Standardized incidence ratios and risk factors for cancer in patients with systemic sclerosis: Data from the Spanish Scleroderma Registry (RESCLE).

AIM: Patients with systemic sclerosis (SSc) are at increased risk of cancer, a growing cause of non-SSc-related death among these patients. We analyzed the increased cancer risk among Spanish patients with SSc using standardized incidence ratios (SIRs) and identified independent cancer risk factors in this population. MATERIAL AND METHODS: Spanish Scleroderma Registry data were analyzed to determine the demographic characteristics of patients with SSc, and logistic regression was used to identify cancer risk factors. SIRs with 95% confidence intervals (CIs) relative to the general Spanish population were calculated. RESULTS: Of 1930 patients with SSc, 206 had cancer, most commonly breast, lung, hematological, and colorectal cancers. Patients with SSc had increased risks of overall cancer (SIR 1.48, 95% CI 1.36-1.60; P < 0.001), and of lung (SIR 2.22, 95% CI 1.77-2.73; P < 0.001), breast (SIR 1.31, 95% CI 1.10-1.54; P = 0.003), and hematological (SIR 2.03, 95% CI 1.52-2.62; P < 0.001) cancers. Cancer was associated with older age at SSc onset (odds ratio [OR] 1.22, 95% CI 1.01-1.03; P < 0.001), the presence of primary biliary cholangitis (OR 2.35, 95% CI 1.18-4.68; P = 0.015) and forced vital capacity <70% (OR 1.8, 95% CI 1.24-2.70; P = 0.002). The presence of anticentromere antibodies lowered the risk of cancer (OR 0.66, 95% CI 0.45-0.97; P = 0.036). CONCLUSIONS: Spanish patients with SSc had an increased cancer risk compared with the general population. Some characteristics, including specific autoantibodies, may be related to this increased risk.

Left ventricular diastolic dysfunction in systemic sclerosis: Clinical, immunological and survival differences in the Spanish RESCLE registry.

OBJECTIVES: Left ventricular diastolic dysfunction (LVDD) remains poorly studied in Systemic Sclerosis (SSc). To determine the prevalence and to define factors associated with LVDD and survival in a large cohort of patients with SSc. METHODS: An observational study was conducted with data from the multicentre Spanish Scleroderma Registry (RESCLE) to identify factors associated with LVDD and estimate survival. RESULTS: Out of 1517 patients, 319 (21.0%) had LVDD. The subset of sine scleroderma SSc was associated to LVDD (14.7% vs. 10.6%, p =0.048), whilst diffuse cutaneous SSc was more prevalent in non-LVDD (16.0 % vs. 21.2%, p =0.041). Multivariable analysis identified that LVDD was associated with older age at diagnosis of SSc (OR 1.05; 95% CI 1.04 to 1.06), longer time from diagnosis (OR 1.04; 95% CI 1.03 to 1.06), presence of telangiectasia (OR 1.42; 95% CI 1.08 to 1.88), treatment with calcium channel blockers (CCB) (OR 1.51; 95% CI 1.16 to 1.96), and inversely related to angiotensin-converting-enzyme inhibitors (ACEi) use (OR 0.59; 95% CI 0.44 to 0.80). SSc patients with LVDD had increased mortality (23.8 vs. 17.4%, p =0.010) and shortened survival from the first SSc symptom (p =0.040), even though it was not found to be an independent risk factor for death. CONCLUSIONS: LVDD is relatively common in SSc patients, and it is associated with worst prognosis, older age, longer time from diagnosis of SSc, presence of telangiectasia and vasodilator treatment.

Evaluación holística de las necesidades paliativas de los pacientes con insuficiencia cardiaca avanzada / Holistic evaluation of palliative needs in patients with advanced heart failure

Antecedentes y objetivo: Los pacientes con insuficiencia cardiaca (IC) presentan síntomas y preocupaciones a nivel físico, emocional, espiritual e intelectual, sobre todo en fases avanzadas de la enfermedad. En muchas ocasiones no son conocidas por el personal sanitario que les atiende y esto puede influir en las decisiones sobre los cuidados al final de la vida. El objetivo de este estudio es recoger información sobre estas necesidades y preferencias, y la valoración médica recibida para intentar abordarlas. Materiales y métodos: Estudio descriptivo observacional transversal basado en una entrevista estructurada, utilizando preguntas cerradas y cuestionarios validados en español. Se incluyeron 32 pacientes con IC avanzada ingresados en Medicina Interna por descompensación de su IC. Resultados: Los síntomas físicos más frecuentes fueron insomnio (75 %), cansancio (50 %) y disminución del bienestar (50 %). Presentaban ansiedad 9 pacientes (28,1 %), depresión 6 (18,8 %) y ambos síntomas 7 (21,8 %). Un total de 30 pacientes (93,8 %) no habían tratado temas espirituales con su médico. Deseaban que se realizara reanimación cardiopulmonar en caso de parada cardiorrespiratoria 24 pacientes (75 %), aunque solo uno había tratado este tema en el ingreso. No habían recibido información sobre la evolución y el pronóstico de su enfermedad 26 pacientes (81,3 %) y 21 (65,5 %) deseaban que se les informara sobre la fase terminal de su enfermedad. Conclusiones: La valoración integral del paciente con IC avanzada debe formar parte de la asistencia médica recibida. Profundizar en la comunicación médico-paciente para conocer sus necesidades y preferencias permitirá una adecuada planificación de los cuidados al final de la vida. (AU)

Background and objectives: Patients with heart failure (HF) have physical and emotional symptoms, spiritual and intellectual concerns, especially in end-stage HF. Frequently, these needs are unknown by health care professionals attending to these patients. This could influence decisions about end-of-life care. Our objective was to assess the information about needs and preferences in these patients, and the medical care received to manage them. Material and methods: This was a descriptive, observational, cross-sectional study using a survey with different questions and validated Spanish questionnaires. We interviewed 32 patients with advanced HF who were admitted to the internal medicine department due to acute decompensation of HF. Results: Most frequent physical symptoms were insomnia (75 %), tiredness (50 %) and decreased feeling of well-being (50 %). Anxiety was present in 9 patients (28.1 %), depression in 6 (18.8 %), and both symptoms in 7 (21.8 %). A total of 30 patients (93.8 %) had not talked about their spiritual needs with their doctor. Twenty-four (75 %) patients expressed a wish to have cardiopulmonary resuscitation if needed, although only one had talked about this during admission. Twentysix patients (81.3 %) had not received any information about their evolution and prognosis of HF. Twenty-one patients (65.5 %) expressed a wish to receive information about end-stage HF. Conclusions: An integral evaluation of patients with advanced HF must be part of their medical care. Early doctor-patient communication regarding the needs and preferences of these patients will enable an adequate planning of end-of-life care. (AU)

Podocitopatía como inicio de un lupus eritematoso sistémico / Podocytopathy as debut of a systemic lupus erythematosus

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Ictiosis adquirida paraneoplásica en adenocarcinoma de pulmón / Paraneoplastic acquired ichthyosis in lung cancer

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