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The ideal duration of anticoagulation therapy in patients with autoimmune disorders and venous thromboembolism (VTE) is controversial. We used the Registro Informatizado de Enfermedad TromboEmbólica (RIETE) database to compare the incidence rate of major bleeding during anticoagulation versus the incidence rate of VTE recurrences after its discontinuation. We included 1061 patients with autoimmune disorders and VTE followed-up after discontinuing anticoagulant therapy: rheumatoid arthritis, 321; polymyalgia rheumatica, 159; ulcerative colitis, 134; Crohn’s disease, 111; systemic lupus erythematosus (SLE), 82; giant cell arteritis, 58; ankylosing spondylitis, 39; Behcet disease, 17; other vasculitides, 140. During anticoagulation (median, 183 days), 64 patients had major bleeding. After discontinuing anticoagulation (median, 190 days), 112 patients developed symptomatic VTE recurrences. In most subgroups, the incidence rate of major bleeding during therapy was similar to the incidence rate of VTE recurrences after its discontinuation. However, in patients with SLE (10.0 major bleeds, 95% confidence interval [CI] 4.07-20.9) per 100 patient-years vs. 3.62 VTE recurrences, 95% CI 1.15-8.74) or ankylosing spondylitis (10.9 major bleeds [95% CI 3.47-26.3] vs. 4.69 VTE recurrences, 95% CI 1.19-12.8) the incidence rates of major bleeding during anticoagulation were over twofold higher than the incidence rates of VTE recurrences after its discontinuation.

Summary Autoimmune disease is a risk factor for first incident venous thromboembolism (VTE). However, data on the risk of recurrent VTE in people with autoimmune disease is sparse. We explored the risk of recurrent VTE using the RIETE registry, comparing people with autoimmune disease (n = 1305) to those without (n = 50608). Overall rates were 6.5 and 5.1 recurrent VTE/100 years for patients with autoimmune disease vs controls, respectively. After adjustment for sex and unprovoked/provoked VTE yielded an adjusted hazard ratio of 1.29 (95%CI 1.03‐1.62). The analysis was limited by short median follow up time (161 days overall), precluding definitive conclusions on recurrent VTE risks.

El tutor de la tesis es Ángel Sampériz Legarre Es ampliamente aceptado que la enfermedad tromboembólica venosa es más frecuente en enfermos con condiciones autoinmunes que en población general. El registro RIETE es la base de datos más amplia que recoge sujetos con enfermedad tromboembólica documentada. Dentro de las variables que incluye, existe una categoría específica en la que constan diferentes enfermedades autoinmunes. El primer trabajo de este compendio, estudia el período durante el tratamiento anticoagulante y compara todos los enfermos con enfermedades autoinmunes incluidos en RIETE con el resto. Encuentra ausencia de diferencias en recurrencias de enfermedad tromboembólica, en sangrados mayores y una menor mortalidad en el grupo a estudio. Además, desgrana una a una las entidades autoinmunes incluidas dentro del grupo a estudio detectando importantes diferencias. El segundo estudio que, aunque no incluido en el compendio, sí desarrolla dicha línea investigadora, aborda el período incluido una vez se suspende el tratamiento anticoagulante. Compara recurrencias de enfermedad tromboembólica entre todos los sujetos con una condición autoinmune y el resto del RIETE. Detecta mayores recurrencias en el grupo a estudio. El tercer artículo aborda el mismo escenario que el primer artículo a nivel local del Hospital reina Sofía de Tudela. El cuarto artículo es una crítica constructiva desde el punto de vista metodológico a la publicación que aborda el tema a estudio del grupo COMMAND VTE japonés. El quinto artículo se adentra de lleno en la comparativa directa entre el comportamiento clínico de enfermedad tromboembólica entre las diferentes enfermedades autoinmunes incluidas en RIETE. Detecta tasas de sangrado durante el tratamiento y recurrencias al suspenderlo similares en todas las enfermedades incluidas en el estudio salvo en dos, el lupus eritematoso sistémico y espondilitis anquilosante, donde las tasas de sangrado durante el tratamiento son más del doble que las recurrencias al suspenderlo. It is widely accepted that venous thromboembolic disease is more frequent in patients with autoimmune conditions than in the general population. The RIETE registry is the largest database that includes subjects with documented thromboembolic disease. Within the variables that they include, there is a specific category with different autoimmune diseases. The first study involves the period during anticoagulant treatment and compares all patients with autoimmune diseases included in RIETE with the rest. It finds no differences in recurrence of thromboembolic disease, in major bleeding and lower mortality in the study group. In addition, it breaks down one by one the autoimmune entities included in the study group, detecting important differences. The second study, which, although not included in the compendium, does develop this research line, deals with the period included once the anticoagulant treatment is suspended. It compares recurrences of thromboembolic disease between all subjects with an autoimmune condition and the rest of the RIETE and detects higher recurrences in the study group. The third article deals with the same scenario as the first article at the local level of the Reina Sofía Hospital in Tudela. The fourth article is a constructive criticism from the methodological point of view of the publication that addresses the subject under study of the Japanese COMMAND VTE group. The fifth article goes deep into a direct comparison between the clinical behavior of thromboembolic disease among the different autoimmune diseases included in RIETE. It finds that the rates of bleeding during treatment and recurrences upon discontinuation are similar in all the diseases included in the study except for two, systemic lupus erythematosus and ankylosing spondylitis, where the rates of bleeding during treatment are more than double that of recurrences upon discontinuation. Programa de Doctorado en Ciencias de la Salud (RD 99/2011) Osasun Zientzietako Doktoretza Programa (ED 99/2011)

