and the therapeutic progress achieved, there are currently no drugs capable of achieving the remission and healing of this pathology. To date, there are only two drugs, pirfenidone and nintedanib, although not usable by all patients, able to drastically reduce symptoms and exacerbations, significantly improve the quality of life and, above all, stop the decline in respiratory function.

Recently, high-flow nasal oxygen therapy (HFNO) has been introduced and used in severe ipoxiemic patients or in weaning from invasive ventilation. HFNO is based on an heated and humidified high flow oxygen through a nasal cannula, with an open circuit, in which an inspiratory fraction of oxygen (FiO2) and a flow of gas, are set. This procedure determine a wash-out of the anatomical dead space, an effective administration of the set FiO2 and a fluidification of the secretions. Aim of the study was to monitor patient with severe COPD admitted to our pneumology unit, affected by respiratory failure with hypercapnia and to observe if there was a reduction of CO2 during hospitalization and number of hospital re-admissions, with the HFNO therapy on continuous use for twelve months. We followed 20 patients with diagnosis of severe COPD according to GOLD recommendations (12 M- 8 F, mean age 66.15 years), in triple combination therapy LABA+LAMA+ICS. All patients were suffering from hypoxemic and hypercapnic respiratory failure at the time of hospitalization and had an history of at least 1 hospitalization for exacerbation in the previous year. The results of our preliminary data were: a) an improvement in mean pCO2 values (54.75 vs 47.85) during admission, b) a reduction in the average number of re-hospitalizations after 12 months of follow-up (2.1 vs. 0.85), c) the increase of lengthened in the free time from exacerbations (5.7 months vs. 14.1 months). We can try to conclude that continuous HFNO therapy can be a valuable aid, in addition to drug therapy, in reducing the number of exacerbations as well as the need for hospitalization of COPD patients.

Introduction: Currently there is no consensus on the diagnostic criteria, treatment and follow-up in Pulmonary Sarcoidosis (SP). The purpose of the study was to identify a valid process for the diagnosis of SP. Method: Clinical,radiological and FOB data were collected retrospectively in 31 patients (18 F, mean age 57.3) with SP during five years.All patients performed spirometry with plethysmography and DLCO,SpO2, 6MWT and HR chest CT scans, evaluated by two radiologists with high experience in the diagnosis of interstitial lung disease.All patients underwent FOB with BALF (n = 31), TBB(n= 13) and TBNA(n = 4). In the ill-defined cases, the lymph node biopsy (n = 3) or pulmonary VATS (n = 2), have allowed definitive diagnosis. Results: The cytohistological diagnosis was made in 58% using FOB. In patients who did not undergo node biopsy or VATS, the diagnosis was made by association of BALF, HRCT scan and the exclusion of other diseases. The cut-off value of CD4/CD8 ratio for sarcoidosis was determined at 2.16 and shows a 92.4% sensitivity (95% CI, 61.5 to 98.6%) and specificity of 86.2% (96% CI, 72.1 to 92.5%). The severity of interstitial damage at CT scan and the GGO are correlated with the decline in DLCO and PaO2 / FiO2. Conclusion: Our data show that a) the clinical-radiological approach is crucial to address the diagnostic process,b) the use of appropriate endoscopic techniques are still essential for a diagnostic confirmation c) the multidisciplinary approach, the background and clinical experience, are the key for the identification of patients with pulmonary sarcoidosis when there is suspicion of a granulomatous disease. Meyer KC at al. Am J Respir Crit Care Med 2012;185:1004-14.

Background: IPF is a chronic,progressive and lethal interstitial lung disease with poor prognosis.Although pirfenidone is tolerable and safe while reducing lung function decline, few studies document the trend of progression in UIP patients before starting treatment with pirfenidone.Objectives:The purpose of this study is to evaluate the progression of IPF pre and during pirfenidone treatment.Methods:We recruited 20 patients with mild-moderate IPF diagnosis (during the years 2011-2016) according to guidelines. All patients were studied with pulmonary function tests including spirometry, Body Plethysmography , DLCO and 6-minute walk test during one year pretreatment with pirfenidone (T-12), 6 month post introducing treatment (T6) and 1 year post-treatment (T+12).Results: We observed an initial decline in DLCO mean[mean value 54,1% pred (DS 13,26)],FVC [meanvalue 83% pred (DS 19,7)] SpO2 [mean value 95.5% (DS 2.39)] parameters at T-12. The trend of disease progression in mild-moderate IPF remained stable at T6 [FVC mean 77,1 (DS 21,46)] and T+12 [FVC% 76,75 (DS 19,75)] post pirfenidone introduction. However, patients with worse initial data, show a greater decline in DLCO, in 6MWT distance and lowest SpO2% than patients with better initial data.In addition, five patients with four years of treatment, show FVC and DLCO stability.Conclusions: Our data show that pirfenidone determines the stability of pulmonary function parameters in mild-moderate IPF.

