Parc Taulí Hospital Universitari

Most patients with BRAF-mutant melanoma eventually develop resistance to BRAF/MEK inhibitors (BRAF/MEKi) and immune-checkpoint blockade. Emerging evidence from retrospective cohorts indicates promising activity from re-exposure to BRAF/MEKi after a treatment-free interval of targeted therapy (TT), probably due to tumor regression and proliferation of sensitive clones. However, there is limited prospective evidence on this approach. GEM1801 is a prospective observational study by the Spanish Melanoma Group (GEM), including 1123 patients treated at 37 centers. We conducted a descriptive analysis of baseline characteristics, objective response rate (ORR), progression-free survival (PFS), and overall survival (OS) for the 24 patients who received a second course of BRAF/MEKi, either as retreatment (after relapse following prior adjuvant TT) or rechallenge (following prior progression on TT in the metastatic setting). Five patients underwent retreatment, and 19 received rechallenge. The BRAF/MEKi median free interval for retreatment was 20.9months (range 12.4-57.3), with an ORR of 20%, and median PFS and OS of 5.5 and 8.4months, respectively. The median free interval of TT for rechallenge was 6.8months, with an ORR of 31.6%, and median PFS and OS of 5.7 and 7.6months. Patients with ECOG ≤ 1 experienced longer survival with rechallenge (8.2 vs 4.3months; HR 9.07 [95% CI 1.37-59.8]; p = 0.022). Retreatment or rechallenge with BRAF/MEKi in the metastatic setting has shown clinical activity in patients with metastatic melanoma who lack therapeutic alternatives to prolong survival. Therefore, in our experience, it may represent a valid therapeutic strategy for selected patients. GOV: NCT03605771 (REGISTRATION DATE: 20-07-2018): NCT03605771.

Objective: To compare the efficacy of ultrasound-guided alcohol sclerotherapy versus laparoscopic cystectomy for the management of ovarian endometriomas, focusing on complications, recurrence, pain relief, and healthcare costs. Design: Multicentre, ra

BackgroundJuvenile Dermatomyositis (JDM) is the most common chronic idiopathic inflammatory myopathy in children. The diagnosis is clinical. Baseline laboratory and complementary studies trace the phenotype of these patients. The objective of this study was to describe epidemiological, clinical and laboratory characteristics at diagnosis of JDM patients included in the Spanish JDM registry, as well as to identify prognostic factors on these patients.MethodsWe retrospectively reviewed clinical features, laboratory tests, and complementary studies at diagnosis of JDM patients included on the Spanish JDM registry. These data were analyzed to assess whether there was a relationship with the development of complications and time to disease inactivity.ResultsOne hundred and sixteen patients from 17 Spanish paediatric rheumatology centres were included, 76 girls (65%). Median age at diagnosis was 7.3 years (Interquartile range (IQR) 4.5–10.2). All patients had pathognomonic skin lesions at the beginning of the disease. Muscle weakness was present in 86.2%. Median Childhood Muscle Assessment Scale was 34 (IQR 22–47). Twelve patients (34%) had dysphagia and 3,5% dysphonia. Anti-p155 was the most frequently detected myositis specific antibody, followed by anti-MDA5. Twenty-nine patients developed calcinosis and 4 presented with macrophage activation syndrome. 70% reached inactivity in a median time of 8.9 months (IQR 4.5–34.8). 41% relapsed after a median time of 14.4 months (IQR 8.6–22.8) of inactivity. Shorter time to treatment was associated with better prognosis (Hazard ratio (HR) = 0.95 per month of evolution, p = 0.02). Heliotrope rash at diagnosis correlates with higher risk of development complications.ConclusionsWe describe heliotrope rash as a risk factor for developing complications in our cohort of JDM patients, an easy-to-evaluate clinical sign that could help us to identify the group of patients we should monitor closely for this complication.

