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Pulmonary nodular lymphoid hyperplasia, also known as pseudolymphoma, is an uncommon reactive lymphoproliferative disorder of unknown etiology that can be found in Sjogren's syndrome patients. Here, we present a case of a previously healthy woman in which the incidental finding of a lung mass compatible with nodular lymphoid hyperplasia led to the subsequent diagnosis of Sjogren's syndrome. We also performed a literature review for the association between both entities and described the main clinical aspects of the reported cases. Although its rarity, we consider that pulmonary nodular lymphoid hyperplasia should be considered in the differential diagnosis of lung nodules or masses among Sjogren's syndrome patients.

The purpose of this study was to explore the occupational performance and needs of patients with hereditary transthyretin amyloidosis (ATTRv). A semi-structured interview was conducted by an occupational therapist with 44 patients with Val50Met-ATTRv recruited through patient associations. The interview addressed three related dimensions. The first one, the physical dimension, was evaluated using the Spanish versions of the Barthel Index, the Lawton and Brody scale, and the Norfolk questionnaire; the second one, the psychological dimension, was assessed with the Warwick–Edinburgh Mental Well-Being Scale and the SF-36 questionnaire; and the third dimension, the occupational performance, was assessed through unstructured questions on daily occupations, work, roles, and hobbies given the lack of standardized scales. Twenty participants (45.4%) responded that the disease had affected their basic activities of daily living, twenty- four (54.5%) perceived an impact on their instrumental activities of daily living, and all the participants reported that the disease symptoms had affected their ability to perform advanced activities as well as their employment status. Only three patients (6.8%) reported a lack of psychological impairment following disease diagnosis. These findings suggest that a semi-structured interview conducted by an occupational therapist can provide essential information that should be considered for the implementation of occupational therapy programs targeting patients living with a diagnosis of ATTRv.

© 2020 Elsevier Ltd. All rights reserved., Background: Hereditary transthyretin-mediated amyloidosis is a rare, inherited, progressive disease caused by mutations in the transthyretin (TTR) gene. We assessed the safety and efficacy of long-term treatment with patisiran, an RNA interference therapeutic that inhibits TTR production, in patients with hereditary transthyretin-mediated amyloidosis with polyneuropathy. Methods: This multicentre, open-label extension (OLE) trial enrolled patients at 43 hospitals or clinical centres in 19 countries as of Sept 24, 2018. Patients were eligible if they had completed the phase 3 APOLLO or phase 2 OLE parent studies and tolerated the study drug. Eligible patients from APOLLO (patisiran and placebo groups) and the phase 2 OLE (patisiran group) studies enrolled in this global OLE trial and received patisiran 0·3 mg/kg by intravenous infusion every 3 weeks with plans to continue to do so for up to 5 years. Efficacy assessments included measures of polyneuropathy (modified Neuropathy Impairment Score +7 [mNIS+7]), quality of life, autonomic symptoms, nutritional status, disability, ambulation status, motor function, and cardiac stress, with analysis by study groups (APOLLO-placebo, APOLLO-patisiran, phase 2 OLE patisiran) based on allocation in the parent trial. The global OLE is ongoing with no new enrolment, and current findings are based on the interim analysis of the patients who had completed 12-month efficacy assessments as of the data cutoff. Safety analyses included all patients who received one or more dose of patisiran up to the data cutoff. This study is registered with ClinicalTrials.gov, NCT02510261. Findings: Between July 13, 2015, and Aug 21, 2017, of 212 eligible patients, 211 were enrolled: 137 patients from the APOLLO-patisiran group, 49 from the APOLLO-placebo group, and 25 from the phase 2 OLE patisiran group. At the data cutoff on Sept 24, 2018, 126 (92%) of 137 patients from the APOLLO-patisiran group, 38 (78%) of 49 from the APOLLO-placebo group, and 25 (100%) of 25 from the phase 2 OLE patisiran group had completed 12-month assessments. At 12 months, improvements in mNIS+7 with patisiran were sustained from parent study baseline with treatment in the global OLE (APOLLO-patisiran mean change -4·0, 95 % CI -7·7 to -0·3; phase 2 OLE patisiran -4·7, -11·9 to 2·4). Mean mNIS+7 score improved from global OLE enrolment in the APOLLO-placebo group (mean change from global OLE enrolment -1·4, 95% CI -6·2 to 3·5). Overall, 204 (97%) of 211 patients reported adverse events, 82 (39%) reported serious adverse events, and there were 23 (11%) deaths. Serious adverse events were more frequent in the APOLLO-placebo group (28 [57%] of 49) than in the APOLLO-patisiran (48 [35%] of 137) or phase 2 OLE patisiran (six [24%] of 25) groups. The most common treatment-related adverse event was mild or moderate infusion-related reactions. The frequency of deaths in the global OLE was higher in the APOLLO-placebo group (13 [27%] of 49), who had a higher disease burden than the APOLLO-patisiran (ten [7%] of 137) and phase 2 OLE patisiran (0 of 25) groups. Interpretation: In this interim 12-month analysis of the ongoing global OLE study, patisiran appeared to maintain efficacy with an acceptable safety profile in patients with hereditary transthyretin-mediated amyloidosis with polyneuropathy. Continued long-term follow-up will be important for the overall assessment of safety and efficacy with patisiran.

