Savino Sciascia

Università degli Studi di Torino | UNITO · Dipartimento di Scienze Cliniche e Biologiche

Objective: To develop new antiphospholipid syndrome (APS) classification criteria with high specificity for use in observational studies and trials, jointly supported by the American College of Rheumatology (ACR) and EULAR. Methods: This international multidisciplinary initiative included 4 phases: 1) Phase I, criteria generation by surveys and...

Objective To develop new antiphospholipid syndrome (APS) classification criteria with high specificity for use in observational studies and trials, jointly supported by the American College of Rheumatology (ACR) and EULAR. Methods This international multidisciplinary initiative included four phases: (1) Phase I, criteria generation by surveys and...

Treatment-refractory lupus nephritis (LN) has a high risk of a poor outcome and is often life-threatening. Here we report a case series of six patients (one male and five females) with a median age of 41.3 years (range, 20–61 years) with refractory LN who received renal biopsies and were subsequently treated with intravenous daratumumab, an anti-CD...

Long-term vitamin K antagonist (VKA) anticoagulation is the cornerstone of the management of subjects with thrombotic antiphospholipid syndrome (APS). Recent investigations have opened up new discussion points regarding the potential for stopping anticoagulant medication in patients with a history of thrombotic APS who no longer have detectable aPL...

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) encompasses a group of rare, multisystem autoimmune disorders characterised by the occurrence of inflammation and damage to small blood vessels, leading to a wide range of clinical manifestations. They include granulomatosis with polyangiitis (GPA) and microscopic polyangiitis...

Objectives In this large multicentre study, we compared the effectiveness and safety of tocilizumab intravenous versus subcutaneous (SC) in 109 Takayasu arteritis (TAK) patients. Methods We conducted a retrospective multicentre study in referral centres from France, Italy, Spain, Armenia, Israel, Japan, Tunisia and Russia regarding biological-targ...

Background and Aims Systemic amyloidosis is a cluster of disorders characterized by tissue deposition of amyloid (highly ordered fibrils composed of low molecular weight subunits of a variety of proteins). Transthyretin (TTR) amyloidosis (ATTR) is either an autosomal dominant inherited condition (ATTRv, where v stands for “variant”) or a non heredi...

Background and Aims The introduction of innovative therapies, resulting from revisiting cancer as a disease of the immune system, has changed the scenario of complications. These new classes of drugs, such as targeted therapies and immune checkpoint inhibitors, assure substantial advantages in cancer therapy, despite some side affecting various org...

Background and Aims Rituximab (RTX) has shown to be an effective induction treatment for small-vessel vasculitides associated with antineutrophil cytoplasm antibodies (AAV) in both newly diagnosed and relapsing patients. However, the role of RTX in the management of the most severe cases of AAV remains to be fully elucidated. The aim of this study...

Introduction To date, almost 7 billion doses of the different types of vaccine against SARS-CoV-2 have been administered worldwide. Although the severity of new cases of SARS-CoV-2 has progressively decreased, and the pressure on national health systems has declined, the development of de novo glomerular injuries has been suggested. Methods This s...

Background and Aims A considerable number of patients with high clinical suspicion for cryoglobulinaemic vasculitis either show negative results for the detection of cryoglobulins or show only trace amounts which cannot be characterized for composition. We aimed at establishing whether the failure to detect or the detection of trace amounts of cryo...

Background and Aims Remarkable results in severe HCV-related cryoglobulinemic vasculitis have been obtained with Rituximab. Details of the clinical characteristics and effective treatment of non HCV-related cryogloulinemic syndromes are presently lacking. Method This paper reports on a prospective single-Center open study aimed at evaluating the c...

Background and Aims Thrombotic microangiopathies, comprising atypical hemolytic uremic syndrome (aHUS) and other diseases, can present with a broad clinical and histopathological spectrum. On our way to an evidence-base for the nephropathological work-up of TMAs, we have chosen a machine-learning approach, thus eliminating suboptimal reproducibilit...

Background and Aims The aim of this prospective study is to investigate the long term safety and efficacy of intesified B-cell depletion therapy (IBCDT) in patients with active lupus nephritis (LN), in comparison to a conventional immunosuppresant therapy, followed by a 3 year maintenence micophenolate mofetil (MMF) regimen. Method Thirty patients...

Background and Aims Daratumumab is an anti-CD38 monoclonal antibody recently approved as a first-line therapy on top of standard therapy for the treatment of multiple myeloma and AL amyloidosis. The mechanism of action of Daratumumab is based on its ability to bind CD38, a transmembrane receptor expressed in particular by pathogenic plasma cells, i...

