Cut-offs and response criteria for the Hospital Universitario la Princesa Index (HUPI) and their comparison to widely-used indices of disease activity in rheumatoid arthritis

Objective To estimate cut-off points and to establish response criteria for the Hospital Universitario La Princesa Index (HUPI) in patients with chronic polyarthritis. Methods Two cohorts, one of early arthritis (Princesa Early Arthritis Register Longitudinal PEARL] study) and other of long-term rheumatoid arthritis (Estudio de la Morbilidad y Expresión Clínica de la Artritis Reumatoide EMECAR]) including altogether 1200 patients were used to determine cut-off values for remission, and for low, moderate and high activity through receiver operating curve (ROC) analysis. The areas under ROC (AUC) were compared to those of validated indexes (SDAI, CDAI, DAS28). ROC analysis was also applied to establish minimal and relevant clinical improvement for HUPI. Results The best cut-off points for HUPI are 2, 5 and 9, classifying RA activity as remission if =2, low disease activity if >2 and =5), moderate if >5 and <9 and high if =9. HUPI''s AUC to discriminate between low-moderate activity was 0.909 and between moderate-high activity 0.887. DAS28''s AUCs were 0.887 and 0.846, respectively; both indices had higher accuracy than SDAI (AUCs: 0.832 and 0.756) and CDAI (AUCs: 0.789 and 0.728). HUPI discriminates remission better than DAS28-ESR in early arthritis, but similarly to SDAI. The HUPI cut-off for minimal clinical improvement was established at 2 and for relevant clinical improvement at 4. Response criteria were established based on these cut-off values. Conclusions The cut-offs proposed for HUPI perform adequately in patients with either early or long term arthritis

Demographic, clinical and antibody characteristics of patients with digital ulcers in systemic sclerosis: data from the DUO Registry

OBJECTIVES: The Digital Ulcers Outcome (DUO) Registry was designed to describe the clinical and antibody characteristics, disease course and outcomes of patients with digital ulcers associated with systemic sclerosis (SSc). METHODS: The DUO Registry is a European, prospective, multicentre, observational, registry of SSc patients with ongoing digital ulcer disease, irrespective of treatment regimen. Data collected included demographics, SSc duration, SSc subset, internal organ manifestations, autoantibodies, previous and ongoing interventions and complications related to digital ulcers. RESULTS: Up to 19 November 2010 a total of 2439 patients had enrolled into the registry. Most were classified as either limited cutaneous SSc (lcSSc; 52.2%) or diffuse cutaneous SSc (dcSSc; 36.9%). Digital ulcers developed earlier in patients with dcSSc compared with lcSSc. Almost all patients (95.7%) tested positive for antinuclear antibodies, 45.2% for anti-scleroderma-70 and 43.6% for anticentromere antibodies (ACA). The first digital ulcer in the anti-scleroderma-70-positive patient cohort occurred approximately 5 years earlier than the ACA-positive patient group. CONCLUSIONS: This study provides data from a large cohort of SSc patients with a history of digital ulcers. The early occurrence and high frequency of digital ulcer complications are especially seen in patients with dcSSc and/or anti-scleroderma-70 antibodies

