Publications by authors named "Crisafulli F"
Article Synopsis
- * Pregnancy management must be personalized to reflect individual disease features and treatment options to reduce risks for both mother and fetus.
- * The review highlights the importance of discussing fertility, assisted reproductive techniques, and contraception alongside the management of these inflammatory conditions.
Objective: This review aims to summarize the most recent and updated data on pregnancy in patients with axial spondyloarthritis (axSpA), focusing on the recurrence of pregnancy-related complications, the disease activity throughout gestation and the postpartum, and the latest indications for the treatments of future mothers.
Methods: We have conducted a narrative review with an online literature search on Medline and PubMed. We selected only studies written in English published until January 2024, including observational and retrospective studies, meta-analyses, and systematic reviews.
Purpose: The clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Behçet's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown.
Methods: Data were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time.
Article Synopsis
- The study examined pregnancy outcomes in women with autoimmune rheumatic diseases (ARD) enrolled in the Italian P-RHEUM.it cohort from 2018 to 2023, focusing on maternal and infant health.
- Results showed that out of 866 pregnancies, 15.6% experienced maternal disease flares, 30.1% had obstetrical complications, and 91.7% resulted in live births, with a relatively low rate of perinatal deaths and complications.
- The research concluded that effective preconception counseling and proper medication management during pregnancy may help reduce disease-related risks, suggesting that outcomes for women with ARD were comparable to those in the general obstetric population.
Article Synopsis
- - VEXAS syndrome is a new autoinflammatory disease that often affects various organs, with noticeable inflammatory issues in the eyes and orbits.
- - In a study of 59 VEXAS patients, 45.8% exhibited orbital/ocular problems, with periorbital edema and episcleritis being the most common conditions observed.
- - There is a significant link between relapsing polychondritis and eye involvement in VEXAS, and patients with eye issues showed higher mortality rates, indicating the need for closer monitoring by healthcare providers.
Pregnancy in antiphospholipid syndrome: what should a rheumatologist know?
Rheumatology (Oxford)
February 2024
This review focuses on the management of reproductive issues in women who have antiphospholipid syndrome (APS) or are carriers of antiphospholipid antibodies (aPL). The importance of aPL detection during preconception counselling relies on their pathogenic potential for placental insufficiency and related obstetric complications. The risk of adverse pregnancy outcomes can be minimized by individualized risk stratification and tailored treatment aimed at preventing placental insufficiency.
View Article and Find Full Text PDFIntroduction: The effectiveness of canakinumab may change according to the different times it is used after Still's disease onset. This study aimed to investigate whether canakinumab (CAN) shows differences in short- and long-term therapeutic outcomes, according to its use as different lines of biologic treatment.
Methods: Patients included in this study were retrospectively enrolled from the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Still's disease.
Article Synopsis
- VEXAS is a unique disease that combines symptoms of rheumatologic and hematologic disorders, and this study aimed to better understand its diagnosis and genetic features while tracking changes over time with different treatments.
- Researchers gathered data from various centers in Italy, finding that 41 male patients had significant mutations in the UBA1 gene, mostly diagnosed around age 67, all presenting with anemia and common rheumatologic issues like polychondritis.
- A high percentage of these patients also had myelodysplastic syndrome (MDS), showcasing diverse genetic mutations, and the study noted that after treatment like hematopoietic cell transplants, some mutations were cleared.
Article Synopsis
- The study explored the role of complement levels (C3 and C4) as potential biomarkers for monitoring disease activity and risks during pregnancies in women with systemic lupus erythematosus (SLE).
- Data from 532 lupus patients showed that complement levels generally rise during pregnancy but are notably lower in those with prior lupus nephritis and flares, particularly in the first trimester.
- Lower or minimal increases in C3 and C4 levels during early pregnancy were linked to higher rates of complications and gestational flares, suggesting these complement levels could help predict risks for SLE patients.
Behçet's disease (BD) is a heterogeneous multifactorial autoinflammatory disease characterized by a plethora of clinical manifestations. Cutaneous lesions are considered hallmarks of the disease. However, their evolution over time and a thorough description are scarcely reported in non-endemic regions.
View Article and Find Full Text PDFIntroduction: This paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behçet's disease (BD).
Methods: The project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behçet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry.
Article Synopsis
- The study examined how complement levels (C3 and C4) change during pregnancies in women with systemic lupus erythematosus (SLE) and how these changes relate to disease flare-ups and complications.
- A total of 246 pregnancies in 172 SLE patients were analyzed, revealing that low C4 levels at preconception were linked to higher risks of disease flares and obstetric issues, with 89% resulting in live births.
- The findings suggest that monitoring C3 and C4 levels, especially their changes during pregnancy, is important for predicting complications and ensuring better care for SLE patients.
The Autoinflammatory Diseases Alliance Registry of monogenic autoinflammatory diseases.
Front Med (Lausanne)
September 2022
Objective: The present manuscript aims to describe an international, electronic-based, user-friendly and interoperable patient registry for monogenic autoinflammatory diseases (mAIDs), developed in the contest of the Autoinflammatory Diseases Alliance (AIDA) Network.