In Spain, more than 10% of patients discharged with acute heart failure (AHF) are readmitted in the first 30 days. This study is designed to assess whether the treatment of AHF guided by clinical ultrasound (CU) in the setting of hospitalization at home (HAH) reduces the incidence of readmission and mortality compared to the standard care (SC).A randomized, open, parallel, single-center and controlled clinical trial (RCT) was designed (NT05042752). Patients18 years of age admitted for AHF to HAD from January 2021 to April 2021 at the Reina Sofía Hospital in Tudela were consecutively included. The patients were randomized to the UG-ultrasound group (SC and CU performed) and the CG-control group (SC). The diuretic treatment was tailored according to the findings of the SC together with the CU or according to the findings of the SC respectively. The main variables were the relative risk of readmission and mortality from AHF.A total of 79 patients were randomized, 39 to UG and 40 to CG. Of these, only 35 of the UG and 35 of the CG completed the intervention. The risk of readmission due to AHF was reduced by 60% in UG compared to CG (RR 0.4; 95% CI: 0.1-1) and mortality by 30% (RR 0.7; 95% CI: 0.2-2.2). Despite the relevant magnitude of the effect found, the results did not reach statistical significance due to lack of power.Our results suggest that in HAH, a CE guided strategy for AHF could reduce the risk of readmission and mortality compared to SC alone. However, studies with greater statistical power are needed to confirm these results.

Background Atrial fibrillation (AF) may exist before or occur early in the course of pulmonary embolism (PE). We determined the PE outcomes based on the presence and timing of AF. Methods and Results Using the data from a multicenter PE registry, we identified 3 groups: (1) those with preexisting AF, (2) patients with new AF within 2 days from acute PE (incident AF), and (3) patients without AF. We assessed the 90‐day and 1‐year risk of mortality and stroke in patients with AF, compared with those without AF (reference group). Among 16 497 patients with PE, 792 had preexisting AF. These patients had increased odds of 90‐day all‐cause (odds ratio [OR], 2.81; 95% CI, 2.33–3.38) and PE‐related mortality (OR, 2.38; 95% CI, 1.37–4.14) and increased 1‐year hazard for ischemic stroke (hazard ratio, 5.48; 95% CI, 3.10–9.69) compared with those without AF. After multivariable adjustment, preexisting AF was associated with significantly increased odds of all‐cause mortality (OR, 1.91; 95% CI, 1.57–2.32) but not PE‐related mortality (OR, 1.50; 95% CI, 0.85–2.66). Among 16 497 patients with PE, 445 developed new incident AF within 2 days of acute PE. Incident AF was associated with increased odds of 90‐day all‐cause (OR, 2.28; 95% CI, 1.75–2.97) and PE‐related (OR, 3.64; 95% CI, 2.01–6.59) mortality but not stroke. Findings were similar in multivariable analyses. Conclusions In patients with acute symptomatic PE, both preexisting AF and incident AF predict adverse clinical outcomes. The type of adverse outcomes may differ depending on the timing of AF onset.

