Symptomatic post COVID patients have impaired alveolar capillary membrane function and high VE/VCO.

Background: Post COVID-19 syndrome is characterized by several cardiorespiratory symptoms but the origin of patients' reported symptomatology is still unclear.

Methods: Consecutive post COVID-19 patients were included. Patients underwent full clinical evaluation, symptoms dedicated questionnaires, blood tests, echocardiography, thoracic computer tomography (CT), spirometry including alveolar capillary membrane diffusion (DM) and capillary volume (Vcap) assessment by combined carbon dioxide and nitric oxide lung diffusion (DLCO/DLNO) and cardiopulmonary exercise test.

Lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is an ultra-rare, slowly progressive neoplastic cystic disease, belonging to the group of PEComas. It can occur sporadically or associated to tuberous sclerosis complex disease and affects mainly women in child-birth age. Dyspnoea is the most frequent symptom referred to the time of diagnosis, however spontaneous pneumothorax may be a typical presentation associated to extrathoracic manifestations, such as renal angiomyolipomas.

Charlson comorbidity index, neutrophil-to-lymphocyte ratio and undertreatment with renin-angiotensin-aldosterone system inhibitors predict in-hospital mortality of hospitalized COVID-19 patients during the omicron dominant period.

Purpose: To investigate the clinical predictors of in-hospital mortality in hospitalized patients with Coronavirus disease 2019 (COVID-19) infection during the Omicron period.

Methods: All consecutive hospitalized laboratory-confirmed COVID-19 patients between January and May 2022 were retrospectively analyzed. All patients underwent accurate physical, laboratory, radiographic and echocardiographic examination.

Pulmonary Langerhans Cell Histiocytosis and Lymphangioleiomyomatosis Have Circulating Cells With Loss of Heterozygosity of the TSC2 Gene.

Background: Lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH) are cystic lung diseases in which a neoplastic cell is thought to be responsible for disease pathogenesis. The neoplastic LAM cell has mutations in the TSC genes, TSC1 or TSC2, whereas the neoplastic PLCH cell may have mutations in several genes (eg, BRAF, NRAS, MAP2K1). These mutations are not specific for PLCH and have been described in multiple cancers.

Improving Survival in Lymphangioleio-myomatosis: A 16-Year Observational Study in a Large Cohort of Patients.

Background: Over the last 2 decades, great progress has been made in the understanding of the clinical aspects and pathogenesis of lymphangioleiomyomatosis (LAM), leading to publication of guidelines and approval of an effective therapy.

Objectives: Aim of our study was to describe how the management and the natural history of this rare disease have changed after the publication of the ERS and American Thoracic Society/Japanese Respiratory Society guidelines and the introduction of sirolimus.

Methods: We examined 162 LAM patients followed at our center between 2001 and 2017, reporting clinical characteristics and diagnostic approach.

Progressive Fibrosing Interstitial Lung Diseases: Prevalence and Characterization in Two Italian Referral Centers.

Background: The prevalence and natural history of progressive fibrosing interstitial lung diseases (PF-ILDs), and their response to commonly used treatments in real life are largely unknown.

Objectives: The aim of the study was to describe the prevalence, clinical characteristics, management, and outcomes of PF-ILD patients attending 2 Italian referral centers (San Gerardo Hospital, Monza, and San Giuseppe Hospital, Milan) from January 1, 2011, to July 31, 2019.

Methods: From a cohort of non-idiopathic pulmonary fibrosis fibrosing ILD patients with at least 2-year follow-up, we selected only those with progressive disease, defined as per the INBUILD trial, collecting their demographical, clinical, and functional data.

Correlation Between Doppler Echocardiography and Right Heart Catheterisation-Derived Systolic and Mean Pulmonary Artery Pressures: Determinants of Discrepancies Between the Two Methods.

Background: There is still controversy about whether transthoracic echocardiography (TTE) can provide reliable estimations of pulmonary artery pressures (PAP). The primary endpoint of this study was to evaluate the correlation between TTE and right heart catheterisation (RHC) in estimating systolic (SPAP) and mean (MPAP) pulmonary artery pressures.

Methods: Between January 2011 and December 2018, 141 consecutive patients (average age 63.

Ultra-rare cystic disease.

Diffuse cystic lung diseases include a group of heterogeneous disorders characterised by the presence of cysts within the lung parenchyma, sometimes showing a characteristic computed tomography scan pattern that allows diagnosis. The pathogenetic mechanisms underlying cyst formation in the lung are still not clear and a number of hypotheses have been postulated according to the different aetiologies: ball-valve effect, ischaemic dilatation of small airways and alveoli related to infiltration and obstruction of small vessels and capillaries that supply the terminal bronchioles and connective tissue degradation by matrix metalloproteases. A wide number of lung cyst diseases have been classified into six diagnostic groups according to the aetiology: neoplastic, congenital/genetic, lymphoproliferative, infective, associated with interstitial lung diseases, and other causes.