Background:Sjögren’s syndrome is a chronic autoimmune systemic disease that accumulates extragladular manifestations and complications such as lymphoma over time. Poor prognostic factors are described in the scientific literature [1].Objectives:To describe the clinical evolution of patients with Sjögren’s syndrome (SS) in relation to the appearance of new systemic manifestations and disease activity, as well as factors associated with an unfavorable outcome.Methods:SJÖGRENSER PROS (SS-PROS) is an observational, longitudinal, and multicentric study of patients with SS who met the 2002 classification criteria under active follow-up in rheumatology clinics of 28 Spanish hospitals that participated in the cross-sectional phase of the study (SJÖGRENSER TRANS; SS-TRANS) [2]. For the prospective phase, an 8 year follow-up visit was performed (during 2021-2022) and results were compared to the baseline visit (SS-TRANS performed in 2013-2014, 437 patients included). Medical record was reviewed and a medical interview was performed. Epidemiological, clinical and serological variables, as well as causes of death were recorded. Continuous and categorical variables were analyzed using means, medians, and frequencies, with their respective deviations and interquartile ranges (p25-p75). Student’s T test was used to establish statistical associations, considering p<0.05 as significant.Results:Three hundred and fourteen patients have been included, 95% were women, with a mean age of 66 years old (SD 11.5) and mean evolution time from diagnosis to inclusion of 17 years (SD 6.5). Mean ESSDAI scores were similar in SS-PROS and SS-TRANS: 3.67 (SD 5.5) vs 3.5 (SD 5.2), respectively. In the analysis by domains, the joint, hematological and biological domains were the most frequently involved and those that accumulate more changes (decrease or increase) over time; for the rest of the domains, stability was evidenced in ≥95% of the cases (Table 1). By organs, the most affected organ in the follow-up was the lung, with 35 new patients who scored in to some degree of at this domain, being 53 the total number of accumulated patients who scored to some degree at this domain during the 8 year period of follow up. After 8 years, 15 lymphomas (4.8%) have accumulated in this cohort, 6 in the SS-TRANS and 9 new lymphomas in the SS-PROS. The mean ESSPRI improved slightly from SS-TRANS to SS-PROS: 5.24 (SD 2.5) vs 4.66 (SD 2), respectively; The improvement was greater in the pain VAS, 5 (SD 3.2) vs 4 (SD 2.9), respectively; The mean VAS for dryness and fatigue in SS-TRANS and SS-PROS respectively was: 6 (SD 2.6) vs 5.6 (SD 2.5) and 4.6 (SD 3.2) vs 4 (SD 3). Forty-two patients died since their inclusion in SS-TRANS (13.4%), 88% women, with a mean age of 75 years old (SD 11.5), and a 20 years long of disease evolution (SD 7.4). Comparing baseline visit data (SS-TRANS) from the group of deceased (during SS-PROS) and non-deceased (remaining under follow-up in SS-PROS), we observed that age (70 years; SD 10), lasting of the disease (11 years; SD 7.33) and ESSDAI score (6.12; SD 7.7), were higher in deceased vs non-deceased (age 57 years, SD 11; time of evolution 8 years, SD 6; ESSDAI 3, SD 4.6), this difference being significant in age (p<0.001) and with a marked trend in the lasting of the disease and the ESSDAI score, although statistical significance was not reached (p=0.078 and p=0.087 respectively).Conclusion:Patients with SS develop new systemic manifestations over the years, despite maintaining or improving ESSDAI score, suggesting the need for close follow-up. ESSPRI experiences few variations over time, which represents a great challenge for the scientific community. The incidence of lymphoma in this cohort was 4.8%. Mortality in this cohort is 13.4%. Older age, longer duration of the disease, and higher baseline ESSDAI score were associated with a worse outcome.REFERENCES:[1] Mariette X. N Engl J Med 2018;378:931-9.[2] Fernandez -Castro M. Rheumatol Clin 2016;12(4):184-9.Table 1.ESSDAI DomainDomainAccumulated(TRANS+PROS)(number of pacientes)Articular 298 + 59=157 46 + 7=13Pulmonary 53 + 9=12 1013 + 22=35 152 + 4=6Hematological 262 + 69=131 414 + 12=26 62 + 2=4Acknowledgements:“Proyecto SJOGRENSER del grupo de enfermedades autoinmunes sistémicas de la Sociedad Española de Reumatologia.”Disclosure of Interests:None declared.

