Systemic lupus erythematosus: one year in review 2023

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical manifestations and a relapsing-remitting course. New data regarding pathogenic pathways, biomarkers and clinical manifestations of SLE are emerging, and new drugs and therapeutic protocols have been proposed to improve the control of disease activity. Furthermore, new insights into comorbidities and reproductive health in SLE patients are constantly emerging.This annual review aims to summarise the most relevant data on SLE that was published in 2022

O13 Remission and LLDAS as a target for pregnancy planning in SLE?

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Anticorpi antifosfolipidi e gravidanza: esiti materni e perinatali

Gli anticorpi antifosfolipidi (aPL) sono un gruppo eterogeneo di immunoglobuline dirette contro le proteine plasmatiche che legano i fosfolipidi. Sono presenti nell’1-5% della popolazione, e la presenza di aPL in gravidanza è stata associata ad un aumentato rischio di aborto ricorrente, altre complicanze ostetriche ed eventi vascolari. Dato che i meccanismi patogenetici alla base di tali manifestazioni non sono completamente noti, e l’entità del rischio risulta variabile in statistiche diverse, scopo di tale lavoro è stato approfondire l’analisi della problematica identificando le patologie associate alla presenza di aPL e confrontando il rischio in relazione a tale classificazione. Le pazienti da includere nello studio sono state selezionate tra tutte le gestanti indirizzate consecutivamente all’ambulatorio congiunto Ostetrico Reumatologico dell’Università di Pisa nell’arco di tempo compreso tra il 2001 e il 2016. Sono state suddivise in tre gruppi: uno comprendente donne affette da sindrome da aPL (APS), uno costituito da donne affette da Lupus Eritematoso Sistemico (LES), ed un terzo costituito da pazienti con aPL associati o meno ad altre connettivopatie diverse dal LES. Nella nostra casistica le pazienti senza APS né LES presentavano un miglior outcome ed una minor incidenza di complicanze rispetto agli altri 2 gruppi, con differenze statisticamente significative per quanto riguarda epoca della gravidanza alla sua conclusione, età gestazionale al parto, peso del neonato alla nascita, preeclampsia, eventi vascolari materni ed esito avverso grave. Quest’ultimo è un parametro che comprende uno o più eventi che possono comportare, oltre alla morte perinatale, un elevato rischio di deterioramento della qualità della vita per la madre o per il feto. Le pazienti con APS e LES invece presentavano una prognosi peggiore, con un’incidenza simile in termini di: esito della gravidanza, parti a termine, peso alla nascita, crescita fetale rispetto all’età gestazionale ed esito avverso. Rimane quindi il dubbio se la sola positività per gli aPL rappresenti un reale fattore di rischio per perdite fetali e altre complicanze ostetriche

Pregnancy and systemic autoimmune diseases: a monocentric experience over 20 years

Systemic autoimmune diseases (SAD) frequently affect women of child-bearing age, and for these patients pregnancy are considered at high risk of maternal and fetal complications, especially in cases of systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS). Our studies were aimed at describing the course of pregnancy and at identifying predictors of adverse pregnancy outcomes both in terms of obstetric complications and disease flare in patients with undifferentiated connective tissue disease (UCTD), SLE, APS and Sjӧgren syndrome (SS). Pregnancy outcome was significantly better in patients with well controlled disease at the beginning of pregnancy, in particular in cases of disease remission. On the contrary, a residual disease activity at conception (both clinical and serological), might impact on pregnancy outcomes. We have also observed an increased risk of complications in cases of severe organ involvement such as in cases of history of lupus nephritis. In our cohort the second trimester uterine artery Doppler seems to have a good predictive role of adverse pregnancy outcome, while the levels of free β-human chorionic gonadotrophin (hCG) and pregnancy-associated plasma protein A (PAPP-A) detected during first trimester do not seem to be useful to identify patients with SAD at higher risk of complications. A strength of our studies is that they reflect the real-world setting in referral centre for pregnancy care in patients with SAD, and therefore the results of the studies are generalisable to day-to-day practice. Our studies also underline the importance of prenatal planning and closely monitoring during pregnancy and puerperium by an expert multidisciplinary team to ensure a more favourable pregnancy outcome. In conclusion, despite the progress in diagnosis and treatment, pregnancy in patients with SAD are still at risk of maternal and fetal complications also in cases of well controlled disease activity, and further studies are necessary to increase the knowledge on predictors on pregnancy outcome in order to plan further actions aimed at improving the care provided to SAD patients during all the phases around pregnancy planning