Hereditary transthyretin amyloidosis (ATTRV30M) is a rare disease caused by amyloid deposition and characterized by a heterogeneous presentation. Anticipation (AC) is described as the decrease in age at onset (AO) within each generation. Our aim was to study AC in a large number of ATTRV30M kindred from Majorca (Spain), and gain further insight into parent-of-origin effects.In a cohort of 262 subjects with ATTRV30M amyloidosis belonging to 51 families, we found 37 affected pairs. AO is defined as the age at the first symptom and AC (parent's age at disease onset minus that of the offspring) were calculated. Chi-square test, independentOffspring mean AO was 16 years lower than that of the parents (AC was no uncommon in our cohort, and AO tended to decrease in successive generations.

This study was designed to investigate the global utilization of occupational therapy (OT) services by patients with hereditary transthyretin amyloidosis (ATTRv) in Spain. The main objective was to find out whether these patients have access to OT services and the types of interventions being offered to them, together with their satisfaction and real benefits as users. We developed an online questionnaire which was distributed to patients with ATTRv in Spain through patient associations. Seventy-four patients with a diagnosis of ATTRv residing in Spain participated in the study. Thirteen had already used OT services at least once, felt that OT interventions improved their quality of life, would recommend OT services to others, and would return to see an occupational therapist. However, 61 had never used this type of service before. Of these, 35 knew what OT is and 13 declared that they considered that OT interventions in ATTRv could be positive for them. The results suggest that the use of OT services by ATTRv patients is low, mainly because of the lack of information about the occupational profile of individuals with this disease. The low response rate obtained for the survey limits generalization, and thus further research to confirm these preliminary findings is needed.

