Influence of geolocation and ethnicity on the phenotypic expression of primary Sjögren's syndrome at diagnosis in 8310 patients: a cross-sectional study from the Big Data Sjögren Project Consortium.

Objectives: To analyse the influence of geolocation and ethnicity on the clinical presentation of primary Sjögren's syndrome (SjS) at diagnosis.

Methods: The Big Data Sjögren Project Consortium is an international, multicentre registry designed in 2014. By January 2016, 20 centres from five continents were participating.

Prehospital Medical Planning for the 2015 Philadelphia Papal Visit.

In September 2015 Philadelphia hosted the World Meeting of Families, which culminated with a two day visit by Pope Francis. Emergency medical services (EMS) planning for the event was a complex process that involved the cooperation of municipal, state, and federal agencies, as well as many private organizations. Because the visit was designated as a National Security Special Event, Philadelphia had to balance the priorities of the United States Secret Service and other law enforcement agencies to keep the Pope and visitors safe with the medical needs of visitors and the ongoing needs of the city.

Systemic vasculitis and the lung.

Purpose Of Review: The purpose of this review is to provide a critical analysis of the recent literature on this topic, with particular focus on the most relevant studies published over the last year.

Recent Findings: Many studies are published every year on the diagnosis, pathogenesis and treatment of pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). The main subjects covered by this article are the pathogenesis, diagnosis and clinical aspects of lung involvement in ANCA-associated vasculitis and non-ANCA-associated vasculitis.

Chronic skin lichenification as unusual presentation of eosinophilic granulomatosis with polyangitis: case report and literature review.

Eosinophilic granulomatosis with polyangitis (EGPA) is an uncommon ANCA-associated systemic small-vessel necrotizing vasculitis. At times, EGPA presenting manifestations can be very different from the usually recognized disease patterns. We report a 52-year-old female patient with 3 years history of itching.

[Planning a Health Residence for Prison Security Measures, Tuscany (Italy)].

Health Residences for Prison Security Measures are facilities hosting psychotic persons who have committed crimes and providing them with personalized rehabilitation and treatment plans to promote their reinstatement in society. The aim of this study was to describe the criteria for planning and designing a prison health residence in the Tuscany region (Italy), to be managed by the regional healthcare service, in line with current regulations, with dedicated staff for providing specific treatment plans and programmes.

Major Salivary Gland Ultrasonography in the Diagnosis of Sjögren's Syndrome: A Place in the Diagnostic Criteria?

Major salivary gland (SG) ultrasonography (US) represents a noninvasive, nonirradiating imaging modality for evaluation of the major SGs in the diagnosis and follow-up of primary and secondary Sjögren syndrome. Structural changes can be visualized as hyperechogenic and hypoechogenic areas, inhomogeneity, and altered echogenicity in general. The reliability of SG-US is poorly investigated, and the definition of US abnormalities varies in previously published studies.

Sjögren syndrome.

Sjögren syndrome (SjS) is a systemic autoimmune disease that primarily affects the exocrine glands (mainly the salivary and lacrimal glands) and results in the severe dryness of mucosal surfaces, principally in the mouth and eyes. This disease predominantly affects middle-aged women, but can also be observed in children, men and the elderly. The clinical presentation of SjS is heterogeneous and can vary from sicca symptoms to systemic disease (characterized by peri-epithelial lymphocytic infiltration of the affected tissue or the deposition of the immune complex) and lymphoma.

Salivary Gland Ultrasonography in Sjögren's Syndrome: Clinical Usefulness and Future Perspectives.

In recent years, salivary gland ultrasonography (SGUS) has emerged as a promising tool for the diagnosis and prognostic stratification of patients with primary and secondary Sjögren's syndrome. Several studies have emphasized that salivary ultrasonography could be a highly specific tool for the diagnosis of the disease. However, before it can be used in daily clinical practice the SGUS procedure needs standardization and validation in larger disease-control groups.

One year in review: systemic vasculitis.

Systemic vasculitis are complex and heterogenous disorders. During the past months great efforts have been made aimed at clarifying disease pathogenesis and at improving patient management and treatment. In this review we summarise the most important scientific contributions on vasculitis pathogenesis, diagnostic tools and treatment published in 2015.

One year in review 2016: Sjögren's syndrome.

Sjögren's syndrome (SS) is a complex heterogeneous disease charactered by a broad spectrum of clinical and serological manifestations, including non-Hodgkin's lymphoma (NHL). Last year, 2015, was an exciting year for research into SS with novel insights into disease pathogenesis, clinical aspects and long-term outcomes. In addition, the use of biologic therapy in SS is rapidly expanding, with new evidence emerging regarding potential therapeutic targets.

Focus on audiologic impairment in eosinophilic granulomatosis with polyangiitis.

Objectives/hypothesis: To evaluate the clinical features of audiologic impairment and its relationship with the nasal, vestibular, and rheumatologic profile in a cohort of patients with eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome.

Study Design: Prospective cross-sectional study.

Methods: Thirty-nine patients with EGPA, considered controlled according to the Birmingham Vasculitis Activity Score and the Vasculitis Damage Index, underwent a complete audiologic evalutaion with otomicroscopy, impedance audiometry, speech audiometry, and auditory brainstem responses; rhinologic evaluation was made by means of fiberoptic endoscopy and nasal cytology; the clinical evaluation was completed with analysis of the facial function and, in patients with referred vertigo, with videonystagmography (VNG).

Asthma Control and Airway Inflammation in Patients with Eosinophilic Granulomatosis with Polyangiitis.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis that occurs in patients with asthma, nasal disease, blood and tissue eosinophilia, and extrapulmonary manifestations.

