CXCR4 pos circulating progenitor cells coexpressing monocytic and endothelial markers correlating with fibrotic clinical features are present in the peripheral blood of patients affected by systemic sclerosis

There is still controversy regarding the role of circulating endothelial and progenitor cells (CECs/CEPs) in the pathogenesis of systemic sclerosis (SSc). Using a sequential Boolean gating strategy based on a 4-color flow cytometric protocol, an increased number of CD31(pos)/CD184(pos)(CXCR4)/CD34(pos)/CD45(pos) and CD31(pos)/CD117(pos) (c-kit-R) /CD34(pos)/ CD45(pos) hematopoietic circulating progenitor cells (HCPCs) was detected in SSc patients compared with healthy subjects. In SSc, no circulating mature and progenitor endothelial cells were observed, while an enhanced generation of erythroid progenitor cells was found to be correlated with the presence of CD117+ HCPCs. The presence of freshly detected CXCR4posHCPC was correlated either to the in vitro cultured spindle-shaped endothelial like cells (SELC) with an endo/myelomonocytic profile or to SDF-1 and VEGF serum level. These data are related to more fibrotic clinical features of the disease, thus supporting a possible role of these cells in fibrosis

Bronchoalveolar lavage in systemic sclerosis with lung involvement: role and correlations with functional, radiological and scintigraphic parameters

To evaluate the role and the prognostic value of bronchoalveolar lavage (BAL) in scleroderma patients withinterstitial lung disease. We reviewed the records of 79 patients with systemic sclerosis (SSc) who had dyspnea and pulmonary involvement and underwent BAL study. Sixty-two patients were prospectively followed up for 12–36 months and re-evaluated by pulmonary function tests (PFTs). Seventy-nine SSc patients were enrolled (71 F and 8 M), 55 with limited and 24 with a diVuse form; mean age55 +/- 13 years; mean disease duration 55.2 +/- 59 months. All patients were ANA positive, of these 30 were anti-topoisomerase-1 positive (anti-Topo1) and 22 were anti-centromere positive (ACA). Thirty-one patients had alveolitis (39.2%) that was neutrophilic in 12 patients, eosinophilic in 3 and mixed (neutrophilic and eosinophilic) in 16 patients. Compared to patients without alveolitis, those with alveolitis had a significant reduction of carbon monoxide diffusing capacity (DLCO), forced vital capacity (FVC) and more elevated lung high-resolution computed tomography (HRCT) scores. Furthermore, alveolar clearance was signiWcantly accelerated. No differences were found between patients with and without alveolitis regarding disease subsets (diffuse vs limited-SSc); a significant predominance of anti-Topo1 antibodies was foundin the alveolitis group and of ACA antibodies in the non-alveolitiscohort. During the follow-up, (range: 12–36 months) 62patients, 26 with and 36 without alveolitis were re-evaluated with PFTs. In the alveolitis group, 12 patients (46.1%)showed stable lung function parameters and 14 had worsened (53.8%). In this group, 20 patients (77%) received cyclophosphamide (CYC): 11 (55%) worsened (5 of them died of cardio-pulmonary complications) and 9 (45%) remained stable. Six patients could not be treated; of these 3 remained stableand 3 worsened. Among 36 patients with normal BAL, 11 (30.5%) showed stable lung function parameters, 13 improved (36.1%) and 12 worsened (33.3%); in this last group, 2 patients died of extra-pulmonary complications. Six patients, with progression of lung fibrosis, were treated with CYC: 3 of them improved and 3 remained stable. Our study revealed a trend toward a more severe course in the SSc patients with BAL alveolitis; probably the non-significant result is related to the low number of the examined subjects and to the selection criteria. However, BAL remains the only tool to exclude lung infections and, in our experience, a useful instrument to evaluate interstitial lung disease in SSc patients

3ronchial hyperreactivity in systemic sclerosis patients: influence of associated Sjogren's syndrome

Abstract Objective-To determine the frequency and relative risk of bronchial hyperreactivity to methacholine in systemic sclerosis patients with or without associate

Reducing the environmental impact of surgery on a global scale: systematic review and co-prioritization with healthcare workers in 132 countries