© 2020 The Author(s)., [Objectives]: The objective of this study was to estimate the association between tocilizumab or corticosteroids and the risk of intubation or death in patients with coronavirus disease 19 (COVID-19) with a hyperinflammatory state according to clinical and laboratory parameters., [Methods]: A cohort study was performed in 60 Spanish hospitals including 778 patients with COVID-19 and clinical and laboratory data indicative of a hyperinflammatory state. Treatment was mainly with tocilizumab, an intermediate-high dose of corticosteroids (IHDC), a pulse dose of corticosteroids (PDC), combination therapy, or no treatment. Primary outcome was intubation or death; follow-up was 21 days. Propensity score-adjusted estimations using Cox regression (logistic regression if needed) were calculated. Propensity scores were used as confounders, matching variables and for the inverse probability of treatment weights (IPTWs)., [Results]: In all, 88, 117, 78 and 151 patients treated with tocilizumab, IHDC, PDC, and combination therapy, respectively, were compared with 344 untreated patients. The primary endpoint occurred in 10 (11.4%), 27 (23.1%), 12 (15.4%), 40 (25.6%) and 69 (21.1%), respectively. The IPTW-based hazard ratios (odds ratio for combination therapy) for the primary endpoint were 0.32 (95%CI 0.22–0.47; p < 0.001) for tocilizumab, 0.82 (0.71–1.30; p 0.82) for IHDC, 0.61 (0.43–0.86; p 0.006) for PDC, and 1.17 (0.86–1.58; p 0.30) for combination therapy. Other applications of the propensity score provided similar results, but were not significant for PDC. Tocilizumab was also associated with lower hazard of death alone in IPTW analysis (0.07; 0.02–0.17; p < 0.001)., [Conclusions]: Tocilizumab might be useful in COVID-19 patients with a hyperinflammatory state and should be prioritized for randomized trials in this situation., IJ has received honoraria for participating in an advisory board from Gilead Sciences, and for educational activities from ViiV. JB has received research grants from AbbVie, Gilead Sciences, Merck, and ViiV, and honoraria for being a speaker or advisory board participation from AbbVie, Gilead Sciences, Janssen, Merck, and ViiV. JRA received fees for participating in an advisory board, being a speaker, and research grant support from Viiv, Janssen, Gilead, MSD, Teva, Alexa and Serono. PR is involved as speaker or advisory board participant for Gilead Sciences, AbbVie and ViiV. JR-B, JP, JC and MY have no conflicts of interest to declare. SAM-COVID was funded by Spanish Ministry of Science and Innovation, Instituto de Salud Carlos III (COV20/01031) co-funded by European Union (ERDF/ESF, “Investing in your future”) and Fundación SEIMC/GeSIDA. In addition, Juan Berenguer, Jesús Rodríguez-Baño, Inmaculada Jarrín, Jordi Carratalá, Jerónimo Pachón, and José R Arribas received funding for research from Plan Nacional de I+D+i 2013-2016 and Instituto de Salud Carlos III, Subdirección General de Redes y Centros de Investigación Cooperativa, Ministerio de Ciencia, Innovación y Universidades – co- financed by European Development Regional Fund “A way to achieve Europe”, Operative program Intelligent Growth 2014–2020 through the networks: Spanish AIDS Research Network (RIS) [RD16/0025/0017 (JB), RD16/0025/0018 (JRA), RD16/0025/00XX (IJ)] and Spanish Network for Research in Infectious Diseases (REIPI)[RD16/0016/0001 (JRB), RD16/0016/0005 (JC), and RD16/0016/0009 (JP).

Introduction Transthyretin amyloidosis (ATTR amyloidosis) is a clinically heterogeneous disease caused by mutations in the transthyretin (TTR) gene or aggregation of wild-type transthyretin (ATTRwt). In Spain, there are two large endemic foci of ATTR amyloidosis caused by the Val30Met variant, with additional cases across the country; however, these data may be incomplete, as there is no centralized patient registry. The Transthyretin Amyloidosis Outcomes Survey (THAOS) is an ongoing, global, longitudinal, observational survey of patients with ATTR amyloidosis, including both inherited and wild-type disease, and asymptomatic patients with TTR mutations. This analysis aimed to gain a deeper understanding of the clinical profile of patients with ATTR amyloidosis in Spain. Methods This was a descriptive analysis of the demographic and clinical characteristics of symptomatic patients enrolled at six sites geographically dispersed throughout Spain (data cutoff: January 6, 2020). Patient data at enrollment, including genotype, demographics, and clinical presentation for symptomatic patients, were recorded. Patients were grouped by predominant phenotype based on clinical measures at enrollment: predominantly cardiac, predominantly neurologic, or mixed (cardiac and neurologic). Results There were 379 patients (58.0% male; 63.3% symptomatic) enrolled in the six THAOS sites in Spain. Predominant genotypes were the Val30Met mutation (69.1%) or ATTRwt (15.6%). Predominant phenotype distribution was neurologic (50.4%), mixed (35.8%), and cardiac (13.8%) for all symptomatic patients (n = 240); neurologic (67.8%), mixed (21.2%), and cardiac (11.0%) for symptomatic Val30Met (n = 146); and mixed (64.9%), cardiac (22.8%), and neurologic (12.3%) for symptomatic ATTRwt (n = 57). Symptomatic patients reported a range of ATTR amyloidosis signs and symptoms at enrollment, with autonomic neuropathy and sensory neuropathy common in all phenotypes. Conclusions These results from THAOS highlight the phenotypic heterogeneity associated with ATTR amyloidosis in Spain and the importance of comprehensive neurologic and cardiac evaluations in all patients with ATTR amyloidosis. post-print 392 KB

