Search

Background. Some studies with inconclusive results have reported a link between sarcoidosis and an increased risk of pulmonary embolism (PE). This study aimed at assessing a possible correlation between potential risk factors and PE in sarcoidosis patients. Methods. A total of 256 sarcoidosis patients (84 males and 172 females, mean age at diagnosis 49 ± 13) were enrolled after giving written informed consent. Clinical evaluations, laboratory and radiology tests were performed to evaluate the presence of pulmonary embolism. Results. Fifteen sarcoidosis patients with PE (4 males and 11 females, mean age at diagnosis 50 ± 11), diagnosed by lung scintigraphy and 241 sarcoidosis patients without PE (80 males and 161 females, mean age at diagnosis 47 ± 13), were observed. There was a statistically significant increase of the presence of antiphospholipid antibodies in the sarcoidosis group with pulmonary embolism. There was no statistically significant difference between the two groups as to smoking habit, obesity or hereditary thrombophilia frequency (p &gt, 0.05, respectively). Conclusions. This study demonstrates a significant correlation between the presence of antiphospholipid antibody positivity and the pulmonary embolism events in our sarcoidosis patients. Furthermore, we propose screening for these antibodies and monitoring, aimed at timely treatment.

The latest guidelines for the hospital care of patients affected by coronavirus disease 2019 (COVID-19)-related acute respiratory failure have moved towards the widely accepted use of noninvasive respiratory support (NIRS) as opposed to early intubation at the pandemic onset. The establishment of severe COVID-19 pneumonia goes through different pathophysiological phases that partially resemble typical acute respiratory distress syndrome (ARDS) and have been categorized into different clinical–radiological phenotypes. These can variably benefit on the application of external positive end-expiratory pressure (PEEP) during noninvasive mechanical ventilation, mainly due to variable levels of lung recruitment ability and lung compliance during different phases of the disease. A growing body of evidence suggests that intense respiratory effort producing excessive negative pleural pressure swings (Ppl) plays a critical role in the onset and progression of lung and diaphragm damage in patients treated with noninvasive respiratory support. Routine respiratory monitoring is mandatory to avoid the nasty continuation of NIRS in patients who are at higher risk for respiratory deterioration and could benefit from early initiation of invasive mechanical ventilation instead. Here we propose different monitoring methods both in the clinical and experimental settings adapted for this purpose, although further research is required to allow their extensive application in clinical practice. We reviewed the needs and available tools for clinical–physiological monitoring that aims at optimizing the ventilatory management of patients affected by acute respiratory distress syndrome due to severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection.

Immune reconstitution inflammatory syndrome (IRIS) is a violent inflammatory response in a patient previously immunosuppressed that discontinued this status and develops a paradoxical clinical deterioration, not explainable with drug-toxicity or opportunistic infection. We describe the case of a 59-year-old non-smoking man, treated for arthropathic psoriasis with adalimumab for 16 months who developed severe IRIS and pulmonary tuberculosis. The reintroduction of the TNF-blocker agent was effective.

OBJECTIVE: Diaphragm dysfunction is a complication of cardiac surgery with partial or absent spontaneous recovery in most cases. Surgical diaphragm plication represents the only option when symptoms persist. Because training improves functional nerve recovery after a nerve lesion, we hypothesized that early diaphragm muscle training may be beneficial. METHODS: A prospective, randomized at 2:1 ratio, controlled trial of diaphragm training using an adjustable pressure device (Threshold; Philips Respironics Inc, Murrysville, Pa) versus no training (sham device) was performed in patients with diaphragm paralysis after major cardiac surgery. This 1-year study recruited consecutive adult patients with sniff fluoroscopy-defined diaphragm paralysis after coronary bypass, valve replacement, or both. The outcome measures were diaphragm function recovery assessed by sniff fluoroscopy, maximum inspiratory and expiratory pressures, and lung function tests. RESULTS: A total of 69 patients were randomized. At 12 months, 52 patients completed the study assessments, 36 in the treatment group and 16 in the control group. Inspiratory muscle training produced a significant improvement of diaphragm mobility after 12 months (P < .001). Most patients in the training group (77.78%) experienced a partial improvement (41.67%) or achieved a complete improvement (36.11%) versus no improvement (87.5%) or partial recovery (12.5%) among controls. CONCLUSIONS: Inspiratory muscle training may improve inspiratory muscle strength and increases paralyzed diaphragm mobility.