Abstract Background Healthcare cannot achieve net-zero carbon without addressing operating theatres. The aim of this study was to prioritize feasible interventions to reduce the environmental impact of operating theatres. Methods This study adopted a four-phase Delphi consensus co-prioritization methodology. In phase 1, a systematic review of published interventions and global consultation of perioperative healthcare professionals were used to longlist interventions. In phase 2, iterative thematic analysis consolidated comparable interventions into a shortlist. In phase 3, the shortlist was co-prioritized based on patient and clinician views on acceptability, feasibility, and safety. In phase 4, ranked lists of interventions were presented by their relevance to high-income countries and low–middle-income countries. Results In phase 1, 43 interventions were identified, which had low uptake in practice according to 3042 professionals globally. In phase 2, a shortlist of 15 intervention domains was generated. In phase 3, interventions were deemed acceptable for more than 90 per cent of patients except for reducing general anaesthesia (84 per cent) and re-sterilization of ‘single-use’ consumables (86 per cent). In phase 4, the top three shortlisted interventions for high-income countries were: introducing recycling; reducing use of anaesthetic gases; and appropriate clinical waste processing. In phase 4, the top three shortlisted interventions for low–middle-income countries were: introducing reusable surgical devices; reducing use of consumables; and reducing the use of general anaesthesia. Conclusion This is a step toward environmentally sustainable operating environments with actionable interventions applicable to both high– and low–middle–income countries

L'interessamento polmonare in corso di polimisite/dermatomiosite e di connettivite mista.

L'interessamento polmonare è una importante causa di morbilitàù e moratalità nelle poli-dermatomiositi e nellaconnettivite mista. L'interstiziopatia polmonare rappresenta la complicanza più importante e potenzialmente più grave. E' nota l'associazione dell'interstiziopatia con gli anticorpi anti-sintetasi. Nella MCTD la complicanza più grave èp rapprersentata dalla ipertensione polmonare

Helicobacter pylori and Non-Steroidal Anti-Inflammatory Drugs.

The relationship between H.pylori and NSAID use are under discussion. Susceptibility to H.pylori is not increased in NSAIDs users nevertheless an already mucosa damaged by H.pylori may be more susceptible to NSAIDs toxic effects

La riserva funzinale del pancreas esocrino in corso di sindrome di Sjögren [The exocrine pancreatic function in Sjögren's syndrome]

Exocrine pancreatic function was studied in a homogeneous group of 33 female patients aged 42-67 years. Of these patients, 11 were classified as rheumatoid arthritis (RA), 11 as Sjögren's syndrome I (SSI) and 11 as Sjögren's syndrome associated with RA (SSII). Clinical features, laboratory tests and special instrumental techniques excluded gastroenteric-hepatobiliary causes of pancreatic diseases. These patients were subjected to direct pancreatic stimulation with secretin and caerulein (S. Cae test). Test results, compared to control-group (10 voluntary healthy females) showed, in the last 30 m of stimulation, a statistically significant decrease (p<0.05) in duodenal juice volume, bicarbonates and trypsin in 6 cases (54.5%) of SSI and in 3 cases (27.2%) of RA. SSII S. Cae test showed a decrease of volume and bicarbonates in 6 patients (54.5%) and in only 4 of these (36.4%) it was associated with a concomitant decrease in trypsin levels. Authors discuss the subclinical exocrine pancreatic function in relationship to siccasyndrome, possible immunological factors and primary disease of pancreatic ducts

CERTAINTIES AND CONTROVERSIES OF TB DISEASE. DESCRIPTION OF TWO CLINICAL CASES

Introduction and aim. Unspecific clinical presentation of Tuberculosis (TB) and similarity of its signs and symptoms with other diseases make its diagnosis difficult, especially in low burden settings. Early TB diagnosis is extremely important especially when diagnostic delay may result in complications and prolonged treatment as in the patients herein described. Case description. Two cases were enrolled in the Infection Diseases Unit of Ferrara. The first one was a 85 year old Italian man with an history of colic and gastric cancer, surgically treated. In August 2014 he was hospitalized in a Medical Unit for the onset of fever, cough and weight loss. Chest X-ray and CT scan revealed an interstitial pneumonia and a “tree in bud” pattern respectively, diagnosed as “bronchiolitis” and treated with piperacillin/tazobactam and clarithromycin. Two weeks later all symptoms relapsed and the patient was again hospitalized. A chest CT scan showed again the same pattern. A new diagnosis of lung metastases was formulated and a steroid therapy was promptly begun. One week later, owing to a new clinical worsening, the patient was admitted in our Unit, because of unavailability in other wards. On the basis of CT scan, microbiological, citologic and histologic specimens were collected by bronchoscopy and examined. A M. tuberculosis (MTB) PCR performed on brochoalveolar lavage (BAL) resulted positive. The second patient was a 46 year old Moroccan woman suffering from weight loss, fever, cough and night sweats. She also suffered from eosinophilia, epistaxis and dyspnea responsive to bronchodilators. Chest X-ray revealed multiple pulmonary consolidations, confirmed by CT scan which described also a “honeycomb” pattern. MTB search in blood, urine and BAL resulted negative. A “non bacillary TB” was diagnosed and the patient was treated with anti-TB standard regimen. Due to the onset of vision disorders, an opthalmological evaluation and a retinal fluorangiography were required and disclosed a bilateral chorioretinitis which needed discontinuation of ethambutol. While anti-TB treatment was in progress, all the symptoms relapsed. The patient was hospitalized in our Unit, in respiratory isolation precaution. A new chest CT scan showed migrant pulmonary infiltrates with “honeycomb and ground-glass” pattern. IgE dosage was normal. A new bronchoscopy was performed and MTB search was again negative. In the suspect of a Churg-Strauss vasculitis, ANCAs were required and resulted positive. A lung biopsy confirmed this diagnosis. Both patients are now in good physical conditions and symptom-free. The Italian patient has interrupted steroids and started anti-TB therapy, in contrast to the Moroccan woman. Conclusion. TB remains an elusive disease even in countries with advanced medical technology. We underline that difficulties may be encountered in the differential diagnosis due to similar constitutional symptoms and Rx findings peculiar of TB and other non-infectious diseases