Invasive pneumococcal disease (IPD) presents high mortality in the population at risk. The aim of this work is to know the evolution, clinical and microbiological characteristics of IPD in the adult population of Majorca, since the introduction of a public funded program for pneumococcal conjugate vaccine (PCV-13) in the pediatric population in the Balearic Islands in 2016. For this purpose, a retrospective multicenter study was carried out in which all episodes of IPD in adult patients from the four hospitals of the public health system of Majorca were included, comparing the periods between 2012 and 2015 and between 2016 and 2019. Clinical variables, serotypes and antibiotic sensitivity were collected. There were 498 cases of IPD, 56.8% were male with a mean age of 67 (standard deviation: 16). Most infections were bacterial pneumonias (73.7%). Of the total cases, 264 (53%) presented complications. Of the 498 cases, 351 strains were obtained, of which 145 (41.3%) belong to vaccinal serotypes (included in the PCV-13 vaccine) and 206 (58.7%) to non-vaccinal serotypes (not included in the PCV-13 vaccine). The percentage of IPD caused by vaccinal serotypes was lower in the second period (47.8% vs. 34.5%, p = 0.012).

Background Polyserositis (PS) is the inflammation, with effusion, of different serous membranes. It has been associated with different aetiologies, but the aetiology of PS remains unknown in a high percentage of patients. Aims The general objective of this retrospective study was to analyse the aetiology of PS cases seen at Son Llatzer Hospital in an 11-year period. Other objectives were to determine the epidemiological, clinical and analytical characteristics of these patients. Methods An observational, descriptive and retrospective study to analyse the aetiology of PS cases seen at Son Llatzer Hospital. The inflammation of two or more serous membranes confirmed by an imaging test was required and at least one of the serous fluid should be an exudate. Results A total of 92 patients was included in the study. The most common diagnosis was neoplasm (nearly one-third of cases) followed by infectious and autoimmune diseases. PS aetiology was unknown in more than one-third. Pleura and pericardium were the most common sites of serosal involvement (83%). Antinuclear antibodies' positivity in serum and increased levels of adenosine deaminase in pleural effusion were significantly associated with a final diagnosis of autoimmune disease. Increased pleural lactate dehydrogenase levels were significantly associated with a final diagnosis of neoplasm. In 9 of 14 patients with a previous cancer, PS represented a recurrence of their cancer. Cases of unknown aetiology presented most frequently as pleural and pericardial involvement, and the majority resolved. In very few patients, an infectious aetiology could be proven. Conclusion PS is a frequent clinical entity that is associated with different diseases and its diagnosis could be challenging, with a high rate of unknown aetiologies.

Anti-MDA5 antibodies have been strongly associated with rapidly progressive interstitial lung disease (RP-ILD) in dermatomyositis (DM) patients, especially in the clinically amyopathic subset (CADM). We present a case of anti-MDA5 antibody-associated RP-ILD in a patient with arthritis but with no other clinical signs suggestive of DM or CADM successfully treated with a combination of cyclophosphamide, cyclosporine and corticoids. A review of the literature was also done. Despite its rarity, anti-MDA5 antibody-associated ILD should be suspected in cases of RP-ILD even without other signs of DM or CADM as prompt and aggressive treatment could improve prognosis.

