Functional magnetic resonance imaging for the assessment of autonomic dysfunction in patients with antineutrophil cytoplasmic antibody-associated vasculitides.

Introduction: Nervous system involvement is common in antineutrophil cytoplasmic antibody-associated vasculitides (AAV). While the involvement of the peripheral and central nervous system is well described, it is still unclear how and to what extent the autonomic nervous system (ANS) is affected. Functional magnetic resonance imaging (fMRI) can provide information on both structure and potential damage of the brain, as well as on the function of selected brain centers.

Multimorbidity in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Results From a Longitudinal, Multicenter Data Linkage Study.

Objective: Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is considered a chronic, relapsing condition. To date, no studies have investigated multimorbidity in AAV nationally. This study was undertaken to characterize temporal trends in multimorbidity and report excess health care expenditures associated with multimorbidities in a national AAV cohort from Scotland.

Characterizing infection in anti-neutrophil cytoplasmic antibody-associated vasculitis: results from a longitudinal, matched-cohort data linkage study.

Objectives: Infection exerts a major burden in ANCA-associated vasculitis (AAV), however, its precise extent and nature remains unclear. In this national study we aimed to longitudinally quantify, characterize and contextualize infection risk in AAV.

Methods: We conducted a multicentre matched cohort study of AAV.

Treatment and its side effects in ANCA-associated vasculitides - Study based on POLVAS registry data.

Purpose: The aim of this study is to present the treatment modalities and associated side effects in a Polish nation-wide ANCA-associated vasculitides (AAV) patients' cohort.

Materials And Methods: Retrospective analysis of patients diagnosed with AAV between 1990 and 2016, included in the POLVAS registry was performed. Standard descriptive statistic methods were used with an emphasis on the treatment modalities.

Clinical characteristics of Polish patients with ANCA-associated vasculitides-retrospective analysis of POLVAS registry.

Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers-members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS).

Methods: We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome.

The European Vasculitis Society 2016 Meeting Report.

The 2016 European Vasculitis Society (EUVAS) meeting, held in Leiden, the Netherlands, was centered around phenotypic subtyping in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). There were parallel meetings of the EUVAS petals, which here report on disease assessment; database; and long-term follow-up, registries, genetics, histology, biomarker studies, and clinical trials. Studies currently conducted will improve our ability to discriminate between different forms of vasculitis.

Are the 1990 American College of Rheumatology vasculitis classification criteria still valid?

Objectives: Advances in diagnostic techniques have led to better distinction between types of vasculitis, potentially affecting the utility of the 1990 ACR classification criteria for vasculitis. This study tested the performance of these criteria in a contemporary vasculitis cohort.

Methods: The Diagnosis and Classification in Vasculitis Study provided detailed clinical, serological, pathological and radiological data from patients with primary systemic vasculitis and clinical context-specific comparator conditions.

Development of a score for assessment of radiologic damage in large-vessel vasculitis (Combined Arteritis Damage Score, CARDS).

Objectives: Outcome assessment in large-vessel vasculitis (LVV) remains challenging and this impairs patient management and the conduct of clinical studies. Previous proposals for outcome tools have not included imaging. This study aimed to develop an imaging score to quantify damage in LVV and to assess the difference between Takayasu (TAK) and giant cell arteritis (GCA).

Skewing toward Treg and Th2 responses is a characteristic feature of sustained remission in ANCA-positive granulomatosis with polyangiitis.

The objective of our study was to evaluate the T-helper (Th) and regulatory T (Treg) cell profile in ANCA-positive granulomatosis with polyangiitis (GPA) and its relation to disease activity. In a prospective study, we studied two groups of GPA patients: (i) disease flare (active-GPA, BVAS>6, n = 19), (ii) sustained remission (≥ 1-year prior enrollment, inactive-GPA, BVAS = 0, n = 18). 24 age-sex matched healthy subjects served as controls.

Linear perturbation renormalization group for the two-dimensional Ising model with nearest- and next-nearest-neighbor interactions in a field.

