Invasive Aspergillus tracheobronchitis in a patient with hairy cell leukemia and previous Plasmodium falciparum infection.

Invasive Aspergillus tracheobronchitis is a relatively rare form of invasive pulmonary aspergillosis characterized by invasion of the tracheobronchial tree by Aspergillus spp. Invasive pulmonary aspergillosis is predominantly detected in severely immunocompromised patients. Notably however, pulmonary and tracheobronchial cases of invasive aspergillosis have also been reported, particularly in the context of severe malaria caused by Plasmodium falciparum. Herein, we present a case of invasive Aspergillus tracheobronchitis in a patient with hairy cell leukemia and previous Plasmodium falciparum infection.

A Fatal Case of Presumptive Diagnosis of Leptospirosis Involving the Central Nervous System.

Leptospirosis is a reemerging zooanthroponosis with a worldwide distribution, though it has a higher incidence in areas with tropical climate. A characteristic finding of the disease is its wide spectrum of symptoms and organ involvement, as it can appear either with very mild flu-like manifestations or with multiorgan failure, affecting the central nervous system (CNS) with a concomitant hepatorenal dysfunction (Weil's syndrome) and significant high mortality rate. We report herein a fatal case of a 25 years old female, previously healthy, with impaired neurological status. She had high fever and severe multiorgan failure. The clinical data and the epidemiological factors were not conclusive for the diagnosis, and the first serology test from the cerebrospinal fluid (CSF) and sera samples were negative. When the repetition of the blood test showed elevated IgM antibodies, Leptospirosis was the presumptive diagnosis. Although CNS involvement is rare, the diagnosis should be considered when there is an elevated risk of exposure. The diagnostic protocol should encompass direct evidence of the bacterium and indirect measurement of antibodies. Timely detection and management are imperative to forestall complications and fatality associated with the disease.

Overnight desaturation in interstitial lung diseases: links to pulmonary vasculopathy and mortality.

Background: Overnight desaturation predicts poor prognosis across interstitial lung diseases (ILDs). The aim of the present study was to investigate whether nocturnal desaturation is associated with pulmonary vasculopathy and mortality. Methods: A retrospective single centre study of 397 new ILD patients was carried out including patients with idiopathic pulmonary fibrosis (IPF) (n=107) and patients with non-IPF fibrotic ILD (n=290). This is the largest study to date of the effect of significant nocturnal desaturation (SND) (≥10% of total sleep time with oxygen saturation ≤90% measured by pulse oximetry). Results: The prevalence of SND was 28/107 (26.2%) in IPF and 80/290 (27.6%) in non-IPF ILD. The prevalence of SND was higher in non-IPF ILDs than in IPF (p=0.025) in multivariate analysis. SND was associated with noninvasive markers of pulmonary hypertension (PH): tricuspid regurgitation velocity (TRV) (p<0.0001), brain natriuretic peptide (p<0.007), carbon monoxide transfer coefficient (p<0.0001), A-a gradient (p<0.0001), desaturation >4% in 6-min walking test (p<0.03) and pulmonary artery diameter (p<0.005). SND was independently associated with high echocardiographic PH probability in the entire cohort (OR 2.865, 95% CI 1.486-5.522, p<0.002) and in non-IPF fibrotic ILD (OR 3.492, 95% CI 1.597-7.636, p<0.002) in multivariate analysis. In multivariate analysis, SND was associated with mortality in the entire cohort (OR 1.734, 95% CI 1.202-2.499, p=0.003) and in IPF (OR 1.908, 95% CI 1.120-3.251, p=0.017) and non-IPF fibrotic ILD (OR 1.663, 95% CI 1.000-2.819, p=0.041). Separate models with exclusion of each one of the diagnostic subgroups showed that no subgroup was responsible for this finding in non-IPF ILDs. SND was a stronger marker of 5-year mortality than markers of PH. Conclusion: SND was associated with high echocardiographic probability and mortality and was a stronger predictor of mortality in IPF and non-IPF ILDs grouped together to power the study.