Methods: This is an electronic platform, based on the Research Electronic Data Capture (REDCap) tool, used for real-world data collection of demographics, clinical, laboratory, instrumental and socioeconomic data of mAIDs patients. The instrument has flexibility, may change over time based on new scientific acquisitions, and communicate potentially with other similar registries; security, data quality and data governance are corner stones of the platform.
Women with Rheumatoid Arthritis (RA) can experience flares during pregnancy that might influence pregnancy outcomes. We aimed at assessing the disease course during pregnancy and identifying risk factors for flares. Data about prospectively-followed pregnancies in RA were retrospectively collected before conception, during each trimester and in the post-partum period.
View Article and Find Full Text PDFObjective: The present paper describes the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry specifically dedicated to patients with Schnitzler's syndrome.
Methods: This is a clinical physician-driven, population- and electronic-based registry implemented for the retrospective and prospective collection of real-life data from patients with Schnitzler's syndrome; the registry is based on the Research Electronic Data Capture (REDCap) tool, which is designed to collect standardized information for clinical research, and has been realized to change over time according to future scientific acquisitions and potentially communicate with other existing or future similar registries.
Results: Since its launch, 113 centers from 23 countries in 4 continents have been involved.
Objective: The aim of this paper is to present the AutoInflammatory Disease Alliance (AIDA) international Registry dedicated to Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome, describing its design, construction, and modalities of dissemination.
Methods: This Registry is a clinical, physician-driven, population- and electronic-based instrument designed for the retrospective and prospective collection of real-life data. Data gathering is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain real-world evidence for daily patients' management.
Article Synopsis
- This study investigates whether low-dose acetylsalicylic acid (LDASA) should be given to pregnant patients with Systemic Lupus Erythematosus (SLE) who have no history of kidney issues or positive antiphospholipid antibodies.
- Researchers analyzed 216 pregnancies from 187 patients, comparing outcomes between those treated with LDASA and those who were not; they found no significant differences in adverse pregnancy outcomes (APOs) between the two groups.
- While LDASA treatment did show a lower incidence of pre-eclampsia (2.4% vs 8.3%), the overall conclusion is that LDASA does not significantly reduce severe obstetric complications,
Managing puerperium in patients with systemic autoimmune diseases: an update.
Expert Rev Clin Immunol
April 2022
Introduction: Puerperium is a critical period for patients affected by autoimmune rheumatic diseases for the risk of disease's flares and difficulties in treating lactating mothers. We want to summarize the literature data about psychological and pharmacological management of these patients and possible risk factors of disease's flares.
Areas Covered: We made a narrative review on recent studies about puerperium in rheumatic autoimmune diseases patients.
Article Synopsis
- Schnitzler's syndrome is a rare autoinflammatory disease, and while IL-1 inhibitors show promise, there is limited data on their long-term efficacy and safety.
- A retrospective study analyzed 20 patients treated with IL-1 inhibitors across 9 Italian centers, revealing a 73.4% retention rate at one year and an overall successful treatment continuation for all patients by the last follow-up.
- The data suggests that patients diagnosed definitively using the Strasbourg criteria had better retention rates, supported by a noticeable reduction in the need for conventional drugs and a decrease in serum amyloid A levels.
Reproductive Issues and Pregnancy Implications in Systemic Sclerosis.
Clin Rev Allergy Immunol
June 2023
Systemic sclerosis (SSc) is a rare systemic autoimmune disease that can influence reproductive health. SSc has a strong female predominance, and the disease onset can occur during fertility age in almost 50% of patients. Preconception counseling, adjustment of treatment, and close surveillance during pregnancy by a multidisciplinary team, are key points to minimize fetal and maternal risks and favor successful pregnancy outcomes.
View Article and Find Full Text PDFArticle Synopsis
- Antiphospholipid antibodies (aPL) can lead to fetal loss, and while some studies suggest low complement levels (C3/C4) may be linked to pregnancy complications, the results aren't universally agreed upon.
- A multicenter study involving 503 pregnancies examined the relationship between preconception complement levels and adverse pregnancy outcomes (APO) in women with antiphospholipid syndrome (APS) and aPL carriers.
- The study found that low C3/C4 levels before pregnancy were significantly associated with higher rates of pregnancy loss, particularly in women who tested positive for triple aPL, indicating that measuring complement levels could help identify those at risk for pregnancy complications.
Article Synopsis
- - The study aimed to find out if being in disease remission or a low disease activity state at the start of pregnancy improves outcomes for patients with Systemic Lupus Erythematosus (SLE).
- - Researchers monitored 347 pregnancies in SLE patients, discovering that a significant portion were in remission at the beginning, and those in remission or on hydroxychloroquine (HCQ) had lower rates of disease flare and adverse pregnancy outcomes (APOs).
- - The findings suggest that careful prenatal planning focused on achieving disease remission can significantly lower the risk of complications during pregnancy for SLE patients.
The long-term outcome of children born to SLE mothers still represents a controversial topic in literature, with some studies reporting a possible increased prevalence of different neurologic and psychiatric diseases (NPD), including neurodevelopmental disorders (ND), and in particular learning disorders (LD). Different risk factors have been advocated, such as the exposure to auto-antibodies and drugs, particularly Azathioprine (AZA). A case-control study was designed to compare pregnancies treated with AZA (cases) with those not treated with AZA (controls).
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