Explore the vast range of titles available.

The aim of this Research Topic is to describe the state of the art in AAV prognostic tools, treatment goals, and potential biomarker use. Treatment goals often depend on assessing disease activity, for which tools are still limited. [...]there is a growing need for adequate and validated candidate biomarkers. [...]this Research Topic collects studies highlighting the current state of the art in the field of AAVs, together with original manuscripts identifying both treatment options and new ways of clustering AAVs.

ObjectiveTo evaluate the effectiveness and safety of secukinumab in patients with a mucosal and articular Behçet’s phenotype resistant to conventional and biologic treatment.MethodsA multicentre retrospective study was performed on 15 patients with a mucosal and articular phenotype of Behçet’s syndrome fulfilling the International Criteria for Behçet’s Disease and refractory to treatment with colchicine, disease-modifying antirheumatic drugs and at least one antitumour necrosis factor-α agent. Minimum follow-up was set at 6 months. Six patients with a polyarticular involvement were treated with secukinumab 300 mg/month, while all other cases received secukinumab 150 mg/month. Dose increase from 150 to 300 mg per month and shortening of administration frequency were allowed for poor disease control. Response evaluation was based on the number of oral ulcers in the previous 28 days and Disease Activity Score-28 for articular manifestations.ResultsAt 3 months of follow-up, nine (66.7%) patients achieved a response (complete or partial), and this proportion further increased to 86.7% at 6 months, 76.9% at 12 months, 90.0% at 18 months and 100.0% after 24 months. Notably, all patients who started with secukinumab 300 mg/month achieved complete response by month 6. Seven (46.7%) patients could achieve a response only after switching to a higher dosage.ConclusionsOur study suggests that secukinumab at a dose of 150 and 300 mg per month is safe and effective for the long-term treatment of patients with Behçet’s syndrome with a mucosal and articular phenotype refractory to previous treatments. Notably, secukinumab 300 mg/month resulted in superior complete mucosal and articular responses with no serious or dose-related adverse effects.

A total of 20%–50% of the cases are immunoglobulin G4 (IgG4)-related, based on histological evidence of IgG4+ plasma cell infiltration (on a background of dense lymphoplasmacytic infiltrates, storiform fibrosis and tissue eosinophilia) and/or increased serum IgG4.1 Glucocorticoids are the first-line therapy for CP.2 However, some patients are refractory, frequently relapsing or have contraindications to glucocorticoids. The anti-CD20 monoclonal antibody rituximab proved efficacious in systemic forms of IgG4-related disease (IgG4-RD) including IgG4-related CP,3 but data on IgG4-unrelated CP are scarce.4–6 In this study, we tested rituximab in CP patients without evidence of IgG4-RD who had relapsing/refractory disease or contraindications to standard-dose glucocorticoids. At month 6, all patients were symptom-free; erythrocyte sedimentation rate (ESR) (figure 1) and C-reactive protein (CRP) dropped (respectively, p<0.0001 and p=0.01, vs baseline).

In a recent report, one patient with periaortitis secondary to IgG4-related systemic disease was successfully treated with rituximab, although the response of the retroperitoneal mass was not described. 8 Rituximab is increasingly used in different autoimmune disorders such as rheumatoid arthritis, systemic lupus erythematosus and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. 9 CP is also thought to have an autoimmune background, given its association with autoimmune diseases 6 10 and with the autoimmunity-predisposing HLA-DRB1*03 allele. 11 The efficacy of rituximab also suggests a crucial pathogenetic role for B cells in CP. [...]rituximab may be effective in CP.