Patients with autoimmune disorders are at an increased risk of venous thromboembolism (VTE), but this association has not been consistently evaluated. We used the RIETE (Registro Informatizado Enfermedad Trombo Embólica) database to compare the rates of VTE recurrences, major bleeding, and death during the course of anticoagulation, according to the presence or absence of autoimmune disorders. Of 71 625 patients with VTE recruited in February 2018, 1800 (2.5%) had autoimmune disorders. Median duration of anticoagulant therapy was slightly longer in patients with autoimmune disorders (median, 190 vs 182 days; P = .001). On multivariable analysis, patients with autoimmune disorders had a similar risk of VTE recurrences (hazard ratio [HR]: 0.93; 95% confidence interval [CI]: 0.68-1.27) or major bleeding (HR: 1.07; 95% CI: 0.82-1.40) and a lower risk to die (HR: 0.66; 95% CI: 0.54-0.81) than those without autoimmune disorders. Patients with giant cell arteritis had the highest rates of major bleeding (8.6 events per 100 patient-years) and the lowest rate of recurrences (zero). In other subgroups, the rates of both events were more balanced. During anticoagulation, patients with or without autoimmune disorders had similar rates of VTE recurrences or major bleeding. However, there were some differences between subgroups of patients with autoimmune disorders.

Background: The choice of the imaging modality for diagnosis of pulmonary embolism (PE) could be influenced by provider, patient or hospital characteristics, or over time. However, little is known about the choice of the diagnostic modalities in practice. The aim of this study was to evaluate the variations in the use of imaging modalities for patients with acute PE. Methods: Using the data from Registro Informatizado Enfermedad TromboEmbolica (RIETE), a prospective international registry of patients with venous thromboembolism (March 2001–January 2019), we explored the imaging modalities used in patients with acute PE. The imaging modalities included computed tomography pulmonary angiography, ventilation/perfusion scanning, pulmonary angiography, a combination of these tests, or PE signs and symptoms plus imaging-confirmed proximal deep vein thrombosis but no chest imaging. Results: Among 38 025 patients with confirmed PE (53.1% female, age: 67.3±17 years), computed tomography pulmonary angiography was the dominant modality of diagnosis in all RIETE enrollees (78.2% [99% CI, 77.6–78.7]); including pregnant patients (58.9% [99% CI, 47.7%–69.4%]) and patients with severe renal insufficiency (62.5% [99% CI, 59.9–65.0]). A greater proportion of patients underwent ventilation/perfusion scanning in larger hospitals compared with smaller hospitals (13.1% versus 7.3%, P P Conclusions: In a large multinational PE registry, variations were observed in the use of imaging modalities according to patient or institutional factors and over time. However, computed tomography pulmonary angiography was the dominant modality of diagnosis, even in pregnancy and severe renal insufficiency. The safety, costs, and downstream effects of these tests on PE-related and non-PE-related outcomes warrant further investigation.

OBJECTIVE: Systemic lupus erythematosus (SLE) is characterized by an unpredictable and fluctuating course. Although various methods have been developed to measure disease activity, there is still a lack of consensus about the optimal criteria for SLE remission. The principal aim of our study was to identify the number of lupus patients achieving a complete remission (implying that for 3 years there were no clinical or serologic features and no treatment with steroids and immunosuppressive drugs) in a single cohort of patients followed for a period of up to 32 years. In addition, we have identified patients in clinical but not serologic remission (known as serologically active, clinically quiescent disease [SACQ]) and vice versa. We were particularly interested to determine the factors associated with complete remission. METHODS: Eligible patients were followed up in the University College Hospital Lupus cohort from January 1978 until December 2010 for a period of at least 3 years. Complete remission was defined as a period of at least 3 years with clinical inactivity (British Isles Lupus Assessment Group scores of C, D, or E only) and laboratory remission (no antibodies to double-stranded DNA and normal complement C3 levels), and being off-treatment with corticosteroids and immunosuppressants. Antimalarial and nonsteroidal antiinflammatory drugs were allowed. RESULTS: Of 624 lupus patients at our hospital, a total of 532 patients met the strict inclusion criteria for the study. Of these 532 patients, 77 patients (14.5%) achieved complete remission for at least 3 years, and 23 (4.3%) achieved complete remission for a minimum period of 10 years. Ten of these 77 patients were subsequently lost to followup, and, interestingly, flares occurred subsequently in 15 of the 67 remaining patients (22.4%). Three patients relapsed after the tenth year of remission. Forty-five patients (8.5%) fulfilled the requirement for SACQ, and 66 patients (12.4%) achieved only serologic remission. CONCLUSION: Our study indicated that 14.5% of lupus patients achieved a complete remission for 3 years. However, flares may continue to occur beyond 10 years of remission. Long-term followup of SLE is therefore mandatory.

