RESUMEN
ANCA-associated vasculitides (AAVs) are characterised by heterogeneous molecular and pathophysiological traits, causing ambiguous differential diagnosis and taxonomy. Response to therapy has proven far from successful, contributing to high mortality. Transcriptome analysis of different vasculitis subtypes adds new leads in elucidating mechanisms of disease and the role of specific cell subsets to them. Recent findings have shown that mitophagy is a procedure whose imbalance could lead to immune dysregulation with certain involvement to autoimmunity. Inflammatory response related mitophagy is yet to be described in AAVs. We here describe a research protocol to investigate mitophagy in monocytes, neutrophils, and T cells in AAV patients, and the relationship of disturbed mitophagy with ANCA seropositivity.
RESUMEN
The therapeutic armamentarium in Systemic Lupus Erythematosus (SLE) is expanding with the introduction of novel biologic and small-molecule agents. Complementary to randomized controlled trials, registry-based studies are advantageous due to the inclusion of a wider range of patients from daily practice and the potential for long-term monitoring of the efficacy and safety of therapies. Moreover, data from registries can be used to identify disease phenotypes that best respond to biologic agents, and to correlate clinical response with parameters such as co-administered therapies and comorbidities. In this project, we will use the configuration of the Hellenic Registry of Biologic Therapies for inflammatory arthritides in order to design a dedicated SLE module with variables pertaining to global and organ-specific disease activity, severity, flares, organ damage/outcome, comorbidities and adverse events. The second stage will involve the pilot implementation of this platform for the multicentric registration of SLE patients who are treated with belimumab. The significance lies in the development of a structured registry that enables the assessment of the disease burden and the long-term efficacy and safety of existing and future biological agents in SLE. Piloting the registry can serve as a basis for establishing nationwide collaborative efforts.
RESUMEN
Pregnancy in women with SLE (Systematic Lupus Erythematosus) is considered of "high risk" since it has been related with adverse events both in the mother and the foetus. Many studies have reported relapse of the disease during the pregnancy and the first trimester post-labour, while others have found no difference in terms of frequency and type of relapses. Moreover, adverse obstetrical events like recurrent pregnancy loss, preeclampsia, prematurity, intrauterine growth restriction and neonatal lupus syndrome tend to occur more often in patents with SLE. However, most of these data regarding the burden and pregnancy outcomes in SLE come from retrospective studies of previous decades, and in non-Caucasian patients. To this end, more recent studies have suggested overall improved outcomes of pregnancy, still their results are often conflicting. The purpose of this study is to record, through a prospective observational (non-interventional) study, the contemporary prognosis of pregnancy in women with SLE who are followed-up by private and hospital physicians in Greece. In particular, we aim to establish a registry to study the course of the disease during pregnancy, the outcome of pregnancy and the possible negative or positive prognostic factors, the effect of drugs on pregnancy and the foetus.
RESUMEN
BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by significant clinical heterogeneity with early diagnosis being a major challenge, complicated by the absence of formal diagnostic criteria. Instead, classification criteria have been developed to enable the homogenous inclusion of patients in clinical trials, with the most commonly used those of the American College of Rheumatology (ACR 1997) and the Systemic Lupus International Collaborating Clinics Classification Criteria (SLICC 2012). These criteria are widely used in clinical practice as diagnostic tools, although they fail to diagnose up to 20% of patients with SLE or may delay diagnosis. These restrictions have led to the recent (2018) introduction of new classification criteria jointly by the European League Against Rheumatism (EULAR) and ACR. AIMS OF THE STUDY: We will compare the sensitivity and specificity of the earlier and new classification criteria after a systematic analysis (retrospective study) of a group of SLE patients. In addition, we will examine which set of criteria permits the earliest classification of the disease in a prospective cohort of patients with undifferentiated connective tissue disease (UCTD). The prognostic impact (permanent organ damage) of the classification of SLE patients with the three sets of criteria will also be examined. METHODS: Data from the existing Cretan lupus registry will be used to retrospectively include consecutively registered patients aged ≥15 years diagnosed with SLE during 01/2005-12/2016 by an expert physician and followed-up for at least 6 months. All sets of criteria (ACR 1997, SLICC 2012, EULAR/ACR 2018) will be tested at the time of physician-based diagnosis and also at last follow-up. A prospective study arm will include cases with a diagnosis of UCTD and will be followed-up in the outpatient clinic for 3-5 years. ANTICIPATED BENEFITS: This is the first study to include the application of the new criteria (EULAR/ACR 2018) to a group of SLE patients. Determining their diagnostic value in comparison to existing criteria or diagnosis by a specialist will provide important information both for the value of their application at the level of clinical studies and for their use in clinical practice as diagnostic criteria.