Inferential statistical methods failed in identifying reliable biomarkers and risk factors for relapsing giant cell arteritis (GCA) after glucocorticoids (GCs) tapering. A ML approach allows to handle complex non-linear relationships between patient attributes that are hard to model with traditional statistical methods, merging them to output a forecast or a probability for a given outcome. The objective of the study was to assess whether ML algorithms can predict GCA relapse after GCs tapering. GCA patients who underwent GCs therapy and regular follow-up visits for at least 12 months, were retrospectively analyzed and used for implementing 3 ML algorithms, namely, Logistic Regression (LR), Decision Tree (DT), and Random Forest (RF). The outcome of interest was disease relapse within 3 months during GCs tapering. After a ML variable selection method, based on a XGBoost wrapper, an attribute core set was used to train and test each algorithm using 5-fold cross-validation. The performance of each algorithm in both phases was assessed in terms of accuracy and area under receiver operating characteristic curve (AUROC). The dataset consisted of 107 GCA patients (73 women, 68.2%) with mean age ( ± SD) 74.1 ( ± 8.5) years at presentation. GCA flare occurred in 40/107 patients (37.4%) within 3 months after GCs tapering. As a result of ML wrapper, the attribute core set with the least number of variables used for algorithm training included presence/absence of diabetes mellitus and concomitant polymyalgia rheumatica as well as erythrocyte sedimentation rate level at GCs baseline. RF showed the best performance, being significantly superior to other algorithms in accuracy (RF 71.4% vs LR 70.4% vs DT 62.9%). Consistently, RF precision (72.1%) was significantly greater than those of LR (62.6%) and DT (50.8%). Conversely, LR was superior to RF and DT in recall (RF 60% vs LR 62.5% vs DT 47.5%). Moreover, RF AUROC (0.76) was more significant compared to LR (0.73) and DT (0.65). RF algorithm can predict GCA relapse after GCs tapering with sufficient accuracy. To date, this is one of the most accurate predictive modelings for such outcome. This ML method represents a reproducible tool, capable of supporting clinicians in GCA patient management.

Another condition that has limited information during pregnancy is undifferentiated connective tissue disease (UCTD).Serena et al.review the most recent literature and raise some concerns, such as the possibility of severe disease and progression to definite SAD. Notably, SAD can also be associated with gynecological complications outside the pregnancy period.Orlandi et al., within the frame of a multidisciplinary collaboration between rheumatologists and gynecologists, investigate menstruation-related disorders and their impact on the quality of life of women with rheumatic diseases using a self-administered questionnaire. Conflict of interest The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Objective. To date, there is no shared national guideline in Italy for the management of reproductive health in rheumatic diseases (RHRD). The Italian Society for Rheumatology (SIR) has committed to developing clinical practice recommendations to provide guidance on both management and treatment regarding RHRD in Italy. Methods. Using the GRADE-ADOLOPMENT methodology, a systematic literature review was conducted to update the scientific evidence that emerged after the publication of the reference recommendations from the American College of Rheumatology. A multidisciplinary group of 18 clinicians with specialist experience in rheumatology, allergy and clinical immunology, internal medicine, nephrology, gynecology and obstetrics, and neonatology, a professional nurse, a clinical psychologist, and a representative from the National Association of Rheumatic Patients discussed the recommendations in collaboration with the evidence review working group. Subsequently, a group of stakeholders was consulted to examine and externally evaluate the developed recommendations. Results. Recommendations were formulated for each area of interest: contraception, assisted reproductive technology, preconception counseling, and use of drugs before, during, and after pregnancy and during breastfeeding, considering both paternal and maternal exposure. Conclusions. The new SIR recommendations provide the rheumatology community with a practical guide based on updated scientific evidence for the management of RHRD.