Taking advantage of an interesting clinical scenario, we want to introduce a discussion about fatality in our daily practice and the need to accept that. An 80 year-old man with non-traumatic spontaneous bleeding tendency came to the clinics. Although being on warfarin as a consequence of primary thrombotic prophylaxis due to an atrial fibrillation, full assessment was performed. Not only the rare entity found on him, but also the severe complication that happened afterwards challenged clinicians and led them to risky treatment options.

Advances in our knowledge of the pathogenic mechanisms of antiphospholipid syndrome have been achieved in the past few years. Apart from the well-known role of anti-β2-glycoprotein I antibodies, complement, endocrine and genetic factors and a variety of other molecules are now under investigation. These new approaches should lead to novel explanations and potential new treatment options.

Primary hyperfibrinolysis States are rare entities associated with different clinical scenarios and can be sometimes their first presentation. PHS outcome relies on the underlying cause. We introduce the case of a man presenting with a complex clotting abnormality malignancy related. Not having an accurate diagnosis hindered us to give an easy solution and compromised the prognosis overall. Misdiagnosing a severe entity often leads to fatal outcomes. We want to highlight the rarity of our case, the challenging differential diagnosis and the importance of being aware of PHS. Because our patient appeared to have an extended carcinoma, definitive treatment was not an option on our case.When an uncommon and challenging disease is presented, having all the clues is mandatory to succeed.

The role of Serratia marcescens changed from a harmless saprophytic microorganism to an important opportunistic human pathogen. It often causes nosocomial device-associated outbreaks and rarely serious invasive community acquired infections. We present a case of a community-acquired Serratia marcescens bacteremia leading to Respiratory Distress Syndrome in a previously healthy 51-year-old man without identifiable risk factors. Full recovery was achieved with solely medical treatment and observation in ICU during three days. To our knowledge it is an extremely uncommon presentation and just few cases have been previously reported in the literature.

Systemic lupus erythematosus (SLE) is characterized by an unpredictable and fluctuating course. Although various methods have been developed to measure disease activity, there is still a lack of consensus about the optimal criteria for SLE remission. The principal aim of our study was to identify the number of lupus patients achieving a complete remission (implying that for 3 years there were no clinical or serologic features and no treatment with steroids and immunosuppressive drugs) in a single cohort of patients followed for a period of up to 32 years. In addition, we have identified patients in clinical but not serologic remission (known as serologically active, clinically quiescent disease [SACQ]) and vice versa. We were particularly interested to determine the factors associated with complete remission.Eligible patients were followed up in the University College Hospital Lupus cohort from January 1978 until December 2010 for a period of at least 3 years. Complete remission was defined as a period of at least 3 years with clinical inactivity (British Isles Lupus Assessment Group scores of C, D, or E only) and laboratory remission (no antibodies to double-stranded DNA and normal complement C3 levels), and being off-treatment with corticosteroids and immunosuppressants. Antimalarial and nonsteroidal antiinflammatory drugs were allowed.Of 624 lupus patients at our hospital, a total of 532 patients met the strict inclusion criteria for the study. Of these 532 patients, 77 patients (14.5%) achieved complete remission for at least 3 years, and 23 (4.3%) achieved complete remission for a minimum period of 10 years. Ten of these 77 patients were subsequently lost to followup, and, interestingly, flares occurred subsequently in 15 of the 67 remaining patients (22.4%). Three patients relapsed after the tenth year of remission. Forty-five patients (8.5%) fulfilled the requirement for SACQ, and 66 patients (12.4%) achieved only serologic remission.Our study indicated that 14.5% of lupus patients achieved a complete remission for 3 years. However, flares may continue to occur beyond 10 years of remission. Long-term followup of SLE is therefore mandatory.

