696 research outputs found

    Severe neuropsychiatric systemic lupus erythematosus successfully treated with rituximab: An alternative to standard of care

    No full text
    Demyelinating syndrome secondary to systemic lupus erythematosus (DS-SLE) is a rare encephalomyelitis burden with a high risk of disability and death. We report on a 49-year-old Caucasian woman with systemic lupus erythematosus (SLE) complicated by severe cognitive dysfunction, brainstem disease, cranial nerve palsies, weakness and numbness in limbs and multiple discrete magnetic resonance imaging (MRI) areas of damage within the white matter of semioval centers, temporal lobe, external capsule, claustrum, subinsular regions and midbrain. She also had multiple mononeuritis diagnosed through sensory and motor nerve conduction study. She was diagnosed with severe DS-SLE prominently involving the brain and was treated with 500 mg methylprednisolone (PRE) pulses for 3 consecutive days, followed by one single pulse of 500 mg cyclophosphamide, and 1 g rituximab, which was then repeated 14 days later. PRE 25 mg/day, rapidly tapered to 7.5 mg/day in 6 months, and mycophenolate mofetil 1 g/day were prescribed as maintenance therapy. She had progressive and sustained improvement in neurological symptoms with almost complete resolution of brain MRI lesions after 1 year. B-cell depleting therapy could be considered as a possible alternative to standard of care in the management of severe inflammatory neuropsychiatric SLE but it should be associated with a conventional immunosuppressant as maintenance treatment to reduce the risk of flare and reduce corticosteroids dose

    Correction to: Size‐Dependent Enforcement, Tax Evasion and Dimensional Trap

    No full text
    The article “Size‐Dependent Enforcement, Tax Evasion and Dimensional Trap”, written by Raffaella Coppier, Elisabetta Michetti and Luisa Scaccia, was originally published electronically on the publisher’s internet portal on 05 July 2023 without open access. With the author(s)’ decision to opt for Open Choice the copyright of the article changed on 24 February 2024 to © The Author(s) 2024 and the article is forthwith distributed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made

    Demyelinating syndrome in SLE: review of different disease subtypes and report of a case series

    No full text
    Demyelinating syndrome (DS) is a rare manifestation of systemic lupus erythematosus (SLE) (1%) with high clinical heterogeneity and potentially severe prognosis. It can represent a diagnostic and therapeutic challenge for clinicians. A recent study described 5 different patterns of demyelinating disease presentation, characterised by specific clinical, laboratory and brain and spine magnetic resonance imaging abnormalities: 1) neuromyelitis optica; 2) neuromyelitis optica spectrum disorders; 3) DS prevalently involving the brain; 4) DS prevalently involving the brainstem; 5) clinically isolated syndrome. In this review we briefly discuss typical characteristics of each DS presentation in SLE and we describe 5 illustrative clinical cases, one for each subset of DS, considering both diagnostic and therapeutic options

    L’azione diretta nella responsabilità sanitaria all’alba del regolamento attuativo della legge Gelli-Bianco

    No full text
    Nel saggio, dedicato all’azione diretta nella responsabilità sanitaria, prevista dalla l. 24/2017, la figura esaminata viene ricondotta al paradigma generale dell’azione diretta nella responsabilità da circolazione di autoveicoli pur evidenziandosi la presenza anche di alcune differenze derivanti dagli specifici caratteri del settore medico-sanitario oggetto della nuova disciplina. Alla disamina delle questioni di natura sostanziale si accompagna l’analisi delle problematiche processuali evidenziandosi, per entrambe, le novità introdotte dalla l. 24/2017. Completa il lavoro la critica disamina dello schema del regolamento attuativo della legge Gelli-Bianco, in corso di approvazione, introduttivo dei requisiti minimi delle polizze assicurative obbligatorie previste ex lege e del relativo Fondo rischi nonché delle eccezioni opponibili che l’assicurazione può opporre al danneggiato

    Treatment Target in Newly Diagnosed Systemic Lupus Erythematosus: The Association of Lupus Low Disease Activity State and Remission With Lower Accrual of Early Damage

