Studio clinico e fisiopatologico dei disturbi psichiatrici nel Morbo di Behçet mediante valutazione psicometrica, biochimica, clinica e anatomica cerebrale con risonanza magnetica

Un argomento molto dibattuto attualmente in letteratura è il frequente riscontro di disturbi psichiatrici nei pazienti con MB. Fino ad ora le manifestazioni psico-patologiche sono state attribuite a due fattori principali: il peggioramento della qualità della vita a cui i pazienti vanno incontro, soprattutto a causa del recidivare dell’impegno muco-cutaneo e oculare, e l’interessamento del SNC che spesso esita in lesioni cerebrali organiche permanenti. Tuttavia, nella pratica clinica si osserva che i sintomi psichiatrici possono essere presenti anche al di fuori delle fasi di attività di malattia e in soggetti senza interessamento neurologico. In considerazione della cosiddetta “neurogenesis hypothesis” di Jacobs e Duman, secondo cui una riduzione della neuorgenesi, e quindi del volume, a livello ippocampale sarebbe alla base di alcuni disturbi della sfera affettiva (in particolare della depressione unipolare e del PTSD), nel nostro lavoro si è voluta correlare la fenomenica psicopatologica con la valutazione morfologica dell’anatomia cerebrale mediante risonanza magnetica, in pazienti con MB senza coinvolgimento neurologico. In questo modo, si vuole comprendere la natura fisiopatogenetica dell’impegno psichiatrico in corso di malattia. E’ stata inoltre confrontata la prevalenza dei sintomi psichiatrici in questo gruppo di pazienti con quella di un gruppo di pazienti con diagnosi di Neuro-Behçet e infine si è indagata l’eventuale correlazione delle manifestazioni psichiatriche con l’attività di malattia, il profilo demografico e il quadro bioumorale dei nostri pazienti

Salivary Proteomics Markers for Preclinical Sjögren’s Syndrome: A Pilot Study

Primary Sjögren’s syndrome (pSS) is a complex autoimmune disorder that particularly affects the salivary and lachrymal glands, generally causing a typical dryness of the eyes and of the mouth. The disease encompasses diverse clinical representations and is characterized by B-cell polyclonal activation and autoantibodies production, including anti-Ro/SSA. Recently, it has been suggested that autoantibody profiling may enable researchers to identify susceptible asymptomatic individuals in a pre-disease state. In this pilot study, we used mass spectrometry to analyze and compare the salivary proteomics of patients with established pSS and patients with pre-clinical SS, identifying a common protein signature in their salivary fluid. We found that several inflammatory, immunity-related, and typical acinar proteins (such as MUC5B, PIP, CST4, and lipocalin 1) were differently expressed in pSS and in pre-clinical SSA+ carriers, compared to healthy controls. This suggests that saliva may closely reflect exocrine gland inflammation from the early phases of the disease. This study confirms the value of salivary proteomics for the identification of reliable biomarkers for SS that could be identified, even in a preclinical phase of the disease

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

Skull Base Fungal Osteomyelitis: A Case Report and Review of the Literature

Skull base osteomyelitis (SBO) is an invasive infection refractory to therapy, closely linked with malignant otitis externa (MOE). It is characterized by a mild clinical presentation that can delay cross-sectional imaging considered as the key to revealing it. Skull base osteomyelitis typically affects elderly diabetics and immunocompromised patients (>70 years). It most commonly has an otogenic origin due to an extension of MOE. The prognosis can be very poor without the administration of adequate and timely therapy at an early disease stage. Nowadays, Pseudomonas aeruginosa remains the most common pathogen associated with SBO. Fungi are a rare cause of MOE. This report documents a rare case of otogenic SBO caused by Candida parapsilosis in a diabetic patient, with persistent otologic symptoms as clinical onset and resistance to medical treatment. Fungal MOE has more subtle symptoms and is more aggressive than its bacterial counterpart. When MOE is resistant to antibacterial drugs, this should raise the suspicion of a fungal etiology of MOE. The current guidelines do not exhaustively describe the diagnosis, antifungal drugs of choice, and optimum duration of treatment. The description of these rare clinical cases should help with the multidisciplinary management of this disease in order to optimize the diagnosis and therapeutic protocol

270 The discordance between patient and physician perception of the disease: the paradigm of SLE

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184 Patient perception of SLE burden: the role of disease activity, comorbidities and treatment

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

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Similarities and differences between younger and older disease onset patients with newly diagnosed systemic lupus erythematosus