Acidosis láctica: algunas consideraciones

La hiperlactacidemia significa clínicamente problemas para los pacientes. La acidosis láctica es un trastorno ácido-básico consecutivo a la acumulación del ácido láctico, el cual se comporta en el nivel celular, como la contrapartida reducida del ácido pirúvico. Este último, resulta de la degradación de la glucosa en el citosol, proceso que se realiza de manera anaoeróbica y que puede culminar en CO2 H2O si sigue la vía del ácido cítrico de Krebs. El diagnóstico de esta entidad se confirma al medir la concentración sanguínea del lactato, aunque existen diversas características clínicas y de laboratorio que dan indicios de la existencia de este trastorno. Las causas de acidosis láctica se dividen en las producidas por hipoxia hística (tipo A) y las no producidas por este trastorno (tipo B); dentro de estas últimas se sitúan las debidas a alteraciones sistémicas, al uso de fármacos o toxinas y a las que acompañan a errores innatos del metabolismo.<br>Hyperlactatemia is a clinical problem for patients. The lactic acidosis is an acid-base disorder following the builup of lactic acid which at cellular level hehaves as a reduced counterpart of the pyruvic acid. The latter results from the degradation of glucose into citosol, a process that is anaerobically carried out and may end up in CO2 H2O if it takes the Krebs? citric acid route. The diagnosis sof this entity is confirmed by measuring blood lactate concentration although there are several clinical and lab characteristics that demonstrate the existance of this disorder. The causes of lactic acidosis are divided into those caused by hystic hypoxia (type A) and those not caused by this disorder (type B) such as lactic acidosis due to systemic disorders, use of drugs or toxins and acidosis resulting from inborn metabolic errors

Nuevas consideraciones fisiopatológicas sobre el síndrome de respuesta inflamatoria sistémica relacionada con la sepsis

El shock séptico constituye una de las primeras causas de morbilidad y mortalidad en las unidades de cuidados intensivos y forma parte de los estadios evolutivos la respuesta inflamatoria sistémica (SRIS) en relación con la infección, que fueron modificados por Jaffari para su aplicación en la edad pediátrica. Está relacionado con la exposición del organismo a agentes exógenos y/o mediadores endógenos, que producen una inadecuada perfusión hística con alteración en el consumo de oxígeno, en la permeabilidad vascular y la aparición de trastornos hemodinámicos que comprometen la vida del paciente. Se considera que el mediador central en el shock séptico es el factor de necrosis tumoral-a(FNT- a), aunque también responden de forma descontrolada otras citocinas, los polimorfonucleares, el sistema de contacto, el fibrinolítico, el de la coagulación, así como otras sustancias endógenas. El conocimiento de su fisiopatología hace posible el diagnóstico y tratamiento precoz, sobre todo en los grupos de riesgo, y la reducción de la mortalidad en las unidades de terapia intensiva por esta afección.<br>The septic shock is one of the first causes of morbidity and mortality in intensive care units, and the systemic inflammatory response syndrome (SIRS) is a part of the evolutive stages related to the infection, that were modified by Jaffari for its application in the pediatric age. Septic shock is related with the organism exposition to exogenous agents, and/or endogenous mediators that produce an inadequate histic perfusion with alteration in oxygen consumption, in vascular permeability, and the presentation of hemodynamic disorders that compromise the patient's life. It is considered that the main mediator in septic shock is the tumoral necrosis factor - (TBF- ), although other cytosines also reply in an uncontrolled fashion, the polymorphonuclears, the contact system, the fibrinolytic, the coagulation one, as well as other endogenous substances. Knowing the physiopathology of septic shock renders possible the diagnosis and an early treatment, specially in the risk groups, and the decrease of the mortality by this affection in the intensive care units

Factores de riesgo asociados a la mortalidad por enterocolitis necrotizante

La enterocolitis necrotizante es una urgencia gastrointestinal de causa multifactorial muy relacionada con el neonato pretérmino. Su elevada mortalidad radica en la falta de prevención por el médico a cualquier nivel de atención y a su diagnóstico tardío en los grupos de riesgo. Se realizó un estudio retrospectivo de los 63 pacientes fallecidos por enterocolitis necrotizante durante un período de 25 años en el Hospital Pediátrico Docente de Centro Habana, donde se encontró que el 71,4 % de los afectados era de la raza blanca y el 68,2 % del sexo masculino. La edad más frecuente se encontró en los menores de 3 meses de edad (36,5 %) y el 46 % del total de la muestra estudiada tuvo un peso al nacer inferior a los 1 500 g. La prematuridad apareció asociada en el 55,5 % de los fallecidos y el 65 % tuvo lactancia mixta desde el momento de nacimiento.<br>Necrotizing enterocolitis is a gastrointestinal urgency of multifactorial cause taht is closely connected with the preterm neonatus. Its high mortality results from the lack of prevention on the part of the physician at any level of attention and from its late diagnosis in the risk groups. A retrospective study of 63 patients who died of necrotizing enterocolitis during a period of 25 years at the Pediatric Teaching Hospital of Central Havana was conducted. It was found that 71.4 % of the affected were white and 68.2 % were males. It was more frequent among those under 3 months (36.5 %). 46 % of the total of the sample studied had a birth weight of less than 1 500 g. Prematurity appeared associated in 55 % of the dead, whereas 65 % had a mixed lactation since their birth