Background and Aims Machine learning (ML) holds great promise for improving diagnostics, prognostication and theranostics in nephropathology. So far, applications have not gone much further than segmentation of tissue compartments on whole slide images (WSIs) of paraffin sections. As a proof-of-concept study, we describe the development of a diagno...

Introduction: While the use of different immunosuppressants has been investigated in immunoglobulin A nephropathy, further investigation is needed to assess the effect of a regimen of mycophenolate mofetil combined with a short course of glucocorticosteroids in the subset of patients with histologically active features. We compared the efficacy an...

Objectives: Our primary objective was to quantify damage burden measured by Damage Index for Antiphospholipid Syndrome (DIAPS) in antiphospholipid antibody (aPL)-positive patients with or without a history of thrombosis in an international cohort. Secondly, we aimed to identify clinical and laboratory characteristics associated with damage in aPL-...

Background Patients on B-cell depleting agents may have a suboptimal response to vaccination, placing them at a higher risk of contracting SARS-CoV-2 or suffering from a more severe prognosis. Indeed, available data on pre-exposure prophylaxis with tixagevimab/cilgavimab (Evusheld) in subjects with glomerular diseases (GD) who received rituximab is...

(1) Background: Immunological laboratory testing is known to be complex, and it is usually performed in tertiary referral centers. Many criticalities affect diagnostic immunological testing, such as limited availability, the need for specifically trained laboratory staff, and potential difficulties in collecting blood samples, especially in the mos...

Diagnosis of autoimmune diseases is in most cases challenging for clinicians as there is not a single specific laboratory or histological marker to diagnose or exclude the presence of the conditions. This review focused on the current knowledge of the role of autoantibodies' testing in various diseases, such as systemic lupus erythematosus, rheumat...

Background The nature of the deposits in immune-mediated glomerulonephritis with a membranous pattern and masked IgG-Kappa deposits (MGMID) remains still to be elucidated. Case presentation We present a case of 33-year-old woman developing a continuous asymptomatic proteinuria (0.8–1 g/24 h) with no overt connective tissue diseases. She tested pos...

Introduction: Significant heterogeneity still exists in the nomenclature of renal involvement in antiphospholipid syndrome (APS). Methods: We applied a hierarchical cluster analysis to determine subgroups of patients according to clinical, laboratory, and renal histology characteristics in a cohort of subjects with confirmed antiphospholipid ant...

A syndemic is the co-existence of two or more health problems (including both social and biological features) that adversely influence each other with negative consequences on disease outcomes and perpetuation of inequalities. The syndemic approach can be applied to better understand the course of rheumatic musculoskeletal diseases (RMD) involving...

Unlabelled: The prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies [ANCAs] in patients with lupus nephritis [LN] is not fully elucidated. Our aim was to determine whether LN patients with ANCA positivity had different clinicopathological features and outcomes compared to ANCA-negative patients. Methods: Among our LN p...

We aimed to investigate the epidemiology, the clinical and laboratory characteristics of the pediatric involvement of antiphospholipid syndrome (APS), by performing a review of the current evidence and reviewing local experience in the Northwest Italy. To achieve this, we performed a detailed literature search to identify articles describing clinic...

Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and re...

Objective: Our study aimed to elucidate the presence, antigen specificities, and potential clinical association of anti-neutrophil extracellular trap (anti-NET) antibodies in a multinational cohort of antiphospholipid antibody (aPL)-positive patients who did not have lupus. Methods: Anti-NET IgG/IgM were measured in sera of 389 aPL-positive pati...

Introduction: Rare diseases (RD) are a health priority worldwide, overall affecting hundreds of millions of people globally. Early and accurate diagnosis is essential to support clinical care but remains challenging in many countries, especially the low- and medium-income ones. Hence, undiagnosed RD (URD) account for a significant portion of the o...

Urinary and serological markers play an essential role in the diagnostic process of autoimmune diseases. However, to date, specific and reliable biomarkers for diagnosing Behçet’s disease (BD) are still lacking, negatively affecting the management of these patients. To analyze the currently available literature on serological and urinary BD biomark...

Systemic lupus erythematosus (SLE) is characterized by an aberrant immune response, leading to an extremely heterogeneous clinical presentation, potentially affecting different systems and organs. Despite the fact that SLE mortality has greatly decreased since the introduction of steroids, some forms of refractory/severe SLE still have the potentia...

Introduction The introduction of innovative therapies, resulting from revisiting cancer as a disease of the immune system, has changed the scenario of complications. These new classes of drugs, such as targeted therapies and immune checkpoint inhibitors, assure substantial advantages in cancer therapy, despite some side effects affecting various or...