We evaluated the clinical features and retinal and disk perfusion characteristics by using optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) in a subset of giant cell arteritis (GCA) patients who manifested anterior ischemic optic neuropathy (AION), in a subset of GCA patients without ocular involvement, and in a control group composed of healthy controls. We performed an observational study on the eyes of GCA patients affected by arteritic AION both in acute and chronic phases, unaffected eyes of AION, eyes of GCA patients without ocular involvement, and in a control group of healthy eyes of healthy individuals. All patients underwent a complete ophthalmic examination and an OCT and OCTA of the macula and the disk. The study evaluated 10 eyes of GCA patients with AION (AION group), 8 unaffected eyes of GCA patients with AION in another eye (unaffected eyes of AION group), 16 eyes of GCA patients without ocular involvement (non-ocular group), and 22 eyes of healthy patients (healthy group). The ganglion cell complex (GCC) superior and inferior thicknesses were significantly lower in the AION group compared to the unaffected eyes of the AION group (  = 0.045 and  = 0.034, respectively). All OCTA vascular density parameters of the optic disk analyzed in this study (optic nerve head (ONH) whole, superior, inferior, radial peripapillary capillary plexus (RPCP) whole, superior, inferior, lamina cribrosa (LC) whole, superior, inferior) resulted significantly lower in the AION group compared to the unaffected eyes group (  < 0.05 for all the comparisons). The ONH whole and inferior were statistically higher in the healthy group in comparison to the group of GCA patients without ocular involvement (  = 0.008 and  = 0.006, respectively). The ONH inferior was also statistically higher in the unaffected eyes of the AION group in comparison to the non-ocular group (  = 0.045). Regarding the OCTA macular vessel density parameters, the superficial capillary plexus (SCP), whole and inner, were statistically lower in the AION group compared with the unaffected eyes of the AION group. We found a profound vascular impairment in eyes affected by AION and areas of hypoperfusion in the eyes of patients with GCA without ocular involvement, good BCVA, and no clinically significant features. We hypothesized that these areas of lower vessel density might represent areas of subclinical hypoperfusion that cannot be detected ophthalmoscopically.

ObjectiveTo describe pregnancy outcomes in patients with SLE treated with belimumab before and/or during pregnancy.MethodsData of prospectively-followed pregnancies (2014–2022) in 7 Italian centers were retrospectively collected.ResultsTwenty-four SLE pregnancies were included (median age at conception: 33 [21–37] years; 15 primigravidae).Belimumab was stopped in 4 cases preconceptionally, in 10 at positive pregnancy test and in 10 during pregnancy (4 during the 1st trimester, 3 during the 2nd trimester and 3 during the 3rd trimester). The timing of discontinuation was planned with the patient during preconception counselling.Other medications includedprednisone (92%); antimalarials (83%); azathioprine (46%); calcineurin-inhibitors (25%); low-dose aspirin (88%); heparin (58%).At preconception, median SLEDAI was 4(2–4).One patient who discontinued belimumab at the 11th week had active nephritis from preconception.Three flares (cutaneous; pericarditis; hematologic) occurred during the 3rd trimester in the group of patients who discontinued belimumab at positive pregnancy test, while 1 flare (cutaneous + articular) occurred in the 1st trimester in the group of patients who continued belimumab.Live-birth rate was 87.5%. Two miscarriages and 1 intrauterine fetal death (37th week; fetus with 21-trisomy and atrio-ventricular defect) occurred. One perinatal death occurred (patient with thrombotic+obstetric APS and lupus nephritis who underwent heterologous assisted reproductive technology -embryodonation- and developed eclampsia with cerebral haemorrage at 25th week; an urgent cesarean section was performed; the newborn died after 3 days).Two cases of pre-eclampsia in patients with multiple risk factors were observed.Five newborns were hospitalized in Intensive Care Unit for: milk protein intolerance; desaturation; respiratory distress; prematurity (2 cases). One sepsis starting from urinary tract infection occurred in a 2-months-old infant with calico-pyelic and ureteral dilatation at birth. One newborn presented with interatrial defect and situs inversus (paternal 10 chromosome inversion).ConclusionsDespite our data do not allow definitive conclusions, the live birth rate and the exclusion of drug-related congenital defects are encouraging. SLE flares occurred more frequently after Belimumab discontinuation at positive pregnancy test. We suggest that women on good disease control while on belimumab could be offered to continue it and to discuss discontinuation timing according to their specific risk/benefit ratio.Acknowledgements‘Gender Medicine’ Study Group of the Italian Society for Rheumatology