Expression of SDF-1/CXCR4 axis in bone marrow mesenchymal stem cells derived from rheumatoid arthritis-usual interstitial pneumonia.

OBJECTIVES: To explore the SDF-1/ CXCR4 axis as driving mechanism of bone marrow mesenchymal stem-cells to the injured lung in patients with rheumatoid arthritis associated usual interstitial pneumonia (RA-UIP). METHODS: We evaluated the m-RNA expression of SDF-1 and CXCR4 with real-time PCR in bone marrow mesenchymal stem cells of 7 RA-UIP and 10 RA patients without lung involvement. RESULTS: The axis was not expressed in RA whereas both SDF-1 and CXCR4 were expressed in RA-UIP [1.93 (1.32, 2.00) and 0.008 (0, 0.01)] respectively. CONCLUSIONS: The development of pulmonary fibrosis in RA may be considered as the key event for the migration of stem cells to the injured lung through the SDF-1/CXCR4 axis.

Disseminated Bacillus Calmette-Guérin (BCG) infection presenting as severe respiratory failure and septic shock.

Introduction: Intravesical Bacillus Calmette-Guérin (BCG) instillation is the most effective adjuvant therapy for superficial urinary bladder carcinoma, prolonging disease-free survival. Although it is usually well tolerated, moderate to severe local or systemic infectious complications, including sepsis involving multiple organs, may occur. Case report: We report the unusual case of a man in his mid '70s who presented with septic shock and severe acute respiratory failure requiring intubation. Lack of response to antibiotics, history of intravesical BCG instillation and consistent imaging findings led to further investigations, with bronchoalveolar lavage (BAL) fluid polymerase chain reaction (PCR) results indicating pneumonitis due to Mycobacterium bovis dissemination. Prompt anti-tuberculosis treatment combined with corticosteroids resulted in significant clinical and radiological improvement, supporting the diagnosis of disseminated BCG infection. Conclusions: Due to its non-specific clinical presentation and the relatively low diagnostic yield of conventional microbiological tests, a high index of suspicion is required for prompt diagnosis and treatment of systemic BCG infection. PCR-based assays for mycobacterial DNA identification may represent a valuable tool facilitating timely diagnosis of this uncommon, yet potentially life-threatening infection.

Lung and diaphragm protective ventilation: a synthesis of recent data.

INTRODUCTION: : To adhere to the Hippocratic Oath, to 'first, do no harm', we need to make every effort to minimize the adverse effects of mechanical ventilation. Our understanding of the mechanisms of ventilator-induced lung injury (VILI) and ventilator-induced diaphragm dysfunction (VIDD) has increased in recent years. Research focuses now on methods to monitor lung stress and inhomogeneity and targets we should aim for when setting the ventilator. In parallel, efforts to promote early assisted ventilation to prevent VIDD have revealed new challenges, such as titrating inspiratory effort and synchronizing the mechanical with the patients' spontaneous breaths, while at the same time adhering to lung-protective targets. AREAS COVERED: This is a narrative review of the key mechanisms contributing to VILI and VIDD and the methods currently available to evaluate and mitigate the risk of lung and diaphragm injury. EXPERT OPINION: Implementing lung and diaphragm protective ventilation requires individualizing the ventilator settings, and this can only be accomplished by exploiting in everyday clinical practice the tools available to monitor lung stress and inhomogeneity, inspiratory effort, and patient-ventilator interaction.

Management of Inadvertent Arterial Catheterization during Central Venous Catheter Placement: A Case Series.

Percutaneous central venous catheterization, although a widely used technique in ICU patients worldwide, is not devoid of complications even under real-time ultrasound guidance. Arterial puncture is a well-recognized complication, while unintended subclavian or carotid artery cannulations during attempted central venous catheterization are infrequent, but documented complications with potentially deleterious consequences. Recently, endovascular balloon tamponade has emerged as the preferred initial approach to repair inadvertent arterial cannulations. Herein, we present a case series of inadvertent arterial catheterization during an attempted ultrasound-guided access of the right internal jugular and the left subclavian vein that were successfully managed with endovascular balloon tamponade.