Refractory lupus nephritis (RLN) is a clinical condition with high risk of a poor outcome and often life-threatening. Six patients (one male and 5 females), aged 41.3 years (range 20 to 61 years) were treated with Daratumumab monotherapy, a monoclonal antibody targeting CD38 which is highly expressed on the surface of many immune cells, especially...

Fibrosis can be defined as a pathological process in which deposition of connective tissue replaces normal parenchyma. The kidney, like any organ or tissue, can be impacted by this maladaptive reaction, resulting in persistent inflammation or long-lasting injury. While glomerular injury has traditionally been regarded as the primary focus for class...

Chronic kidney disease (CKD) is a widely diffuse pathological condition which deeply impacts upon an affected patient’s quality of life and its worldwide rate is predicted to further rise. The main biological mechanism underlying CKD is renal fibrosis, a non-reversible process representing, for the affected system, a point of no return of tissue da...

Renal flares constitute major determinants of poor prognosis in people living with systemic lupus erythematosus (SLE). The aim of the present study was to investigate changes in B cell subsets in relation to renal flares upon initiation of standard therapy (ST) plus belimumab or placebo in patients with SLE. Using data from the BLISS-76, BLISS-SC,...

The etiological complexity of Behçet syndrome (BS), an immune-mediated rare form of vasculitis characterized by multi-organ involvement, is still elusive due to an incomplete understanding of the synergy between genetic susceptibility, environmental triggers, and an abnormal immune response. Long-standing theories regarding the origins of BS includ...

Objectives: To evaluate the safety and tolerability of belimumab given for 24 months in patients persistently positive for antiphospholipid antibodies (aPL) with clinical features attributable to aPL [refractory and/or non-criteria manifestations of the antiphospholipid syndrome (APS)]. Methods: In this investigator-initiated, single-centre, ope...

The hepatitis B virus (HBV) infection leads to chronic hepatitis, cirrhosis, and hepatocarcinoma. However, about 20% of patients experience extrahepatic manifestations such as polyarteritis nodosa, non-rheumatoid arthritis, non-Hodgkin lymphoma, cryoglobulinemic vasculitis, and glomerulonephritis. These influence the patient’s morbidity, quality of...

Antiphospholipid syndrome (APS) is an autoimmune condition characterized by thrombosis (arterial, venous, and microvascular) and/or pregnancy morbidity occurring in subjects persistently positive for antiphospholipid antibodies (aPL). While the APS classification criteria are being currently updated to improve homogeneity in clinical research, pati...

Background/Purpose APS ACTION Registry was created to study the outcomes of patients with persistently positive antiphospholipid antibodies (aPL) with or without other systemic autoimmune disease (SAIDx). Given that immunosuppression (IS) is used for certain aPL manifestations, for example, thrombocytopenia (TP), our primary objective was to descri...

Renal-limited hemophagocytic syndrome (HPS) is a rare clinical setting characterized by abnormal activation of the immune system. Fever associated with pancytopenia, hepatosplenomegaly with liver dysfunction, and hypofibrinogenemia are usually observed in HPS. From a histological level, the presence of non-malignant macrophages infiltrating bone ma...

Objective To assess predicting factors that might influence Systemic lupus erythematosus (SLE) disease activity in women in an extended follow-up period of 2 years after giving birth and clinical assessment every three months. Methods The study was design as an international retrospective study, enrolling 119 women with a first birth and with a 2...

Purpose To investigate changes in B cell subsets in relation to renal flares upon initiation of standard therapy (ST) plus belimumab or placebo in patients with systemic lupus erythematosus (SLE). Methods We analysed data from the BLISS-76, BLISS-SC and BLISS Northeast Asia trials. Circulating CD19+ B cell subsets were characterised through flow-c...

Purpose Complement system is a key-player in the pathogenesis of systemic lupus erythematosus (SLE); its decreases correlate with disease activity and precedes flare. Since synthesis of complement proteins increase during gestational course, it is debated whether complement levels exert a prognostic role in pregnant women with SLE.We performed a ne...

The majority of university curricula for health professionals does not incorporate courses on human nutrition and its links with human and planetary health. This primarily applies to medical and pharmacy students, who have important counselling roles and are at the forefront of public health. To address this important issue, EIT Food recently launc...

Objective: To describe obstetric outcomes based on COVID-19 vaccination status, in women with rheumatic and musculoskeletal diseases (RMDs) who developed COVID-19 during pregnancy. Methods: Data regarding pregnant women entered into the COVID-19 Global Rheumatology Alliance registry from 24 March 2020-25 February 2022 were analysed. Obstetric ou...