Diagnosis of latent tuberculosis infection in patients with immune-mediated inflammatory chronic diseases (IMIDs) can be challenged as diagnostic test reliability could be impaired by immunosuppression. We retrospectively analyzed the Quantiferon Gold-Test in-Tube (QFT-G-IT) results of all patients with IMIDs seen at the Department of Internal Medicine of Son Llatzer Hospital, Palma de Mallorca (Spain), looking for the factors related to QFT-G-IT indeterminate results. During the study period (2008–2015), 520 patients met the inclusion criteria. Factors associated with indeterminate QFT-G-IT results in a univariate analysis were inflammatory bowel disease, disease activity, lymphopenia, and medium-to-high doses of corticosteroids. In a subsequent multivariate analysis, only lymphopenia (defined as

IgG4-related disease (IgG4-RD) is a recently described entity characterized by lymphoplasmacytic infiltrates, usually mimicking tumors, affecting almost every organ or system. Nevertheless, serosal involvement has been rarely reported. In this article, we report two cases of IgG4-RD with serosal involvement and review the literature. Because of the varied clinical pictures found in our review, we suggest a new terminology for the description of IgG4-RD with serosal involvement.

Hereditary transthyretin amyloidosis (hATTR) is an autosomal dominant systemic amyloidosis due to point mutations in the TTR gene (chr18q12.1), resulting in misfolded proteins. Although more than 1...

IgG4-related disease is a recently described clinicopathological entity showing a wide spectrum of clinical manifestations that share a common pathology. Its most characteristic feature is the formation of inflammatory tumors in different organs, which makes differentiation mainly with neoplastic diseases fundamental. The inflammatory process is typically comprised of IgG4 lymphoplasmacytic cells. The pathophysiological role of the immunoglobulin is not clear. The treatment of choice is corticosteroids. This article aims to summarize the main features of the disease.

Resumen Objetivos Describir las caracteristicas clinicoepidemiologicas de la sifilis en la poblacion de hombres que tienen sexo con hombres en un area de Mallorca. Metodos Revision retrospectiva de los casos de sifilis en pacientes hombres que tienen sexo con hombres (HSH) entre enero de 2005 y junio de 2013 en un hospital de Mallorca. Resultados Encontramos 55 casos de sifilis en HSH, el 34,3% de los casos de sifilis del periodo de estudio. El 74,5% tenian coinfeccion por el virus de la inmunodeficiencia humana (VIH). El 70,7% de los diagnosticos de infeccion por VIH y sifilis fueron simultaneos. Los pacientes con infeccion por VIH tenian una mediana de CD4 de 456 (29-979). Se diagnosticaron por clinica el 49,1% y por cribado el 50,9% de los casos. La sifilis latente tardia o indeterminada fue la entidad mas frecuente (41,9%). Los pacientes HSH tenian mas riego de estar infectados por VIH que los varones no HSH. Conclusiones La mayoria de pacientes HSH con sifilis presentan coinfeccion por el VIH, siendo por lo tanto fundamental el cribado de VIH en estos pacientes, lo que incluso puede permitir un diagnostico temprano de la misma.

Objective To describe the clinical and epidemiological characteristics of syphilis in men who have sex with men (MSM) in an area of Mallorca, Spain. Methods We performed a retrospective analysis of syphilis cases in MSM seen at a hospital in Mallorca between January 2005 and June 2013. Results Fifty-five cases of syphilis were recorded in MSM during the study period (34.3% of all cases diagnosed), and 74.5% of these patients had human immunodeficiency virus (HIV) coinfection. The two diseases had been diagnosed simultaneously in 70.7% of this population. Patients with HIV coinfection had a median CD4 count of 456 cells/μL (range, 29-979 cells/μL). Syphilis was diagnosed clinically in 49.1% of cases and by screening in the remaining 50.9%. The most common form of syphilis was late latent or indeterminate syphilis (41.9% of cases). In the group of men with syphilis, MSM had a higher risk of HIV infection. Conclusions A majority of MSM with syphilis had HIV coinfection. HIV screening is therefore essential in this population and could even result in early diagnosis.