Ventilatory Ratio Threshold for Unassisted Breathing: A Retrospective Exploratory Analysis.

BACKGROUND: The ventilatory ratio (VR) is a simple index of ventilatory efficiency and dead space. Because increased dead space and high ventilatory demands impose a limitation to unassisted ventilation, and may predispose patients to injurious strong efforts during assisted ventilation, evaluation of the VR could provide helpful information during weaning. We hypothesize that there is a threshold of VR associated with tolerance of unassisted breathing. METHODS: In a retrospective analysis, we included subjects ventilated in a control mode for at least 24 h, who were successfully liberated from mechanical ventilation, without use of noninvasive ventilation, and discharged alive from the ICU. We focused on the successful weaning attempts (the last, if more than one was performed) and evaluated the VR at the beginning and at the end of the assisted ventilation period. RESULTS: We examined 2,000 medical records and included in our analysis 572 subjects (age: 68 y, R5-95 = 25-85, 68% male) with main admission diagnosis of respiratory failure (23%), sepsis (11%), brain injury (34%), and postoperative (14%). The VR at the beginning and the end of the assisted ventilation period was 1.5 (R5-95 = 1-2.1) and 1.4 (R5-95 = 1-2), respectively. The median duration of assisted ventilation in subjects with a VR ≥ 2 at the beginning of the assisted ventilation period was 3 d (R5-95 = 0-14 d), significantly longer than in those with a VR < 2, 0.5 d (R5-95 = 0-8 d, P < .001). CONCLUSIONS: Successful liberation from assisted ventilation was associated with a VR < 2. A VR > 2 was associated with longer duration of weaning. The VR could be used as an additional tool to facilitate the decision-making process during weaning.

Role of VEGF-stromal cell-derived factor-1alpha/CXCL12 axis in pleural effusion of lung cancer.

CONTEXT AND OBJECTIVE: It has been suggested that stromal cell-derived factor-1alpha ((SDF-1alpha) or CXCL12, both transcripts, TR1 and TR2) and its cognate receptor CXCR4 may regulate cancer metastasis. We have investigated the role of vascular endothelial growth factor (VEGF), angiopoietins (Ang-1 and Ang-2) and the biological axis of CXCL12-CXCR4, in patients with malignant pleural effusions (PEs). MATERIAL AND METHODS: Twenty five patients, seven with transudative PEs due to heart failure and 18 with exudative malignant PEs (7 with small cell lung cancer (SCLC) and 11 with nonsmall cell lung cancer (NSCLC)) were included in the study. Expression analysis of the mediators was performed in pleural fluid pellet using real-time reverse transcription-PCR. Protein expression has been evaluated by western blot analysis. RESULTS: SDF-TR1 (P = 0.02) but not SDF-TR2 (P = 0.23) or CXCR4 levels (P = 0.23) were higher in malignant PEs than in transudates. SDF-TR1 (P = 0.04) and SDF- TR2 levels (P = 0.04) but not CXCR4 levels (P = 0.123) were higher in SCLC PEs than in heart failure PEs. SDF-TR1 (P = 0.03) but not SDF-TR2 levels (P = 0.6) and CXCR4 levels (P = 0.4) were higher in NSCLC PEs than in transudates. Ang-1 has not been expressed in PEs, whereas no significant difference has been detected in VEGF and Ang-2 expression between malignant PEs and transudates. However, protein expression showed increased VEGF and SDF expression in malignant PEs. CONCLUSIONS: These results suggest that elevated SDF-1alpha/CXCL12 levels would be suggestive of a link to metastasis and may participate in pleural trafficking in lung cancer.