Training has many beneficial effects, however few studies report its effects on the lungs. The aim of this study was to assess the effects of acute exercise and exercise training on inflammatory responses and remodeling in central and peripheral airways. Sixteen Sprague-Dawley rats trained for 10 weeks, while 14 rats served as controls. Before sacrifice, 8 trained (TRAC) and 8 untrained control (CONAC) rats underwent a single acute exercise bout, while 8 trained (TR) and 6 untrained control (CON) rats were sacrificed without acute exercise. The central and peripheral airways were morphologically examined for inflammatory cells and immunostained for decorin, collagen I, α-smooth muscle actin. No significant differences were found for morphometric analysis in central and peripheral airways, however CONAC showed a significant increase in polymorphonuclear cells in the central airways compared to CON. In contrast, TRAC did not show an inflammatory response different from TR. A similar trend was present in peripheral airways. Training did not induce differences in airways inflammation and remodeling as compared to CON. However, training seemed to limit the inflammatory response induced by acute exercise in the central airways.

Proteoglycans (PG) are altered in the asthmatic airway wall. Because PGs are known to affect cell proliferation and apoptosis, we hypothesized that alterations in PG might influence the airway smooth muscle (ASM) hyperplasia observed in the asthmatic airway. Human ASM cells were seeded on plastic or plates coated with decorin (Dcn), biglycan (Bgn), or collagen type I (Col I) (1, 3, and 10 μg/ml). Cells were stimulated with platelet-derived growth factor (PDGF), and cell number was assessed at 0, 48, and 96 h. Cell proliferation was measured by bromodeoxyuridine (BrdU) incorporation and apoptosis by annexin V and propidium iodide staining at 48 h post-PDGF stimulation. A significant decrease in cell number was observed with cells seeded on Dcn (10 μg/ml) at 0, 48, and 96 h ( P < 0.01). Dcn induced both decreases in BrdU incorporation and increases in annexin V staining ( P < 0.05). Bgn decreased cell number at time 0 only ( P < 0.05) and affected neither proliferation nor apoptosis. Col I (10 μg/ml) caused a significant increase in cell number at 48 and 96 h ( P < 0.01). Adding exogenous Dcn (1–30 μg/ml) to the medium had no effect on cell number. Exposing Dcn-coated matrices to chondroitinase ABC, an enzyme that degrades glycosaminoglycan side chains, reversed the Dcn-induced decrease in cell number. These studies demonstrate that different PGs have variable effects on ASM cell proliferation and apoptosis. Recently described decreases in Dcn in the asthmatic airway wall could potentially permit more exuberant ASM growth.

Proteoglycans (PG) have important effects on the mechanical properties of tissues and the phenotype of various structural cells. Little is known about changes in PG deposition in the airways in animal models of asthma. We studied changes in PG in the airway wall of Brown Norway rats sensitized to ovalbumin (OA) and exposed to repeated OA challenge. Control (Sal) animals were sensitized and challenged with saline. After the 3rd challenge, animals were killed and lungs fixed in formalin. Tissue sections were incubated with antibodies to the small, leucine-rich PG, decorin, and biglycan and collagen type I. Airways were classified according to basement membrane perimeter length (≤0.99, 1–2.99, and ≥3 mm). Decorin, biglycan, and collagen type I were increased in the airways of OA vs. Sal rats. Remodeling was most prominent in central airways. The distribution of PG differed with respect to the subepithelial vs. airway smooth muscle (ASM) vs. adventitial layer. Whereas biglycan was readily detected within the ASM, decorin and collagen were detected outside the ASM and especially in the adventitial layer. Differences in the distribution of these molecules within the layers of the airway wall may reflect their specific functional roles.

Inflammation represents an important hallmark of several diseases. In COPD the inflammatory lung response may be chronic and dysregulated with accumulation of inflammatory cells that causes lung parenchyma damage and airway remodeling. The inflammatory cascade involves different cells, mediators, proteins, oxygen species that leads the local inflammation to become systemic and promotes comorbidities and complications. Different stimuli may worsen the COPD pathology, including the well known COPD exacerbation, a peak of local and systemic inflammation. Also hypoxia may stimulate inflammatory genes worsening local and systemic level of inflammation. Understanding the inflammation can allow us to differentiate COPD phenotype and lead as to personalize the therapy. Several have the specific target of inflammation, particularly PDE4 inhibitors may have a role in certain COPD phenotype.