Polyserositis (PS) is the inflammation, with effusion, of different serous membranes. It has been associated with different aetiologies, but the aetiology of PS remains unknown in a high percentage of patients.The general objective of this retrospective study was to analyse the aetiology of PS cases seen at Son Llàtzer Hospital in an 11-year period. Other objectives were to determine the epidemiological, clinical and analytical characteristics of these patients.An observational, descriptive and retrospective study to analyse the aetiology of PS cases seen at Son Llàtzer Hospital. The inflammation of two or more serous membranes confirmed by an imaging test was required and at least one of the serous fluid should be an exudate.A total of 92 patients was included in the study. The most common diagnosis was neoplasm (nearly one-third of cases) followed by infectious and autoimmune diseases. PS aetiology was unknown in more than one-third. Pleura and pericardium were the most common sites of serosal involvement (83%). Antinuclear antibodies' positivity in serum and increased levels of adenosine deaminase in pleural effusion were significantly associated with a final diagnosis of autoimmune disease. Increased pleural lactate dehydrogenase levels were significantly associated with a final diagnosis of neoplasm. In 9 of 14 patients with a previous cancer, PS represented a recurrence of their cancer. Cases of unknown aetiology presented most frequently as pleural and pericardial involvement, and the majority resolved. In very few patients, an infectious aetiology could be proven.PS is a frequent clinical entity that is associated with different diseases and its diagnosis could be challenging, with a high rate of unknown aetiologies.

Resumen Introduccion La tuberculosis (TB) continua siendo una enfermedad muy prevalente aunque desde el ano 2002 el numero de casos anuales muestra una tendencia decreciente en el mundo y tambien en nuestro pais, donde la incidencia es muy variable entre comunidades autonomas. El objetivo principal de este estudio es describir la experiencia de una unidad monografica de TB de un centro hospitalario de segundo nivel. Pacientes y metodos Estudio descriptivo de los casos de TB diagnosticados en una unidad monografica de un hospital secundario entre 2003 y 2011. Se recogieron datos demograficos, clinicos, epidemiologicos y microbiologicos para su analisis. Resultados Se analizaron 500 casos de TB, encontrando una incidencia anual creciente en todos los subgrupos, incluyendo poblacion autoctona e inmigrante. La mayoria (63,8%) eran varones, con una mediana de edad de 36 anos (rango 8 meses-90 anos). Un 39,8% de los pacientes era inmigrante. En un 11% de los casos existia coinfeccion con el virus de la inmunodeficiencia humana. La localizacion fue pulmonar en el 63,8% de los casos. La letalidad global fue del 5,8% sin encontrar diferencias significativas entre subgrupos (incluyendo poblacion inmigrante y personas infectadas por el virus de la inmunodeficiencia humana). Conclusiones A pesar de la tendencia descendente global en cuanto al numero de casos de TB declarados, en nuestra serie esta es creciente en todos los subgrupos. La existencia de una unidad monografica de TB junto con un exhaustivo programa de estudio de contactos podria explicar este hallazgo.

a b s t r a c t Introduction: Tuberculosis (TB) remains a highly prevalent and potentially severe disease. However, since 2002 the annual incidence has been decreasing both worldwide and in Spain, where the incidence varies widely between regions. The main objective of this study is to describe the experience of a monographic TB unit in a second level hospital. Patients and methods: A descriptive study was carried out which included all cases of TB diagnosed in a monographic unit of a secondary hospital between 2003 and 2011. Demographic, clinical, epidemiological and microbiological data were recorded. Results: We analyzed 500 TB cases and found an increasing annual incidence in all subgroups, including native and immigrant populations. Most cases (63.8%) were male, with a median age of 36 years (range 8 months-90 years). In total, 39.8% of patients were foreign born. Coinfection with human immunode- ficiency virus (HIV) was found in 11.0% of cases. The pulmonary form was most frequently diagnosed (63.8%). Overall mortality was 5.8% with no significant differences between groups (including foreign born and HIV positive patients). Conclusions: Although TB incidence is globally decreasing, in our study we found an increasing number of cases in recent years in all subgroups, which can be explained by this being a monographic unit with an intensive contact tracing program.