Upregulation of stromal cell derived factor-1alpha in collagen vascular diseases-associated interstitial pneumonias (CVDs-IPs).

OBJECTIVE: We speculated that distinct angiogenic profiles are involved in idiopathic interstitial pneumonias (IIPs) in comparison with interstitial pneumonias associated with collagen vascular disease (CVD-IPs). This hypothesis was investigated by measuring the expression of a cardinal biologic axis, the vascular endothelial growth factor (VEGF)-stromal derived growth factor [SDF-1alpha, transcripts 1 and 2 (TR1 and TR2)] and receptor, CXCR4 and the angiogenetic receptors CXCR2 and CXCR3 in bronchoalveolar lavage fluid (BALF) in both conditions. METHODS: We studied prospectively 25 patients with fibrotic IIPs (f-IIPs) [20 with idiopathic pulmonary fibrosis (IPF) and 5 with idiopathic non-specific interstitial pneumonia (NSIP)] and 16 patients with CVD-IPs. mRNA expression was measured by Real-Time RT-PCR and protein was evaluated by Western Blotting. RESULTS: A significantly greater value has been detected in SDF-1alpha-TR1 mRNA expression levels of CVD-IPs (p=0.05) in comparison with IPF group. A similar trend has been also detected in protein expression in favor of CVD-IP group. In addition, VEGF mRNA levels have been found significantly increased in CVD-IPs in comparison with the NSIP group (p=0.05). No significant difference has been found in SDF-1alpha-TR2-CXCR4 mRNA and CXCR2-CXCR3 between the two groups. CONCLUSION: These results showed increased expression of SDF-1alpha in CVD-IPs, suggesting different angiogenic procedures. Further studies are needed in order to better explore the angiogenetic pathway in these disorders.

Hemophagocytic lymphohistiocytosis syndrome associated with Epstein-Barr infection in an immunocompetent patient. A case study.

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening rare disease resulting from the uncontrolled activation of the immune system, leading to unrestrained cytokine release and macrophage activation. It can be either hereditary or acquired due to infections, hematological disease or malignancy. CASE REPORT: We present the case of a 19-year old woman that presented with high fever and acute cholestatic hepatitis. She was initially admitted to the Gastroenterology department and the following days she developed respiratory distress and multiorgan insufficiency that necessitated intubation and support in the Intensive Care Unit. Fever, splenomegaly, hypertriglyceridemia, increased ferritin levels and hemophagocytosis in the bone marrow were found, thus, fulfilling the criteria of hemophagocytic lymphohistiocytosis. Laboratory examination was notable for positive serology (IgM and IgG) and PCR for EBV in the serum. An extensive workup including virology and immunologic workup, blood cultures, a CT of the thorax and the abdomen and a bone marrow biopsy did not reveal any cause of secondary HLH other than the EBV infection. The patient was treated with high dose corticosteroids and intravenous immunoglobulins with slow resolution of her symptoms. CONCLUSIONS: In patients with EBV infection who exhibit persistent high fever and unresponsiveness to antibiotics, the possibility of HLH should be considered. Early diagnosis and rapid initiation of appropriate treatment may avert an unfavorable outcome.

Validation of a Proposed Algorithm for Assistance Titration During Proportional Assist Ventilation With Load-Adjustable Gain Factors.