We conducted a multicentric national study (SEIMC-CEME-22), to describe the clinical and epidemiological profile of the mpox outbreak in Spain, including the management of the disease. This was a retrospective national observational study conducted by Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica (SEIMC) and Foundation SEIMC-GESIDA. We included patients with a confirmed mpox diagnosis before 13 July 2022, and attended at the Spanish health network (the early phase of the outbreak). Epidemiological, clinical, and therapeutic data were collected. Of a total of 1472 patients from 52 centers included, 99% of them were cisgender men, mostly middle-aged, and 98.6% were residents in Spain. The main suspected route of transmission was sexual exposure, primarily among MSM. Occupational exposure was reported in 6 patients. Immunosuppression was present in 40% of patients, mainly due to human immunodeficiency virus (HIV). Only 6.5% of patients had been vaccinated against orthopoxvirus. Virus sequencing was performed in 147 patients (all B.1 lineage). Rash was the most frequent symptom (95.7%), followed by fever (48.2%), adenopathies (44.4%) myalgias (20.7%), proctitis (17%), and headache (14.7%). Simultaneously diagnosed sexually transmitted infections included syphilis (n = 129), gonococcal infection (n = 91), HIV (n = 67), chlamydia (n = 56), hepatitis B (n = 14), and hepatitis C (n = 11). No therapy was used in 479 patients (33%). Symptomatic therapies and antibiotics were used in 50% of cases. The most used therapy regimens were systemic corticoids (90 patients), tecovirimat (6 patients), and cidofovir (13 patients). Smallpox immunoglobulins were used in 1 patient. Fifty-eight patients were hospitalized, and 1 patient died. Mpox outbreak in Spain affected primarily middle-aged men who were sexually active and showed a high rate of HIV infection. A range of heterogeneous therapeutics options was performed.

BackgroundChildhood trauma is intimately related with suicidal behaviour. Patients who have suffered childhood trauma develop impaired Reflective Functioning (RF), which refers to the capacity to understand ourselves and others in terms of intentional mental states. An improvement in RF has been associated with a reduction in suicidal attempts, but the mediating role of RF between childhood trauma and suicidal behaviour has not been addressed so far. ObjectiveWe aim to examine the potential mediating effect of RF among childhood trauma and suicide attempts. MethodWe included 748 patients who had attempted suicide at least once. They were asked to complete the Reflective Functioning Questionnaire (RFQ-8), the Columbia-Suicide Severity Rating scale (CSSRS), and the Childhood Trauma Questionnaire-Short Form (CTQ-SF). We conducted linear regressions by simple mediating model to examine the role of RF in the indirect association between childhood trauma and the number of suicide attempts. ResultsOur results show significant indirect effects through hypo and hypermentalizing between Emotional Abuse (EA) and Sexual Abuse (SA) in childhood and the number of suicide attempts in lifetime. These results indicate that ineffective RF significantly mediates the association between childhood trauma and suicidality. ConclusionThis is the first study supporting the mediational role of RF in the relationship between EA and SA, and the number of suicide attempt in lifetime. These findings have important implications for reducing suicide rates and preventing future re-attempts. Further studies analysing this mediating role and focusing efforts on increasing RF-based interventions are required.

To describe the characteristics of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD), compare their presentation and evolution, and analyse possible complication predictors. Multicenter study. Data were retrieved from a hospital-based study of patients with a diagnosis or suspected diagnosis of sJIA or AOSD according to the responsible physician and followed-up for at least one year. Descriptive variables (classification criteria, clinical manifestations, complications, family, and personal history) were collected at disease onset and during follow-up. We present the clinical characteristics of 326 patients, 67% of whom had a diagnosis of sJIA and 33% of AOSD. Clinical manifestation frequencies were similar between the two groups, except for odynophagia, which was significantly more frequent in AOSD than in sJIA (78.4% vs. 25.5%; p < 0.0001). Among the complications, macrophage activation syndrome (MAS) was significantly more common in sJIA than in AOSD (24.4% vs. 9.5%; p = 0.002), to the extent that an sJIA diagnosis significantly increased the risk of MAS, together with serositis presence, and the need for biological therapy. Patients with sJIA and AOSD showed similar characteristics, supporting the idea that they are both part of Still’s disease, but are expressed at different ages. Differences in manifestations and complications might be due to different management between diseases and immune response maturity.Supplementary InformationThe online version contains supplementary material available at 10.1007/s00296-024-05658-6.