    No full text
    Objectives: To compare the effect of achievement and maintenance of lupus low disease activity state (LLDAS) and clinical remission (CR) in preventing early damage accrual in patients with systemic lupus erythematosus (SLE). Methods: In a monocentric cohort of 116 newly diagnosed SLE patients, LLDAS and CR achievement at 6 months (T1) after treatment initiation and their maintenance over the next 12 months were assessed. Early damage was recorded after 18 months of follow-up (T2) using the SLICC/damage index. Uni- and multivariate analysis were performed to evaluate the association of LLDAS and CR with early damage. Results: LLDAS was significantly more attained than CR both at T1 (42.2% vs. 21.6% of patients, p<0.001) and T2 (46.6% vs. 31.9%, p=0.022). Overlap rate between persistent LLDAS and persistent CR was 41.7% (n=15). On multivariate analysis, achievement of CR (OR 0.07, 95%CI 0.01 to 0.59, p=0.015) and LLDAS (OR 0.25, 95%CI 0.06 to 0.99, p=0.049) at T1 were independently associated with lower accrual of early damage. Patients who achieved LLDAS (including CR) at T1 and steadily persisted in this condition until T2 developed significantly less damage compared to those who failed to maintain it during the T1-T2 interval (p=0.003), those who achieved it later than T1 (p<0.001) or those who had never been in this condition (p<0.001). Conclusions: Although CR is recommended as the primary treatment target in SLE, LLDAS represents a valid alternative in the early stage of SLE management. LLDAS and CR maintenance should be targeted to prevent damage

    Failure to achieve lupus low disease activity state (LLDAS) six months after diagnosis is associated with early damage accrual in Caucasian patients with systemic lupus erythematosus

    No full text
    Background: The aim was to assess the attainability and outcome of the lupus low disease activity state (LLDAS) in the early stages of systemic lupus erythematosus (SLE). Methods: LLDAS prevalence was evaluated at 6 (T1) and 18 (T2) months after diagnosis and treatment initiation (T0) in a monocentric cohort of 107 (median disease duration 9.7 months) prospectively followed Caucasian patients with SLE. Reasons for failure to achieve LLDAS were also investigated. Multivariate models were built to identify factors associated with lack of LLDAS achievement and to investigate the relationship between LLDAS and Systemic Lupus International Collaboration Clinics (SLICC)/Damage Index (SDI) accrual. Results: There were 47 (43.9%) patients in LLDAS at T1 and 48 (44.9%) at T2. The most frequent unmet LLDAS criterion was prednisolone dose >7.5 mg/day (83% of patients with no LLDAS at T1). Disease manifestations with the lowest remission rate during follow up were increased anti-double-stranded DNA (persistently present in 85.7% and 67.5% of cases at T1 and T2, respectively), low serum complement fractions (73.2% and 66.3%) and renal abnormalities (46.4% and 28.6%). Renal involvement at T0 was significantly associated with failure to achieve LLDAS both at T1 (OR 7.8, 95% CI 1.4-43.4; p = 0.019) and T2 (OR 3.9, 95% CI 1.4-10.6; p = 0.008). Presence of any organ damage (SDI â ¥1) at T2 was significantly associated with lack of LLDAS at T1 (OR 5.0, 95% CI 1.5-16.6; p = 0.009) and older age at diagnosis (OR 1.05 per year, 95% CI 1.01-1.09; p = 0.020). Conclusion: LLDAS is a promising treatment target in the early stages of SLE, being attainable and negatively associated with damage accrual, but it fit poorly to patients with renal involvement

    Demyelinating syndrome in SLE encompasses different subtypes: Do we need new classification criteria? Pooled results from systematic literature review and monocentric cohort analysis

    No full text
    Objective To describe features of demyelinating syndrome (DS) in systemic lupus erythematosus (SLE). Methods A systematic review using a combination of Mesh terms in PubMed and a retrospective analysis of 343 adult patients with SLE were carried out to identify patients with DS. Retrieved cases were classified as affected with DS according to 1999 ACR nomenclature and attributed to SLE by applying the 2015 algorithm. DS defined according to the clinical but not temporal 1999 ACR criteria was classified as clinically isolated syndrome (CIS). Results Estimated prevalence of DS (including CIS) in the SLE cohort was 1.3% and incidence rate was 1.5 cases per 1000 patient-years. Overall, 100 cases from literature review and 4 from SLE cohort were identified and are presented as a whole: 49 (47.1%) were classified as neuromyelitis optica spectrum disorders (NMOSD), 29 (27.9%) as CIS, 14 (13.5%) as NMO, 7 (6.7%) as DS prominently involving the brainstem and 5 (4.8%) as DS prominently involving the brain. DS was the SLE onset manifestation in 41 (39.4%) patients. Longitudinally extensive transverse myelitis was the most frequent manifestations being present in 73 (70.2%) patients (37 NMOSD, 21 CIS, 14 NMO, 1 DSB). Methylprednisolone (79.8%) and cyclophosphamide (55.8%) pulses, but also plasma-exchange (16.3%) and rituximab (7.6%) in relapsing-refractory cases, were mostly prescribed. Complete recovery rate ranged between 62% in CIS to 7% in NMO. Conclusion DS in SLE is rare (1%) and encompasses different subtypes including CIS. Timely diagnosis and early treatment are recommended to minimize complications
    corecore