BACKGROUND: The present study aimed to validate a recently proposed algorithm for assistance titration during proportional assist ventilation with load-adjustable gain factors, based on a noninvasive estimation of maximum inspiratory pressure (peak Pmus) and inspiratory effort (pressure-time product [PTP] peak Pmus). METHODS: Retrospective analysis of the recordings obtained from 26 subjects ventilated on proportional assist ventilation with load-adjustable gain factors under different conditions, each considered as an experimental case. The estimated inspiratory output (peak Pmus) and effort (PTP-peak Pmus) were compared with the actual-determined by the measurement of transdiaphragmatic pressure- and the derived PTP. Validation of the algorithm was performed by assessing the accuracy of peak Pmus in predicting the actual inspiratory muscle effort and indicating the appropriate level of assist. RESULTS: In the 63 experimental cases analyzed, a limited agreement was observed between the estimated and the actual inspiratory muscle pressure (-11 to 10 cm H2O) and effort (-82 to 125 cm H2O × s/min). The sensitivity and specificity of peak Pmus to predict the range of the actual inspiratory effort was 81.2% and 58.1%, respectively. In 49% of experimental cases, the level of assist indicated by the algorithm differed from that indicated by the transdiaphragmatic pressure and PTP. CONCLUSIONS: The proposed algorithm had limited accuracy in estimating inspiratory muscle effort and with indicating the appropriate level of assist.

Different activity of the biological axis VEGF-Flt-1 (fms-like tyrosine kinase 1) and CXC chemokines between pulmonary sarcoidosis and idiopathic pulmonary fibrosis: a bronchoalveolar lavage study.

BACKGROUND: We have previously shown a different local and systemic angiogenic profile of CXC chemokines in Idiopathic Pulmonary Fibrosis (IPF) patients compared to sarcoidosis. In particular, sarcoidosis showed an angiostatic microenvironment, as compared with the angiogenic cytokine milieu seen in IPF. Purpose of the Study. Our aim was to further investigate the aforementioned finding by measuring the expression of different chemokines in granulomatous and fibrotic diseases. We estimated the levels of vascular endothelial growth factor (VEGF) and its high-affinity receptor, Flt-1 (fms-like tyrosine kinase 1), in bronchoalveolar lavage fluid (BALF) of patients with IPF and pulmonary sarcoidosis. We have also investigated the mRNA expression of angiogenetic chemokines' receptors such as CXCR2 and CXCR3 and the biological axis of stromal derived factor-1 alpha (SDF-1 alpha or CXCL12 alpha/CXCL12 beta) and receptor, CXCR4. METHODS: We studied prospectively three groups of patients: (i) one group of 18 patients with IPF, (ii) one group of 16 patients with sarcoidosis, and (iii) 10 normal subjects. RESULTS: A statistically significant increase has been detected in VEGF mRNA expression in IPF in comparison with pulmonary sarcoidosis (P = .03). In addition, a significant increase has been measured in CXCL12 alpha in sarcoidosis in comparison to IPF (P = .02). Moreover, a statistically significant decrease has been found in Flt-1 protein levels in pulmonary sarcoidosis in comparison with IPF (P = .03). A significant increase in VEGF (P = .03) and CXCR4 (P = .03) mRNA levels has been also detected in sarcoidosis' patients when compared with healthy controls. CONCLUSIONS: Our data suggest that increased expression of Flt-1 and downregulation of CXCL12 alpha in IPF may further support the hypothesis of a different angiogenetic profile between fibrotic and granulomatous diseases. However, further studies are needed in order to better investigate these enigmatic diseases.

Driving pressure during proportional assist ventilation: an observational study.