Idiopatic inflammatory myopathies: serological and clinical correlations. Analysis of a monocentric series

Objectives. To correlate the serological data obtained with line blot kits for myositis specific antibodies (MSA) and myositis associated antibodies (MAA) with clinical and instrumental data of a monocentric series of patients with idiopathic inflammatory myopathies (MII). Methods. Sera from 60 patients (related to the clinic dedicated to MII) were tested with the EUROIMMUNE Myositis Profiles 3 and 4 kits, which are in vitro qualitative tests allowing the determination of human IgG autoantibodies directed against 16 different specific antigens: Mi-2α, Mi-2β, TIF1, MDA5, NPX2, SAE1, Ku, PM-Scl 100, PM-Scl 75, Jo-1, SRP, PL-7, PL-12, EJ, OJ, Ro52. Patients were then retrospectively evaluated for both demographic and clinical data (fever, Raynaud's phenomenon (FdR), arthritis, mechanics hand (MH), heliotrope rash, papules and sign Gottron, erythema, skin ulcers, sclerodactyly, neoplasia) at presentation. The presence of interstitial lung disease (ILD) was assessed by High resolution computed tomography (HRCT) (measured GOH score for the extension) and spirometry as Forced vital capacity (FVC) and Diffusing capacity (DLCO). Severe ILD was defined by: GOH score >20 and FVC and DLCO <75%. Summary of the results. Of the 60 patients studied 39 (65%) were females. Twelve (21.7%) were suffering from polymyositis (PM) and antisintetasis syndrome (ASS), 18 (30%) from dermatomyositis (DM), 3 (5%) from paraneoplastic PM, 4 (6.7%) from DM paraneoplastic, 3 (5%) from amyopatic DM, 5 (8.3%) from Scleromyositis, 1 from overlap (PM/systemic lupus erythematosus) whereas 2 had unclassifiable myositis. The MSA found were: Jo1 (15), Mi-2 (7), SRP (5), TIF1 (4), NPX2 (2), SAE (2), MDA5 (2), OJ (2), PL7 (1). The MAA were: Ku (3), Ro52 (12), PM-Scl (13). Five patients (8.3%) were positive for 2 or more MSA. The association between MSA and MAA, in particular between Jo1 and Ro52, was frequent (p <0.05). Jo-1, Mi-2, PM-Scl and SRP were associated with ASS, DM, amyopatic DM and PM, respectively (p <0.05). Severe ILD was associated with Jo-1 (p <0.05). Comparing the mean values, Jo-1, Ro 52 and PM-Scl positive (after eliminating Jo-1 positive from the comparison group for the last two antibodies) had GOH values basically higher and lower values of FVC and DLCO, at p <0.05 for GOH and FVC in Jo-1 and PM-Scl positive, and FVC in Ro52 positive. Patients Mi-2, SRP and TIF1 positive, were less affected by ILD with GOH values significantly lower (p <0.05) (see Table). TIF1 was associated with paraneoplastic DM (p <0.05) with a negative predictive value and positive, respectively of 94% and 75% (OR 44.25). Finally the following associations of antibody with clinics were detected (p <0.05): Jo1 with arthritis and MH, Ku with sclerodactyly and FdR, Mi-2 with erythema, MDA5 with skin ulcers. Concluding statements: MSA and MAA play an important role in diagnosis and prognostic stratification of patients with MII allowing to identify patients at higher risk of developing severe complications (cancer, ILD)