Background Cardiac resynchronization therapy (CRT) has benefits on left ventricle (LV) performance, but its mid-term effects on LV load and LV-arterial coupling are unknown. Aims To evaluate CRT mid-term effects on LV-arterial coupling, arterial load and its determinants, and the association between CRT-dependent aortic haemodynamic changes and the arterial biomechanics. Methods and results Cardiac and aortic echographies were done in 25 patients (age: 61 ± 12 years; 14 men; New York Heart Association functional classes III–IV; LV ejection fraction = 28 ± 7%, QRS = 139 ± 20 ms) before and after (23 ± 12 days) CRT. Standard structural and functional parameters and dyssynchrony indices were evaluated. Ascending aorta flow and diameter waveforms were measured. Central pressure was derived using a transfer function and the diameter calibration method. Calculus : arterial elastance ( E A); aortic impedance (Zc) and distensibility (AD); systemic resistances (SVR), total compliance ( C T); global reflection coefficient; LV end-systolic elastance ( E ES); and LV-arterial coupling ( E A/ E ES). After CRT E A diminished (−30%; P = 0.001), E ES increased (29%; P = 0.001) and E A/ E ES improved (pre-CRT: 2.9 ± 0.9, post-CRT: 1.6 ± 0.7; P = 0.001). Arterial elastance changes were associated with changes in arterial properties. Cardiac resynchronization therapy was associated with pressure-independent increase in mean aortic diameter (pre-CRT: 30.0 ± 4.0 mm, post-CRT: 33.0 ± 5.1 mm; P = 0.005) and distensibility (pre-CRT: 3.8 ± 2.6 × 10−3mmHg−1, post-CRT: 6.4 ± 2.5 × 10–3 mmHg−1; P = 0.002), and Zc reduction (pre-CRT: 3.5 ± 1.8 × 10−2mmHg.s/mL, post-CRT:1.9 ± 0.8 × 10−2 mmHg.s/mL; P = 0.001) and SVR (pre-CRT:1.7 ± 0.4 mmHg.s/mL, post-CRT:1.0 ± 0.3 mmHg.s/mL; P = 0.001). Changes in E A determinants were associated with changes in aortic flow. Conclusion Early after CRT central and peripheral arterial biomechanics improved, determining a pressure-independent increase in aortic diameter and a reduction in arterial load. Left ventricular systolic performance and LV-arterial coupling were enhanced. Arterial biomechanical changes were associated with aortic flow changes.