BACKGROUND: During passive mechanical ventilation, the driving pressure of the respiratory system is an important mediator of ventilator-induced lung injury. Monitoring of driving pressure during assisted ventilation, similar to controlled ventilation, could be a tool to identify patients at risk of ventilator-induced lung injury. The aim of this study was to describe driving pressure over time and to identify whether and when high driving pressure occurs in critically ill patients during assisted ventilation. METHODS: Sixty-two patients fulfilling criteria for assisted ventilation were prospectively studied. Patients were included when the treating physician selected proportional assist ventilation (PAV+), a mode that estimates respiratory system compliance. In these patients, continuous recordings of all ventilator parameters were obtained for up to 72 h. Driving pressure was calculated as tidal volume-to-respiratory system compliance ratio. The distribution of driving pressure and tidal volume values over time was examined, and periods of sustained high driving pressure (≥ 15 cmH2O) and of stable compliance were identified and analyzed. RESULTS: The analysis included 3200 h of ventilation, consisting of 8.8 million samples. For most (95%) of the time, driving pressure was < 15 cmH2O and tidal volume < 11 mL/kg (of ideal body weight). In most patients, high driving pressure was observed for short periods of time (median 2.5 min). Prolonged periods of high driving pressure were observed in five patients (8%). During the 661 periods of stable compliance, high driving pressure combined with a tidal volume ≥ 8 mL/kg was observed only in 11 cases (1.6%) pertaining to four patients. High driving pressure occurred almost exclusively when respiratory system compliance was low, and compliance above 30 mL/cmH2O excluded the presence of high driving pressure with 90% sensitivity and specificity. CONCLUSIONS: In critically ill patients fulfilling criteria for assisted ventilation, and ventilated in PAV+ mode, sustained high driving pressure occurred in a small, yet not negligible number of patients. The presence of sustained high driving pressure was not associated with high tidal volume, but occurred almost exclusively when compliance was below 30 mL/cmH2O.

Gastrointestinal dysmotility in critically ill patients.

Gastrointestinal (GI) motility disorders are commonly present in critical illness. Up to 60% of critically ill patients have been reported to experience GI dysmotility of some form necessitating therapeutic intervention. It has been attributed to various factors, related to both the underlying disease and the therapeutic interventions undertaken. The assessment of motility disturbances can be challenging in critically ill patients, as the available tests used to detect abnormal motility have major limitations in the setting of an Intensive Care Unit. Critically ill patients with GI dysmotility require a multifaceted treatment approach that addresses multiple causes and utilizes multiple pharmacological pathways. In this review, we discuss the pathophysiology, assessment and management of GI dysmotility in critically ill patients.

Clinical applications of mesenchymal stem cells in chronic lung diseases.

Mesenchymal stem (stromal) cells (MSCs) are multipotent stromal cells that have the ability to modulate immune response to tissue injury and promote repair in vivo. The therapeutic potential of ex vivo expanded MSCs are currently under investigation for a variety of chronic and acute lung diseases. This review summarizes the encouraging results regarding the safety of MSCs administration from recent and current clinical trials for idiopathic pulmonary fibrosis, acute respiratory distress syndrome, and chronic obstructive pulmonary disease. It also reviews the early preliminary data extracted by the same trials regarding the efficacy of MSCs in the aforementioned lung diseases.

Paroxysmal cough and left sacroiliac joint pain in a 50-year-old Caucasian man.

Can you diagnose this patient with pulmonary symptoms, thoracic and laboratory test abnormalities and sacroiliac joint pain? http://ow.ly/LPyy30kaViz.

Granule cytotoxic activity and oxidative DNA damage in smoking and nonsmoking patients with asthma.

BACKGROUND: Lung cytotoxic mechanisms trigger the release of perforin and granzymes, causing oxidative DNA damage that ultimately leads to apoptosis. These effects, although demonstrated in COPD, have not been investigated in patients with asthma and in particular in patients with asthma who smoke. Our aim was to measure perforin, granzyme A, granzyme B, and 8-OHdG expression in sputum from smoking and nonsmoking patients with asthma, compared with smoking and nonsmoking control subjects. METHODS: Perforin, granzyme A, granzyme B, and 8-OHdG expression levels were detected by enzyme-linked immunosorbent assays in induced sputum specimens. RESULTS: Perforin expression was increased in 40% of smokers and 45% of smoking patients with asthma and in only 7% of nonsmoking patients with asthma (P = .004), compared with control subjects' values. In contrast, granzymes A and B levels were increased in > 40% of patients in all three groups vs control subjects. Finally, 8-OHdG levels were elevated in 35% of smoking patients with asthma, in 20% of smokers, and in only 10% of nonsmoking patients with asthma. Statistical analysis revealed a positive correlation between granzyme A (P < .001) and granzyme B (P = .006) expression levels and the number of pack-years in smoking patients with asthma. CONCLUSIONS: Asthma cytotoxic immune response is mainly represented by granzymes A and B, whereas in smoking patients with asthma perforin and 8-OHdG are additionally involved, resembling the immune response in COPD.