One year in review 2020: systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a relapsing-remitting course that can affect various organs or systems, leading to a broad spectrum of clinical manifestations. In the past year, many studies have been published on SLE, providing a significant advancement in disease knowledge and patient management. The aim of this review is to summarise the most relevant scientific contributions on SLE pathogenesis, clinical manifestations and comorbidities, biomarkers and treatment strategies published in 2019

One year in review 2022: systemic lupus erythematosus

: Systemic lupus erythematosus (SLE) is a chronic multisystem auto-immune disease with extremely varied clinical manifestations and a complex pathogenesis. New insights in SLE about pathogenetic pathways, biomarkers, and data on clinical manifestations are progressively emerging, and new drugs and new therapeutic strategies have been proposed to improve the control of disease activity. Thus, this review is aimed to summarise the most relevant data about SLE emerged during 2021, following the previous annual review of this series

Variations of C3 and C4 Before and During Pregnancy in Systemic Lupus Erythematosus: Association With Disease Flares and Obstetric Outcomes

Objective: To analyze complement level variations in systemic lupus erythematosus (SLE) pregnancies, focusing on disease flares and obstetric complications. Methods: SLE pregnancies prospectively followed by multidisciplinary teams from 1987 to 2018 in 2 Italian rheumatology centers were retrospectively analyzed. As reference, pregnancy-modified ranges of normal levels of C3 and C4 were derived from 175 pregnancies from the general obstetric population (GOP), as previously described by our group. Results: Two hundred forty-six pregnancies in 172 patients with SLE were analyzed. Eighty-nine percent were live births. Thirty-five flares were recorded in 30 pregnancies (12.2%) and obstetric complications occurred in 47 pregnancies (19.1%) including 27 pregnancy losses, 11 severely preterm births (2 resulting in perinatal death), and 15 hypertensive disorders. C3 and C4 levels were higher in the GOP than in patients with SLE, at any time point. C3 and C4 levels progressively increased during pregnancy in both GOP and SLE pregnancies without flare and obstetric complications, whereas this physiological increase was not observed in pregnancies with flares or obstetric complications. A significantly higher frequency of low C4 was found in pregnancies with flares (at preconception and in each trimester) and preterm births (at preconception). In multivariate analysis, low C4 at preconception was associated with flares (odds ratio 13.81, 95% CI 3.10-61.43, P < 0.001). Conclusion: Low C4 at preconception was found to be an independent risk factor for SLE flare during pregnancy. Not only C3 and C4 levels but also their variations should be observed, as their failure to increase can be useful to predict risk of complications and suggest closer monitoring

Impact of first wave of SARS-CoV-2 infection in patients with Systemic Lupus Erythematosus: Weighting the risk of infection and flare.

IntroductionThe aim of this study was to investigate the incidence and clinical presentation of SARS-CoV-2 infections in a Systemic Lupus Erythematosus (SLE) cohort; to assess correlations with disease characteristics and rheumatic therapy; and to evaluate the occurrence of treatment discontinuation and its impact on disease activity.Materials and methodsSLE patients monitored by a single Italian centre were interviewed between February and July 2020. Patients were considered to be positive for SARS-CoV-2 infections in case of 1) positive nasopharyngeal swab; 2) positive serology associated with COVID19 suggesting symptoms. The following data were also recorded: clinical symptoms, adoption of social distancing measures, disease activity and treatment discontinuation.Results332 patients were enrolled in the study. Six patients (1.8%) tested positive for SARS-CoV-2 infection, with the incidence being significantly higher in the subgroup of patients treated with biological Disease-Modifying Anti-Rheumatic Drugs (p = 0.005), while no difference was observed for other therapies, age at enrollment, disease duration, type of cumulative organ involvement or adoption of social isolation. The course of the disease was mild. Thirty-six patients (11.1%) discontinued at least part of their therapy during this time period, and 27 (8.1%) cases of disease flare were recorded. Correlation between flare and discontinuation of therapy was statistically significant (pConclusionTreatment discontinuation seems to be an important cause of disease flare. Our findings suggest that abrupt drug withdrawal should be avoided or evaluated with caution on the basis of individual infection risk and comorbidities