Objective: The aim of our study was to assess the clinical, functional, and inflammatory status of upper and lower airways in 37 patients with EGPA, examined 6.4 ± 4.

Early diagnosis of primary Sjögren's syndrome: EULAR-SS task force clinical recommendations.

Sjögren's syndrome (SjS) is a systemic autoimmune disease that mainly affects the exocrine glands, leading to generalized mucosal dryness. However, primary SjS may initially present with non-sicca (systemic) manifestations. When these features appear before the onset of an overt sicca syndrome, we may talk of an underlying 'occult' SjS.

Is salivary gland ultrasonography a useful tool in Sjögren's syndrome? A systematic review.

Objective: Ultrasonography (US) is a sensitive tool in the diagnosis of major salivary gland abnormalities in primary Sjögren's syndrome (pSS). The aim of this systematic review was to assess the metric properties of this technique.

Methods: PUBMED and EMBASE databases were searched.

Prolonged Survival in a Patient with a Pancreatic Acinar Cell Carcinoma.

Pancreatic acinar cell carcinoma (ACC) is a rare entity. Herein we present the case of a 50-year-old male patient with an unlimited mass on the pancreatic corpus and tail with peripancreatic effusion and multiple metastases in the liver and spleen. A liver biopsy showed a pancreatic ACC.

One year in review 2015: idiopathic inflammatory myopathies.

Idiopathic inflammatory myopathies (IIM) are a group of rare acquired muscle diseases that mainly affect skeletal muscles. Recently, novel insights into the pathogenesis, diagnosis and treatment of these complex diseases have been provided. Herewith we provided an overview of the most significant literature contributions published over the year.

Ultrasonography of major salivary glands: a highly specific tool for distinguishing primary Sjögren's syndrome from undifferentiated connective tissue diseases.

Objectives: Recently, convincing data have been published on the value of salivary gland ultrasonography (SGUS) in differentiating primary SS from non-immune-mediated sicca syndrome. Limited data are available regarding the diagnostic accuracy of SGUS in distinguishing SS from other rheumatic diseases. The purpose of this study was to assess the usefulness of SGUS in distinguishing patients with SS from those with xerostomia and/or xerophthalmia and a diagnosis of stable UCTD.

Anti-SSA/SSB-negative Sjögren's syndrome shows a lower prevalence of lymphoproliferative manifestations, and a lower risk of lymphoma evolution.

Objective: This study aims to compare clinical and laboratory features of patients who, while satisfying the American European Consensus Group (AECG) criteria for primary Sjögren's syndrome (SS), do not present the positivity for anti-Ro/SSA and/or anti-La/SSB, with patients that meet the AECG criteria and are positive for anti-Ro/SSA and/or anti-La/SSB.

Methods: 548 patients were selected based on the following criteria, and exclusion of patients negative for histopathology but positive for anti-Ro/SSA and anti-La/SSB: 1. Fulfilment of the AECG criteria, 2.

Passive standing as an adjunct rehabilitation intervention after stroke: a randomized controlled trial.

Background: Early physical rehabilitation enhances functional recovery in stroke survivors. Supported standing is a common adjunctive therapeutic practice in subjects with several central nervous diseases who are unable to stand actively. Data on the effect of supported positioning on standing frames in individuals with recent stroke are scarce and contradictory.

Myositis in primary Sjögren's syndrome: data from a multicentre cohort.

Objectives: In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequent while myositis has been reported in 3% of patients. The aim of this study was to describe the prevalence of myositis in a multicentre Italian pSS cohort and to address the clinical manifestations, histological findings and therapeutic strategies.

Methods: Clinical, serological and therapeutic data from a pSS cohort of patients were retrospectively collected.

Salivary gland ultrasonography: a highly specific tool for the early diagnosis of primary Sjögren's syndrome.

Introduction: Recently, a great interest has arisen for salivary gland ultrasonography (SGUS) as a valuable tool for the assessment of major salivary gland involvement in primary Sjögren's syndrome (pSS. The aims of this study were to test the accuracy of SGUS for the early detection of pSSand to compare the diagnostic performance of SGUS with minor salivary gland biopsy (MSGB) and unstimulated salivary flow (USFR) in this context.

Method: Patients with suspected pSS and symptoms duration of ≤5 years were consecutively enrolled in this study.

VEGFR2-Targeted Contrast-Enhanced Ultrasound to Distinguish between Two Anti-Angiogenic Treatments.

The aim of this study was to evaluate the capacity of BR55, an ultrasound contrast agent specifically targeting vascular endothelial growth factor receptor 2 (VEGFR2), to distinguish the specific anti-VEGFR2 therapy effect of sunitinib from other anti-angiogenic effects of a therapy (imatinib) that does not directly inhibit VEGFR2. Sunitinib, imatinib and placebo were administered daily for 11 d (264 h) to 45 BalbC mice bearing ectopic CT26 murine colorectal carcinomas. During the course of therapy, B-mode ultrasound, contrast-enhanced ultrasound and VEGFR2-targeted contrast-enhanced ultrasound were performed to assess tumor morphology, vascularization and VEGFR2 expression, respectively.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management.

Objective: To develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (EGPA).

Methods: The EGPA Consensus Task Force experts comprised 8 pulmonologists, 6 internists, 4 rheumatologists, 3 nephrologists, 1 pathologist and 1 allergist from 5 European countries and the USA. Using a modified Delphi process, a list of 40 questions was elaborated by 2 members and sent to all participants prior to the meeting.