Tendências da mortalidade neonatal em São Luís, Maranhão, Brasil, de 1979 a 1996 Neonatal mortality trends in São Luís, Maranhão, Brazil, from 1979 to 1996

O propósito do presente trabalho é avaliar a evolução da mortalidade neonatal em São Luís nos últimos 18 anos, classificá-la de acordo com os dias de vida e pelo critério de evitabilidade de óbitos da Fundação SEADE, a partir de dados do IBGE e do Ministério da Saúde. Detectou-se aumento da mortalidade neonatal, às custas de aumento expressivo do seu componente precoce, especialmente pelas causas reduzíveis por diagnóstico e tratamento precoce, e parcialmente reduzíveis por adequado controle da gravidez. A mortalidade infantil, desse modo, manteve-se inalterada, apesar do decréscimo do seu componente pós-neonatal. O aumento expressivo no coeficiente de mortalidade neonatal a partir de 1995 aponta para a queda na qualidade da assistência obstétrica e neonatal, talvez motivada pelo elevado percentual de cesáreas e pela superlotação dos berçários. A tendência de estabilidade ou aumento da mortalidade neonatal é semelhante à observada recentemente no Brasil como um todo e difere da observada em outras cidades brasileiras, nas quais foi descrita queda lenta, mas persistente, da mortalidade neonatal, em oposição a uma redução mais dramática em países desenvolvidos.<br>This study examined neonatal mortality trends in São Luís in the last 18 years. The early and late components were assessed and causes were classified according to SEADE Foundation criteria based on reducibility of deaths and timing of prevention (during prenatal care, childbirth, or neonatal care). Data were derived from official live birth and death records. We detected an unexpected increase in the neonatal mortality rate, due primarily to a steep rise in early neonatal deaths. Causes reducible by early diagnosis and treatment (other specific infections and other neonatal respiratory causes) and those partially reducible by adequate monitoring of pregnancy (preterm births, low birth weight, and respiratory distress syndrome) showed the largest increase. Conversely, the post-neonatal mortality rate fell. The infant mortality rate remained the same, reflecting these antagonistic trends. The important rise in the neonatal mortality rate from 1995 onwards suggests a deterioration in the quality of obstetric and neonatal services. The high cesarean rate and overcrowded neonatal services (i.e., unable to cope with increasing demands foe specialized neonatal care) indicate the urgent need for restructuring the mother and child health care system

Outcomes of COVID-19 in patients with primary systemic vasculitis or polymyalgia rheumatica from the COVID-19 Global Rheumatology Alliance physician registry: a retrospective cohort study