Sarcoidosis in a 65-year-old woman presenting with a lung mass and pericardial effusion: a case report.

INTRODUCTION: Sarcoidosis is a multi-systemic disorder of unknown origin and most commonly affects the lungs. Diagnosis relies on the presence of non-caseating granulomas on histologic specimens. In high-resolution computed tomography, the most characteristic findings are peribronchovascular thickening, perilymphatic nodular distribution, and bilateral hilar adenopathy. Confluent nodular opacities or large masses are rare manifestations of the disease. It is well recognized that sarcoidosis can mimic infectious, malignant, and granulomatous conditions. Here, we report a case with a high initial index of suspicion for lung malignancy in terms of clinical, lung imaging, and endoscopic findings. CASE PRESENTATION: A 65-year-old Caucasian woman, lifelong non-smoker with an unremarkable medical history, presented with a 10-month history of progressive breathlessness, dry cough, fatigue, arthralgias, and mild weight loss. The only significant clinical finding was bilateral enlargement of auxiliary lymph nodes. High-resolution computed tomography revealed a soft tissue density mass at the right hilum which was surrounding and narrowing airways and vascular components, nodules with vascular distribution, enlarged mediastinal lymph nodes, and pericardial effusion. Our patient underwent a bronchoscopy, which revealed the presence of submucosal infiltration and narrowing of the right upper bronchus. Endobronchial biopsies showed non-caseating granulomas. As local sarcoid reactions with non-caseating granulomas can be observed near tumors, our patient underwent video-assisted thoracoscopy and surgical removal of an auxiliary lymph node, both of which confirmed the presence of non-caseating granulomas and the diagnosis of sarcoidosis. She was treated with steroids with improvement of clinical and imaging findings. However, while on a maintenance dose, she presented with a pleural effusion, which, after the diagnostic work-up, proved to be sarcoidosis-related. Treatment with initially high doses of steroids plus a steroid-sparing agent led to resolution of the effusion. CONCLUSIONS: We report a case with a high initial index of suspicion for lung malignancy. Clinicians should always be aware that sarcoidosis enters the differential diagnosis of patients presenting with a lung mass that encases and narrows bronchial and vascular structures with associated pericardial effusion. Rarely, pleural effusion can be the presenting symptom of disease relapse despite maintenance treatment.

Investigation of Telomerase/Telomeres system in Bone Marrow Mesenchymal Stem Cells derived from IPF and RA-UIP.

OBJECTIVE: Idiopathic Pulmonary Fibrosis and Rheumatoid Arthritis associated usual interstitial pneumonia seem to have the same poor outcome as there is not an effective treatment. The aim of the study is to explore the reparative ability of bone marrow mesenchymal stem cells by evaluating the system telomerase/telomeres and propose a novel therapeutic approach. METHODS: BM-MSCs were studied in 6 IPF patients, 7 patients with RA-UIP and 6 healthy controls. We evaluated the telomere length as well as the mRNA expression of both components of telomerase (human telomerase reverse transcriptase, h-TERT and RNA template complementary to the telomeric loss DNA, h-TERC). RESULTS: We found that BM-MSCs from IPF, RA-UIP cases do not present smaller telomere length than the controls (p = 0.170). There was no significant difference regarding the expression of both h-TERT and h-TERC genes between patients and healthy controls (p = 0.107 and p = 0.634 respectively). CONCLUSIONS: We demonstrated same telomere length and telomerase expression in BM-MSCs of both IPF and RA-UIP which could explain similarities in pathogenesis and prognosis. Maintenance of telomere length in these cells could have future implication in cell replacement treatment with stem cells of these devastating lung disorders.