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Abstract 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.

Abstract 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.

A quiz related to articles published within the issue is presented including shortness of breath in a smoker, the academic performance of twins and singletons and the tests for splenomegaly.

During the past decade important progress has been made regarding the pathogenesis of idiopathic pulmonary fibrosis (IPF), which is the most devastating form of idiopathic interstitial pneumonia with a median survival of 3 years. The knowledge gained has been used to design multicentre, randomised, placebo-controlled trials in order to investigate agents with different mechanisms of action. Encouraging results have led to licensing of the first IPF-specific drug, pirfenidone. However, the road to successful treatment is still long. The main aim for the future should be the careful design of clinical trials, by choosing the most clinically meaningful end-point and keeping in mind that combination of various agents may be more effective. This approach has been used in the treatment of lung cancer with which IPF presents many similarities.

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.

Increasing evidence implicates angiogenesis in the pathogenesis of fibrotic lung diseases. Distinct angiogenic profiles may, in part, explain differences in immunopathogenesis, clinical course and prognosis. The aim of the study was to seek evidence of involvement of the angiogenic axis Angiopoietin-1 and -2 and their tyrosine kinase receptor, Tie-2 in pathogenesis of idiopathic pulmonary fibrosis (IPF) and interstitial pneumonias associated to collagen tissue disorders (CTD-IPs). We prospectively studied 36 patients with IPF, 23 patients with CTD-IP and 10 healthy subjects. Ang-1, Ang-2 and Tie-2 mRNA expression and protein levels were measured in bronchoalveolar lavage fluid pellets and supernatants, respectively. A statistically significant decrease of Ang-1 protein level has been found in IPF in comparison to controls (p=0.02). We also detected an increased expression of Ang-2 protein in IPF in comparison to CTD-IPs. A significant co-expression was detected between Ang-2 and Tie-2 in protein level (p=0.007) in IPF group. In conclusion, a suppression of the angiogenetic factor Ang-1 was observed at the protein level in IPF which may be important in the pathogenesis of this devastating disease. A differential angiogenetic profile regarding Ang-2 was detected between IPF and CTD-IPs.