Objetivo: Demostrar el riesgo añadido derivado de la Hospitalización Convencional (HC) cuando ésta puede ser sustituida por la Hospitalización a Domicilio (HAD) en el contextode reagudizaciones de Enfermedad Pulmonar Obstructiva Crónica (EPOC).Pacientes y métodos: Estudio de cohortes histórico con análisis retrospectivo y comparativo de datos en que se seleccionan pacientes ingresados en HAD desde el 2010 hasta la actualidad. Se comparan 30 pacientes con reagudización de EPOC que cumplían criterios de ingreso en HAD e ingresan en HAD con 30 pacientes con reagudización de EPOC que cumplían criterio de HAD, pero ingresan en HC.Resultados: No se apreciaron diferencias en las características basales de los dos grupos. Se midieron los reingresos en el hospital y la mortalidad por reagudización de EPOC y por otras causas al mes del alta. En el grupo HC se observó mayor reingreso por reagudización de EPOC0,333 (IC 0,118-0,943), mayor reingreso global 0,345 (IC 0,155-0,772) así como mayor mortalidad global 0,103 (IC 0,15-0,696).Conclusiones: Nuestro estudio sugiere que existe un riesgo aumentado de reingreso y mortalidad al mes atribuible a la HC tras un episodio de reagudización de EPOC.

SS is a chronic systemic autoimmune disease characterized by decreased exocrine gland function. A variety of other disease manifestations may also be present, including general constitutional symptoms and extraglandular features. A multidisciplinary approach focused on both local and systemic medical therapies is needed as the disease has a wide clinical spectrum. The current treatment for SS is mainly symptomatic. However, there is evidence that systemic drugs are effective in controlling extraglandular manifestations of the disease. Overall evidence for the role of conventional immunosuppressive therapy is limited. A number of attempts to use biologic therapies have led to variable results. Biologic agents targeting B cells, such as rituximab, epratuzumab and belimumab, have shown promising results, but further studies are needed to validate the findings. Early-phase studies with abatacept and alefacept proved that T cell stimulation inhibition is another potentially effective target for SS treatment. Modulation or inhibition of other targets such as IFN, IL-6 and Toll-like receptor are also currently being investigated. We have summarized the available evidence regarding the efficacy of biologic treatments and discuss other potential therapies targeting pathways or molecules recognized as being involved in the pathogenesis of SS.

Varón de 85 años anticoagulado con Rivaroxaban por una fibrilación auricular, que acude por traumatismo costal izquierdo. El paciente se encontraba en tratamiento con nuevos antcoagulantes (NACOS)debido al antecedente de una hemorragia craneal en relación con Warfarina. Tras objetivarse varias fracturas izquierdas, se decide tratamiento conservador. A las 24 horas, sufre mareo por lo que se re-explora, objetivándose hipoventilación derecha.

Presentamos un caso de rabdomiólisis tras la utilización de un dispositivo de electroestimulación muscular y revisamos los casos previos publicados de esta asociación

espanolPresentamos un caso de una paciente anciana con un sindrome de Ramsay Hunt (SRH) (herpes zoster otico) con afectacion de multiples pares craneales (VII, VIII y V) que presento simultaneamente una meningitis linfocitaria por virus varicela zoster y que evoluciono favorablemente con esteroides y aciclovir. Se revisan las principales caracteristicas del SRH, haciendo enfasis en la coexistencia simultanea de afectacion de multiples pares craneales y meningitis linfocitaria en una paciente anciana inmunocompetente. EnglishWe present a case of Ramsay Hunt syndrome (RHS) (herpes zoster oticus) with multiple cranial nerve involvement (VII, VIII, and V nerves) and lymphocytic meningitis due to varicella-zoster virus in an elderly woman. Clinical evolution was satisfactory with aciclovir and steroids. We review the main characteristics of RHS, underscoring the coexistence of multiple cranial root involvement and lymphocytic meningitis in an elderly immunocompetent host.