Aim To apply thrombin generation assay (TGA) in a large cohort of antiphospholipid antibodies (aPL)-positive patients. Material and methods 108 patients were tested with TGA and lupus anticoagulant (LA) testing and divided according to their aPL profile. Briefly, 21 patients were positive for anti-phosphatidylserine (aPS)/prothrombin (PT) IgG/IgM...

Background Antiphospholipid syndrome (APS) is a multisystemic autoimmune disorder characterized by thrombotic events and/or gestational morbidity in patients with antiphospholipid antibodies (aPL). In a previous single center study, APS-related clinical manifestations that were not included in the classification criteria (livedo reticularis, thromb...

Background Focal segmental glomerular sclerosis (FSGS) is a histologic lesion, rather than a specific disease entity, and represents a cluster of different conditions affecting both children and adults that includes primary, secondary and genetically mediated forms. These forms can be distinguished by electron microscopy (EM) and genetic assessment...

Abstract A considerable number of patients with high clinical suspicion for cryoglobulinaemic vasculitis either show negative results for the detection of cryoglobulins or show only trace amounts which cannot be characterized for composition. We aimed at establishing whether the failure to detect or the detection of trace amounts of cryoglobulin wi...

(1) Background: Autoimmune diseases are characterized by autoantibodies directed to a large number of antigenic targets and are measured using serum as sample matrix. Although serum is a very common specimen type, it comes with certain drawbacks. Most importantly, it depends on venous puncture and requires medical personnel for sampling. This is of...

While the link between cardiovascular and respiratory conditions and air pollution is well-known, recent studies provided a growing body of evidence that polluted air, particularly air with high levels of particulate matter with a diameter smaller than 2.5 micrometers (PM 2.5), can have a range of negative impacts on health, both in terms of mortal...

Background Classification criteria for antiphospholipid syndrome (APS) require that antiphospholipid antibody (aPL) positivity is confirmed after at least 12 weeks. We tested the hypothesis that aPL at high titers remain positive while low titers fluctuate over time. As both platelet-bound C4d (PC4d) and aPL are associated with thrombosis in system...

Self-supervised learning (SSL) methods are enabling an increasing number of deep learning models to be trained on image datasets in domains where labels are difficult to obtain. These methods, however, struggle to scale to the high resolution of medical imaging datasets, where they are critical for achieving good generalization on label-scarce medi...

Background: AL amyloidosis is a systemic disorder characterized by extracellular deposition of characteristic fibrils that results in progressive multi-organ failure and premature death. Recently daratumumab has been demonstrating higher hematological and organ response rates when compared to the standard of care. We hereby report our long-term exp...

Background: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a cluster of potentially life-threatening disorders often involving the kidney with a necrotizing crescentic glomerulonephritis with scanty deposition of immunoglobulins and complement. Historically the role of complement has been considered ancillary. Recently,...

Objectives To describe the outcomes of pregnancies in antiphospholipid antibody (aPL)-positive patients since the inception of the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking Registry. Methods We identified persistently aPL-positive patients recorded as ‘pregnant’ during prospective follow-up, and defined ‘a...

Objectives We aimed to apply and compare the QRISK3 and the adjusted Global AntiPhospholipid Syndrome (APS) Score (aGAPSS) in a cohort of systemic lupus erythematosus (SLE) patients, with and without a concomitant diagnosis of APS, in order to assess their augmented risk of developing cardiovascular diseases (CVDs). Methods Patients (25–85 yo) wit...

Background Patients with autoimmune diseases require appropriate pregnancy counceling, as their disease can negatively impact both maternal and fetal outcomes. In regards to Behçet’s disease (BD), although commonly diagnosed during the childbearing age, only few studies focused on its impact on gestation, with contradictory results. Objectives We...

Background Cardiovascular diseases (CVDs) represent one of the most life-threatening conditions that can affect SLE patients. Assessing the potential CVD risk of these patients is still a challenge and an important aspect in the clinical practice. Recently the QRISK3 score has attempted to encompass for SLE augmented thrombotic risk by adding items...

Background Vulnerable subjects, including systemic lupus erythematosus (SLE) patients have been prioritised to receive anti-SARS-CoV-2 vaccine. Questions have been raised about the effect of vaccines on immunity and their potential role as trigger for flare. Few data about the safety of these vaccines in SLE are available Objectives To investigate...

Background Data on fluctuation of antibodies directed against domain 1 (anti-D1) of β2-glycoprotein I (β2GPI) are scarce. Patients with antiphospholipid syndrome (APS) and all three criteria tests for antiphospholipid antibodies (aPL) display higher titers of anti-D1, which correlate with anti-β2GPI levels. Objectives This project aims at evaluati...