Background Endothelin (ET) is an endothelium-derived multifunctional peptide involved in the local regulation of the vascular tone. Study objectives To assess changes of endogenous ET production/excretion in the acute phase (36 h from the event) of pulmonary embolism (PE). Participants Ten patients with acute PE, nine patients with acute lung injury (ALI), and 12 healthy volunteers (HVs). Measurements and results ET was detected by radioimmunoassay in venous and arterial blood as well as in 24-h urine specimens. For each subject, arterial/venous immunoreactive ET (ir-ET) ratio was evaluated as an index of its pulmonary extraction/synthesis. Creatinine clearance was employed in each case to obtain a corrected renal ir-ET clearance. Renal ir-ET clearance was comparable in all three groups. Arterial/venous ir-ET ratio was comparable in PE and in ALI patients (1.31 ± 0.25 vs 1.24 ± 0.20; p=0.7), while it was significantly higher in PE patients than in HV subjects (0.85 ± 0.07; p=0.0001). Accordingly, 24-h urine ir-ET excretion was higher in PE (120.50 ± 27.36 ng/24 h) and ALI patients (135.80 ± 21.60 ng/24 h) than in HV subjects (68.33 ± 9.31 ng/24 h; p=0.0001). Conclusions Abnormalities of ET metabolism-mainly related to increased synthesis and/or defective pulmonary handling-occur in the acute phase of PE. The relevance of this finding with respect to the pathogenesis and/or management of pulmonary thromboembolism remains to be elucidated.

Merola B, Longobardi S, Sofia M, Pivonello R, Micco A, Di Rella F, Esposito V, Colao A, Lombardi G. Lung volumes and respiratory muscle strength in adult patients with childhood- or adult-onset growth hormone deficiency. Effect of 12 months' growth hormone replacement therapy. Eur J Endocrinol 1996;135:553–8. ISSN 0804–4643 We have described impairment of the respiratory function in adult patients with childhood-onset growth hormone (GH) deficiency. The aim of the present study was to evaluate lung volumes and respiratory muscle strength in patients diagnosed as GH deficient before and after 6 and 12 months of recombinant GH treatment. Ten adults diagnosed as GH deficient in childhood, ten adults diagnosed as GH deficient in adulthood and ten healthy subjects entered the study. For each subject, evaluation of respiratory function followed the same standard approach, consisting of respiratory muscle strength assessment, record of flow–volume curves, measurement of static lung volumes and lung diffusing capacity. Childhood-onset GH-deficient patients had a significant reduction of maximal inspiratory (p < 0.01) and maximal expiratory (p < 0.05) mouth pressures. Total lung capacity, vital capacity and functional residual capacity were significantly reduced compared to healthy subjects (p < 0.05). Conversely, residual volume and diffusing lung capacity did not show any significant change. No significant change of the ratio between the percentage forced expiratory volume in 1 s and the forced vital capacity was observed. The decrease of respiratory mouth pressures was not correlated to the decrease of lung volumes. Adult-onset GH-deficient patients had only a significant reduction of maximal expiratory pressure compared to healthy subjects (p < 0.05). After 6 months of treatment no significant differences in any of the evaluated parameters were found. After 12 months of treatment patients with childhood-onset GH deficiency show a significant improvement of lung volumes (p < 0.01) and maximal respiratory mouth pressures (p < 0.005), whereas adult-onset GH-deficient patients show a significant improvement of maximal expiratory pressure (p < 0.05). In conclusion, the results of this study showed that adult patients affected with childhood-onset GH deficiency suffer from an impairment of the ventilatory function due to a reduction of lung volumes and a decrease of respiratory pressures probably due to a reduction of respiratory muscle strength. This impairment was reversed after 12 months of treatment with recombinant GH. Conversely, adult-onset GH-deficient patients had only an impairment of the maximal expiratory pressure, probably due to respiratory muscle weakness re-established after 12 months of GH therapy. Gaetano Lombardi, Via G. Santacroce 40/a, 80129 Naples, Italy

Searching for IgG and IgM against the mycobacterial antigen A60 has been recognized as a potential diagnostic tool for pulmonary tuberculosis. The role of detection of anti-A60 IgA in improving diagnostic accuracy of serology is not well known. In this study we measured with ELISA serum levels of both anti-A60 IgG and IgA in 216 subjects. 88 healthy volunteers (44 PPD- and 44 PPD+), 44 patients suffering from nontuberculous lung disease and 15 subjects with healed pulmonary tuberculosis constituted the control population; 69 patients with active pulmonary tuberculosis (35 cavitary forms, 26 productive forms and 8 miliary forms) were examined. The sensitivity of IgG test was 73.9% in pulmonary tuberculosis (77.1% in cavitary forms, 65.4% in productive forms, 87.5% in miliary forms); the specificity of the test was 95.9%. For the IgA test we observed a sensitivity of 72.5% (74.3 in cavitary forms, 69.2% in productive forms, 75.0 in miliary forms) and a specificity of 93.9%. Combination of the two tests increased the sensitivity to 84.0% (+10.1% compared to IgG test, +11.5% compared to IgA test); the specificity decreased to 92.5% (-3.4% vs. IgG test; -1.4 vs. IgA test). In conclusion, the combined use of evaluation of anti-A60 IgG and IgA increases the accuracy of serological diagnosis of pulmonary tuberculosis.