Background: Patients with primary systemic vasculitis or polymyalgia rheumatica might be at a high risk for poor COVID-19 outcomes due to the treatments used, the potential organ damage cause by primary systemic vasculitis, and the demographic factors associated with these conditions. We therefore aimed to investigate factors associated with COVID-19 outcomes in patients with primary systemic vasculitis or polymyalgia rheumatica. Methods: In this retrospective cohort study, adult patients (aged ≥18 years) diagnosed with COVID-19 between March 12, 2020, and April 12, 2021, who had a history of primary systemic vasculitis (antineutrophil cytoplasmic antibody [ANCA]-associated vasculitis, giant cell arteritis, Behçet's syndrome, or other vasculitis) or polymyalgia rheumatica, and were reported to the COVID-19 Global Rheumatology Alliance registry were included. To assess COVID-19 outcomes in patients, we used an ordinal COVID-19 severity scale, defined as: (1) no hospitalisation; (2) hospitalisation without supplemental oxygen; (3) hospitalisation with any supplemental oxygen or ventilation; or (4) death. Multivariable ordinal logistic regression analyses were used to estimate odds ratios (ORs), adjusting for age, sex, time period, number of comorbidities, smoking status, obesity, glucocorticoid use, disease activity, region, and medication category. Analyses were also stratified by type of rheumatic disease. Findings: Of 1202 eligible patients identified in the registry, 733 (61·0%) were women and 469 (39·0%) were men, and their mean age was 63·8 years (SD 17·1). A total of 374 (31·1%) patients had polymyalgia rheumatica, 353 (29·4%) had ANCA-associated vasculitis, 183 (15·2%) had giant cell arteritis, 112 (9·3%) had Behçet's syndrome, and 180 (15·0%) had other vasculitis. Of 1020 (84·9%) patients with outcome data, 512 (50·2%) were not hospitalised, 114 (11·2%) were hospitalised and did not receive supplemental oxygen, 239 (23·4%) were hospitalised and received ventilation or supplemental oxygen, and 155 (15·2%) died. A higher odds of poor COVID-19 outcomes were observed in patients who were older (per each additional decade of life OR 1·44 [95% CI 1·31–1·57]), were male compared with female (1·38 [1·05–1·80]), had more comorbidities (per each additional comorbidity 1·39 [1·23–1·58]), were taking 10 mg/day or more of prednisolone compared with none (2·14 [1·50–3·04]), or had moderate, or high or severe disease activity compared with those who had disease remission or low disease activity (2·12 [1·49–3·02]). Risk factors varied among different disease subtypes. Interpretation: Among patients with primary systemic vasculitis and polymyalgia rheumatica, severe COVID-19 outcomes were associated with variable and largely unmodifiable risk factors, such as age, sex, and number of comorbidities, as well as treatments, including high-dose glucocorticoids. Our results could be used to inform mitigation strategies for patients with these diseases. Funding: American College of Rheumatology and the European Alliance of Associations for Rheumatology

The comparative responsiveness of Hospital Universitario Princesa Index and other composite indices for assessing rheumatoid arthritis activity

Objective To evaluate the responsiveness in terms of correlation of the Hospital Universitario La Princesa Index (HUPI) comparatively to the traditional composite indices used to assess disease activity in rheumatoid arthritis (RA), and to compare the performance of HUPI-based response criteria with that of the EULAR response criteria. Methods Secondary data analysis from the following studies: ACT-RAY (clinical trial), PROAR (early RA cohort) and EMECAR (pre-biologic era long term RA cohort). Responsiveness was evaluated by: 1) comparing change from baseline (Delta) of HUPI with Delta in other scores by calculating correlation coefficients; 2) calculating standardised effect sizes. The accuracy of response by HUPI and by EULAR criteria was analyzed using linear regressions in which the dependent variable was change in global assessment by physician (Delta GDA-Phy). Results Delta HUPI correlation with change in all other indices ranged from 0.387 to 0.791); HUPI's standardized effect size was larger than those from the other indices in each database used. In ACT-RAY, depending on visit, between 65 and 80% of patients were equally classified by HUPI and EULAR response criteria. However, HUPI criteria were slightly more stringent, with higher percentage of patients classified as non-responder, especially at early visits. HUPI response criteria showed a slightly higher accuracy than EULAR response criteria when using Delta GDA-Phy as gold standard. Conclusion HUPI shows good responsiveness in terms of correlation in each studied scenario (clinical trial, early RA cohort, and established RA cohort). Response criteria by HUPI seem more stringent than EULAR's