Extensively pretreated subjects with virological failure (VF) may receive salvage regimens containing NRTIs with only residual or no activity. Once virological suppression is achieved, their contribution remains elusive. This was a multicentre, randomized, prospective study. Subjects with at least one prior VF, HIV-1 RNA < 50 copies/mL for a parts per thousand yen6 months and receiving a regimen with at least two active drugs (one of them a boosted PI) were randomized 1:1 to stop (experimental arm) or maintain (control arm) NRTIs. EudraCT: 2012-000198-21. Ninety subjects were randomized (experimental, naEuroS=aEuroS45; and control, naEuroS=aEuroS45). The mean age was 50 years, 80% were male, the mean CD4+ cell count was 542 cells/mm(3) and the median number of prior VFs was 3. Seventy-four subjects (82%) harboured the mutation M184V/I and the median number of thymidine-associated mutations was 3 (IQR: 0-4). In the experimental arm, thirty-two (71%) subjects removed one NRTI and 13 (29%) subjects removed two. Twenty-two of 45 (49%) discontinued tenofovir disoproxil fumarate. Forty-one of 45 (91.1%, experimental arm) and 44 of 45 (97.8%, control arm) had HIV-1 RNA < 50 copies/mL at 48 weeks (difference: -6.7%; 95% CI: -17.4, 4.1). In a post-hoc analysis allowing NRTI reintroduction, efficacy rates were 95.6% and 97.8%, respectively (difference: -2.2%; 95% CI: -7.2, 2.7). Rates of discontinuation at 48 weeks were 2% in both arms. One subject developed a late VF with resistance selection. In patients receiving a successful multidrug salvage regimen with at least two active drugs (one a boosted PI), the withdrawal of inactive NRTIs was safe, rates of VF were low and drug resistance was uncommon at 48 weeks in this small study. This strategy could potentially prevent long-term toxicities, reduce the number of drugs and reduce costs if non-inferiority was met in a fully powered trial.

Pyoderma gangrenosum (PG) is an uncommon, distinctive cutaneous ulceration which is usually idiopathic, but may be associated with many systemic disorders. The etiopathogenesis of PG is still not well understood. PG is part of the spectrum of the neutrophilic dermatoses and it has been proposed as a prototype of cutaneous autoinflammatory disease. PG usually has a good outcome under immunosuppressive treatment. Although PG has been associated with several systemic diseases, it has rarely been reported in association with systemic lupus erythematosus (SLE). In this article we report five cases of SLE-related PG and review the literature. Our findings support the possible relationship between active SLE and PG, although the mechanism remains unclear. Clinical manifestations, used treatments and outcomes of SLE-related PG do not differ from the described for the general population.

We read with interest Zanon et al. 's article1 published in the January 2009 issue, in which the left ventricle (LV)–arterial coupling was evaluated in heart failure patients, as the ratio between the arterial elastance (Ea) and the LV end-systolic elastance (Ees), before and after 1-year of cardiac resynchronization therapy (CRT). The authors found that an increased basal Ea/Ees quotient (afterload mismatch) would determine an increased probability to respond to CRT, when the response was evaluated using the quality-of-life (QoL) score or the LV end-systolic volume (ESV). Based in these results, the authors suggested that ‘the non-invasive quantification of the Ea/Ees may be proposed as an immediate, easy, and optimal tool for quantifying the effect of CRT in patients with heart failure’. The authors said, the Ea/Ees quotient ‘could be useful to select responders to this electrical therapy and to assess the cardiac performance over the time’. We agree with the authors in the Ea/Ees usefulness in the assessment of the cardiac performance. However, we have some questions and comments that we think should be considered at the time of proposing the use of the Ea/Es ratio as a tool to predict the response to CRT and should be taken into account when analysing the Zanon et al. work.1 The authors suggested that the Ea/Ees determination could be useful to quantify the CRT effects and to assess the cardiac performance over time. Accordingly, patients response to CRT was analysed as a function of the basal …

The cardiac resynchronization therapy (CRT) effects on the arterial load components, the mechanisms (i.e. haemodynamic changes-dependence) involved in the load reduction and the factors (i.e. basal load conditions) associated with the load changes after CRT, are to be evaluated. Aims: a) to analyze the potential changes in the arterial load components (peripheral resistances, arterial compliance and impedance) associated with the CRT, b) to determine if the load components changes are associated with variations in haemodynamic variables (pressure, heart rate or blood flow), c) to analyze the relationship between the load components basal state and their changes after CRT. To fulfill these aims cardiac and arterial structural and mechanical parameters were non-invasively evaluated in 8 heart failure patients, pre- and post-CRT (23±8 days). The main results were that short-term after CRT: 1) there were changes in the static and dynamic determinants of the arterial load; 2) the changes in the load components were not associated with heart rate or pressure variations, but with blood flow changes, and 3) the load components basal levels and their changes after CRT were associated.