In spite of evidence-based consensus recommendations on exercise training for patients with pulmonary arterial hypertension (PAH) its use outside specialized rehabilitation Centers has been accounted a handful of reports. Dyspnoea and fatigue are common symptoms reducing the exercise tolerance of subjects with PAH even when specific drug are correctly administered. Moreover, the mechanisms of exercise limitation in subjects with PAH are known and thandful of reports. There is evidence of impaired signaling between the periphery and central hemodynamic performance. Exercise training has recently been proven to be effective and safe to enhance physical performance in patients with PAH already treated with specific drug therapy. The rationale of training in PAH has been specifically proven also in experimental animal models of PAH. Moreover, repeated positive results in randomized controlled trials and observational studies in humans gave convincing evidence on the beneficial effects of exercise training as a routine treatment for patients with PAH. To date only 3 small randomized controlled trials and several non-randomized experiences were published in the last decade. Nevertheless, there is a full concordance among all studies, unrespective of the design, training protocol and any possible bias. Exercise training showed to improve physical performance and daily activity, reducing dyspnoea and fatigue, and enhancing the quality of life and muscle strength. So, the final message from the available literature reports is a strong recommendation to adopt exercise training and cardiopulmonary rehabilitation programs for any patient suffering from exertional dyspnoea and fatigue. Many questions remain unanswered regarding the optimization of the exercise training and rehabilitation programs. Ongoing rehabilitative trials in patients with PAH will help answer some of the remaining questions.

Acute respiratory failure (ARF) is a common cause of morbidity and mortality in most advanced neuromuscular disorders (NMD). Deterioration of lung function during NMD may have different severity and time course. During slowly progressive neuromuscular disease an acute event can precipitate the respiratory failure, while rapidly progressive NMD may present as ARF at the onset of the disease. During ARF a patient with NMD may be affected by inspiratory muscles weakness responsible for decreased ventilation, expiratory muscles weakness responsible for altered coughing effectiveness, and bulbar involvement responsible for altered swallowing and airway protection. Neuromuscular disorders may also affect central control of breathing, sleep disorder, bones and joints deformities with consequent ventilatory dysfunction. Usually ARF in NMD is prompted by precipitating factors such as upper airways infections, pneumonia, atelectasis etc. It is important to distinguish respiratory failure due to an acute event or to a rapidly progression of underlying conditions to properly allocate the patient in the more appropriate setting of care. Generally, excessive invasiveness of care must be avoided, and a pro-active early rehabilitation program may help early recovery and minimize complications. Mechanical non invasive positive pressure ventilation(NIPPV) combined with mechanically assisted coughing (MAC) has been established as the standard treatment of severe ARF in NMD. Superiority of NIPPV vs invasive mechanical ventilation (MV) was showed in term of lower mortality, treatment failure, duration of ICU in several studies. Home mechanical noninvasive ventilation and MAC may result dramatic reduction in the need for hospitalization and a prolongation of life expectancy of these patients. To avoid the progression of respiratory failure an early identification of respiratory deterioration is needed, as such as prevention of acute episodes. So, especially in the progressive diseases, is very important to monitor respiratory status with periodic lung function tests. Any respiratory tract infection in neuromuscular disease could trigger acute respiratory failure so it is mandatory take any aggressive measures to prevent and treat this complication. Some of principal preventive measures include vaccination against Pneumococcus Pn. and influenza virus, early antibiotic treatment when bacterial infection is suspected, chest physiotherapy for removal airway secretions or mechanical assisted maneuvers to increase cough efficiency. In conclusion, a proactive, preventive approach is the key for optimal management acute respiratory problems in neuromuscular disorders.

a b s t r a c t To assess the occurrence of tidal expiratory flow limitation (EFL) and/or dynamic pulmonary hyperinfla- tion (DH) in chronic heart failure (CHF) during exercise 15 patients with stable systolic CHF, aged 69 ± 6 yr, underwent pulmonary function testing and incremental cardio-pulmonary exercise testing. They subse- quently performed constant load exercise testing at 30, 60 and 90% of respective maximum workload. At each step the presence of EFL, by negative expiratory pressure technique, and changes in inspiratory capacity (IC) were assessed. Ejection fraction amounted to 36 ± 6% and ˙ VO2 , peak (77 ± 19% pred.) was reduced. EFL was absent at any step during constant load exercise. In 6 patients IC decreased more than 10% pred. at highest step. Only in these patients TLC, FRC, RV FEF25-75% and DLCO were decreased at rest. ˙ VO2 , peak correlated with DLCO, TLC and IC at rest and with IC (r 2 = 0.59; p < 0.001) and decrease in IC (r 2 = 0.44; p < 0.001) at 90% of maximum workload. During exercise CHF patients do not exhibit EFL, but some of them develop DH that is associated with lower ˙ VO2 , peak.