The linear perturbation renormalization group (LPRG) is used to study the phase transition of the weakly coupled Ising chains with intrachain (J) and interchain nearest-neighbor (J_{1}) and next-nearest-neighbor (J_{2}) interactions forming the triangular and rectangular lattices in a field. The phase diagrams with the frustration point at J_{2}=-J_{1}/2 for a rectangular lattice and J_{2}=-J_{1} for a triangular lattice have been found. The LPRG calculations support the idea that the phase transition is always continuous except for the frustration point and is accompanied by a divergence of the specific heat.

How to treat ANCA‑associated vasculitis: practical messages from 2016 EULAR/ERA‑EDTA recommendations.

The European League against Rheumatism (EULAR) with the European Renal Association - European Dialysis and Transplant Association recently published an update of 2009 EULAR recommendations with a focus on the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). In this article, we discuss the following key messages for clinical practice derived from these recommendations: 1) biopsy should be performed if possible to confirm new diagnosis or relapse; 2) glucocorticoid therapy is an extremely important adjunct to the management of AAV, but it is also responsible for the majority of adverse effects; the dose should be tapered to 7.5 to 10 mg/d at 3 to 5 months; 3) cyclophosphamide or rituximab are the mainstay of remission induction; 4) patients with major relapse should be treated like those with new disease, but rituximab is the preferred option in those patients who relapse after prior cyclophosphamide; 5) minor relapse should not be treated with glucocorticoid alone, and a change in immunosuppressive regimen should be considered; 6) rituximab can be used not only for remission induction but also for maintenance; 7) maintenance therapy should continue for at least 2 years, after which gradual taper could be considered; 8) while ANCA are extremely useful for diagnosis and rising ANCA levels seem to be associated with relapse, serial monitoring should not guide treatment decisions; 9) monitoring of AAV patients should be holistic with a structured assessment tool and monitoring for effects related to the vasculitis as well as treatment; 10) management should be either at or in conjunction with an expert center; and 11) patients should be involved in decision making and have access to educational resources.

Effectiveness of plasmapheresis in ANCA clearance in patients with ANCA-associated vasculitides.

Introduction: Plasmapheresis is a therapeutic method based on removal of high molecular weight particles from blood. It is used in a variety of clinical entities in which pathogenic role of such particles has been proven e.g.

Thermodynamics of weakly coupled Falicov-Kimball chains from renormalization-group theory.

The linear perturbation renormalization group is used to study spinless two-band fermion chains at half-filling. The model consists of two species of spinless fermions, namely localized f and extended p, and it takes into account the following: the kinetic energy of fermions p, the on-site Coulomb repulsion V between p and f fermions, chemical potentials μ(p) and μ(f) adjusted in such a way that the average of the site occupation 〈n(f)(i)〉+〈n(p)(i)〉=1, and a weak interchain hopping t(x). The average occupation number, the specific heat, and the correlation functions are studied as functions of temperature.

Optimisation of vasculitis disease assessments in clinical trials, clinical care and long-term databases.

The systemic vasculitides are a group of rare, chronic, relapsing, but often progressive inflammatory conditions. They are associated with a significant burden of morbidity both due to scarring from the disease itself and as a consequence of treatment with glucocorticoids and other potent immunosuppressive agents. Careful assessment of disease activity is critical to guide appropriate use of these potentially toxic therapies.

Lower doses of rituximab in remission induction for refractory granulomatosis with polyangiitis.

Introduction: Recently, rituximab (RTX)--monoclonal antibody against the CD20 molecule on the surface of B-lymphocytes is used in the treatment of antineutrophil cytoplasmic antibody (ANCA) associated vasculitides. Efficacy of the drug administered in so-called lymphoma treatment protocol (4 x 375 mg/m2/week) has been shown not to be inferior to cyclophosphamide. However, some data published lately suggest that rituximab could also be effective in much lower doses, which could lead to reducing side effects, but above all, the cost of the therapy.

Critical point of a para-ferrimagnetic phase transition of the ANNNI model in a field.