Objectives: Several studies show that age at onset has an impact on the clinical-serological presentation, comorbidities and disease course of patients with systemic lupus erythematosus (SLE). We evaluated whether, in patients with recent onset SLE, the age at onset correlates with clinical-serological manifestations and with comorbidities. Methods: We analysed 171 patients with a SLE diagnosis obtained within 12 months of diagnosis enrolled in the Early Lupus project. Based on the age of onset of the first disease symptom, they were stratified into 2 groups: early onset (18-45 years) and late onset (>45 years). The analysis was replicated by stratifying patients based on age at diagnosis (fulfillment of ACR classification criteria). Each comparison was made at baseline and at 36 months of follow-up. Results: Baseline: patients with late onset displayed comorbidities (hypertension, dyslipidemia and osteoporosis) more frequently than early onset group. 11.4% of late onset patients had a malignancy in medical history, not recorded in the early onset cohort. The two groups differed neither in organ involvement (domain BILAG) nor in disease activity (ECLAM). Patients with early onset showed a disease with signs of higher serologic activity (higher frequency of anti-dsDNA positivity and lower mean C3 and C4 levels) and had malar rash more frequently than the late onset group (36.2% vs. 18.2%, p=0.042). Similar results were obtained by stratifying patients by age of diagnosis (18-45 years and >45 years), except for the higher frequency of discoid rash in the group with age at diagnosis >45 years (18% vs. 6.6%, p=0.045). 36 months: the 2 groups of patients independently of the stratification applied did not differ in the accumulation of damage, but showed a different pattern of 8 organ involvement. Musculoskeletal involvement was more frequent both in the late onset group (18.6% vs. 7.3%, p=0.043) and in the group with age at diagnosis >45 years (20.4% vs. 5.9%, p=0.009) compared to their counterparts, while renal involvement was more frequent in the group with age at diagnosis 18-45 years (21.4% vs. 6.1%, p=0.03).A sub analysis at 36 months on patients without hypertension and osteoporosis at enrollment showed that patients with older age at onset had a higher frequency of these comorbidities, compared to their counterparts. Conclusions: In our cohort, younger disease SLE onset seems to correlate with a more active immunological profile, while late onset with a higher incidence of comorbidities

2023 EULAR recommendations for the management of fatigue in people with inflammatory rheumatic and musculoskeletal diseases

Objectives: Fatigue is prevalent in people with inflammatory rheumatic and musculoskeletal diseases (I-RMDs) and recognised as one of the most challenging symptoms to manage. The existence of multiple factors associated with driving and maintaining fatigue, and the evidence about what improves fatigue has led to a multifaceted approach to its management. However, there are no recommendations for fatigue management in people with I-RMDs. This lack of guidance is challenging for those living with fatigue and health professionals delivering clinical care. Therefore, our aim was to develop EULAR recommendations for the management of fatigue in people with I-RMDs.Methods: A multidisciplinary taskforce comprising 26 members from 14 European countries was convened, and two systematic reviews were conducted. The taskforce developed the recommendations based on the systematic review of evidence supplemented with taskforce members’ experience of fatigue in I-RMDs.Results: Four overarching principles (OAPs) and four recommendations were developed. OAPs include health professionals’ awareness that fatigue encompasses multiple biological, psychological and social factors which should inform clinical care. Fatigue should be monitored and assessed, and people with I-RMDs should be offered management options. Recommendations include offering tailored physical activity and/or tailored psychoeducational interventions and/or, if clinically indicated, immunomodulatory treatment initiation or change. Patient-centred fatigue management should consider the individual’s needs and preferences, their clinical disease activity, comorbidities and other psychosocial and contextual factors through shared decision-making.Conclusions: These 2023 EULAR recommendations provide consensus and up-to-date guidance on fatigue management in people with I-RMDs

Integrating Patient Reported Outcomes, clinical data and quality indicators to physician driven data in clinical management of chronic rheumatic diseases: the paradigm of Systemic Lupus Erythematosus (SLE)

A discordance exists between patients and physicians in the assessment of Systemic Lupus Erythematosus (SLE). A more patient-oriented research to address the multifaceted dimensions of the disease burden is warranted. The aim of this study was to investigate patients’ needs and expectations about their medical care, with the objective of integrating patient-reported data to the traditional physician evaluation towards a new shared strategy for the monitoring of SLE. The first step of the project was a systematic literature review of existing clinical and non-clinical indicators for the management of SLE and of currently available SLE-dedicated IT tools (Apps) designed to involve the patient in the gathering of clinical and quality of life data about their health. On the basis of the results of the literature review, ad-hoc on-line surveys have been designed, targeting European experts in the field and SLE patients. The aim was to investigate both the real use of such indicators in clinical practice and to identify needs and expectations experienced by doctors and patients in management of the disease. Finally, the survey results have been discussed in dedicated focus groups of patients, clinicians but also general practitioners, nurses and one caregiver. According to the project results, the key elements for the development of a new paradigm for SLE management should include: • MULTIDISCIPLINARITY and NETWORKING • PATIENT-PHYSICIAN COMMUNICATION • PATIENT’S EDUCATION • PATIENT’S EMPOWERMENT First of all, the ideal setting for the management of the disease seems to be represented by a dedicated “Lupus clinic”, where the patient can be in charge of a team of expert health care professionals. The networking with multi-disciplinary specialists should be encouraged. From both the patients’ and clinicians’ perspective emerged that the communication between the parts is often inadequate in terms of both contents and means. SLE patients declared that they have a real, shared hunger for information about the disease and, on the other hand, clinicians think that only an educated patient can be really empowered. Some instruments may be of help to make such an educational process to happen. Both clinicians and patients agreed on the fact that an ideal IT solution would be of benefit in the management of the disease. Self-management of simple tasks is important as one of the first steps towards patient’s engagement and empowerment. In conclusion, several unmet needs seem to be present in the management of SLE, both from the patient’s and the clinician’s point of view. In this setting, the results of the project pointed out a path to follow in the management of Lupus patients and lay the foundations for a new shared strategy for disease management in clinical practice which may serve as a prototype for other chronic diseases