Older Idiopathic Pulmonary Fibrosis Male Patients Are at a Higher Risk of Nintedanib Dose Reduction.

Background: Two pharmaceutical agents have been approved for treatment of idiopathic pulmonary fibrosis (IPF): pirfenidone and nintedanib.

Objectives: We investigated the need of dose reduction in consecutive patients treated with nintedanib in relation to gender and body max index, comparing the population over and under 80 years of age.

Methods: We retrospectively reviewed the data of all consecutive IPF patients treated with nintedanib for at least 3 months.

Pulmonary hypertension and chronic lung disease: where are we headed?

Pulmonary hypertension related to chronic lung disease, mainly represented by COPD and idiopathic pulmonary fibrosis, is associated with a worse outcome when compared with patients only affected by parenchymal lung disease. At present, no therapies are available to reverse or slow down the pathological process of this condition and most of the clinical trials conducted to date have had no clinically significant impact. Nevertheless, the importance of chronic lung diseases is always more widely recognised and, along with its increasing incidence, associated pulmonary hypertension is also expected to be growing in frequency and as a health burden worldwide.

Comorbidities in idiopathic pulmonary fibrosis: an underestimated issue.

Idiopathic pulmonary fibrosis (IPF) is a progressive and fibrosing lung disease with a poor prognosis. Between 60% and 70% of IPF patients die of IPF; the remaining causes of death may be due to comorbidities occurring in this ageing population. Interest in the role played by comorbidities in IPF has increased in the past few years.

Hemodynamic Mechanisms of Exercise-Induced Pulmonary Hypertension in Patients with Lymphangioleiomyomatosis: The Role of Exercise Stress Echocardiography.

Background: The pathogenesis of pulmonary hypertension (PH) in lymphangioleiomyomatosis (LAM) has not yet been completely clarified. The aim of this study was to conduct a noninvasive evaluation of the main hemodynamic mechanisms of exercise-induced PH in patients with LAM, assessed using exercise stress echocardiography.

Methods: Fifteen patients with LAM (mean age, 47 ± 13 years; all women) without resting PH were enrolled in a prospective single-center study and compared with 15 healthy female control subjects (mean age, 45.

Physiological insights of exercise hyperventilation in arterial and chronic thromboembolic pulmonary hypertension.

Background: Pulmonary hypertension (PH) patients show, during exercise, an excessive increase in ventilation (V) compared to carbon dioxide output (VCO), determining a high V/VCO slope. There are several possible causes, including an elevated dead space ventilation (V), V/perfusion (Q) mismatch and/or an enhanced peripheral or central chemoreceptor activity. We evaluated the causes of exercise hyperventilation in PH patients.

Severe idiopathic pulmonary fibrosis: what can be done?

Idiopathic pulmonary fibrosis (IPF) remains a challenging disease to manage. Two drugs are now available that can slow disease progression in patients with mild-to-moderate IPF. This means that early diagnosis is mandatory, because there are no proven effective therapies for severe IPF.

The changing face of a rare disease: lymphangioleiomyomatosis.

Lymphangioleiomyomatosis is a rare disease characterised by cystic destruction of the lung, lymphatic abnormalities and abdominal tumours. It affects almost exclusively females and can occur sporadically or in patients with tuberous sclerosis complex. In the past decade remarkable progress has been made in understanding of the pathogenesis of this disease leading to a new therapeutic approach.

Pulmonary Langerhans cell histiocytosis: a comprehensive analysis of 40 patients and literature review.

Background: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial disease affecting primarily young adult smokers. In order to highlight the clinical features of the disease, we conducted a retrospective analysis on clinical data of PLCH patients followed at our center; moreover, we reviewed the current literature on PLCH.

Method And Results: Between January 2004 and July 2014, 40 patients with PLCH were evaluated at our Division.

Pulmonary hypertension in idiopathic pulmonary fibrosis does not influence six-minute walk distance: results from a retrospective study.

Background: The characteristics of the six-minute walk test (6MWT) in patients with idiopathic pulmonary fibrosis (IPF) and pulmonary hypertension (PH) have not yet been described; nevertheless, this test has already been used as a "surrogate end point" in some clinical trials.

Objective: Goal of this retrospective study was to assess whether the presence of PH in patients with IPF might influence 6MWT performances.

Methods: We retrospectively reviewed the data of patients with IPF who were referred to our hospital.