The finite field para-ferrimagnetic phase transition in the axial next-nearest-neighbor Ising (ANNNI) model is studied by using the linear perturbation real space renormalization group transformation. The method is examined in systems of ferromagnetic and antiferromagnetic Ising chains coupled by ferromagnetic interactions in the longitudinal field. As one would expect, the external field in the first case destroys the continuous phase transition and in the second shifts the critical point toward a lower temperature according to the square law.

[Effective application of therapeutic plasma exchange in a case of acute dermatomyositis].

We present a case of 22-year-old male with acute dermatomyositis who did not respond to a standard immunosuppressive therapy. Due to rapidly deteriorating clinical status a series of plasmapheresis was performed subsequent to which a quick clinical improvement was observed.

Myocardial ischaemia in systemic lupus erythematosus: detection and clinical relevance.

Background: Severe cardiovascular complications are among the most important causes of mortality in systemic lupus erythematosus (SLE) patients.

Aim: To assess the usefulness of echocardiography, ECG, and coronary artery calcium scoring (CACS) in the detection of myocardial ischaemia in SLE patients compared to single photon emission computerised tomography (SPECT) and to assess their five-year follow-up.

Methods: In 50 consecutive SLE patients (mean age 39.

Reactivity of pulmonary circulation and right ventricle function to inhaled nitric oxide in systemic sclerosis patients.

Systemic sclerosis (SSc) is complicated by pulmonary hypertension and right ventricle (RV) failure in approximately 10% of the patients. Factors influencing the reactivity of pulmonary circulation to vasodilators are not established, while the examination of vasoreactivity is important in determining the treatment, because systemic administration of oral vasodilators can induce severe adverse events in nonresponders. The mechanism of RV failure in SSc is unclear and may result either from increased RV afterload or intrinsic myocardial disease.

Diastolic heart dysfunction, increased pulmonary capillary wedge pressure and impaired exercise tolerance in patients with systemic sclerosis.

Background: Diastolic heart dysfunction, responsible for dyspnoea in heart failure patients, is an important prognostic factor. Patients with systemic sclerosis (SSc) serve as a model of diastolic heart failure with preserved ejection fraction.

Aim: To quantify diastolic left ventricular (LV) dysfunction and elevation of pulmonary capillary wedge pressures (PCWP) in SSc patients and to assess the effects of these parameters on exercise tolerance.

[Polish guidelines for the prevention and treatment of venous thromboembolism: 2009 update].

The overall objective of the Polish guidelines for the prevention and treatment of venous thromboembolism is to increase patient benefit and safety by appropriate prevention and treatment of deep vein thrombosis and pulmonary embolism as well as proper management of the complications associated with antithrombotic and thrombolytic therapy. These guidelines apply to adult trauma, cancer, surgical, and medical patients as well as those at increased risk of venous thromboembolism. Specific recommendations have been formulated for pregnant women, patients requiring surgery while receiving long-term oral anticoagulant treatment, and patients undergoing regional anesthesia and/or analgesia.

2009 evidence-based clinical practice guidelines for diagnosing a first episode of lower extremities deep vein thrombosis in ambulatory outpatients.

Introduction: The GRADE working group has recently suggested a rigorous framework for clinical practice guidelines (CPG) addressing diagnostic tests and test strategies based on the impact of alternative approaches on patient-important outcomes. The framework mandates explicit evidence summaries, ratings of the quality of evidence, and specifying recommendations as strong or weak.

Objectives: To test the feasibility and performance of the GRADE approach, we applied this framework to well-researched issues in the diagnoses of deep venous thrombosis (DVT).

Genetic associations of variants of the high affinity receptor for immunoglobulin E in Wegener's granulomatosis.

Immunoglobulin E (IgE) and the high affinity IgE receptor (FcepsilonRI) have been suggested to contribute to the pathogenesis of autoimmune disorders. Their role in Wegener's granulomatosis (WG) are, however, poorly recognized. We sought a genetic association between laboratory markers for the disease, i.