ABSTRACT
Background: Cardiovascular disease (CVD) is one of the principal causes of death in antineutrophil cytoplasmic antibody-(ANCA)-associated vasculitis (AAV). Objectives: To evaluate the mortality and it's causes and CVD and its vascular risk factors (VRFs) in AAV patients in Andalusia. Methods: A multicenter cohort of 220 AAV patients followed-up from 1979 until June 2020 was studied in Andalussia, south of Spain. The information, including socio-demographic and clinical data was recorded retrospectively through chart review. Data was analysed using Chi2, ANOVA and Cox proportional hazards regresion as uni and multivariate test with a 95% confidence interval (CI). Results: During a mean ± standard deviation follow-up of 96.79 ± 75.83 months, 51 patients died and 30 presented at least one CVE. Independent prognostic factors of mortality were age (HR 1.083, p=0.001) and baseline creatinine (HR 4.41, p=0.01). Independent prognostic factors of CVE were age [hazard ratio (HR) 1.042, p=0.005] and the presence of hypertension (HTN) six months after diagnosis (HR 4.641, p=0.01). HTN, diabetes and renal failure, all of these important VRFs, are more prevalent in AAV patients than it is described in matched general population. Conclusions: Age and baseline renal function, but not CVEs, are predictors of mortality and age and early HTN are independent predictors for having a CVE. CVD screening in AAV patients is demanded.(AU)
Introducción: La enfermedad cardiovascular (ECV) es una de las principales causas de muerte en las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA) (VAA). Objetivos: Evaluar la mortalidad y sus causas, entre ellas la ECV y sus factores de riesgo vascular (FRV) en pacientes con VAA en Andalucía. Métodos: Se estudió una cohorte multicéntrica de 220 pacientes con VAA seguidos desde 1979 hasta junio de 2020 en Andalucía. La información, incluidos los datos sociodemográficos y clínicos, se registró retrospectivamente a través de la revisión de historias clínicas. Los datos se analizaron mediante Chi2, ANOVA y regresión de riesgos proporcionales de Cox de forma uni y multivariante con un intervalo de confianza (IC) del 95%. Resultados: Durante un seguimiento medio y desviación estándar de 96,79 ± 75,83 meses, 51 pacientes fallecieron y 30 presentaron al menos un ECV. Los factores pronósticos independientes de mortalidad fueron la edad (HR 1,083, p=0,001) y la creatinina basal (HR 4,41, p=0,01). Los factores pronósticos independientes de ECV fueron la edad [hazard ratio (HR) 1,042, p=0,005] y la presencia de hipertensión arterial (HTA) seis meses después del diagnóstico (HR 4,641, p=0,01). La prevalencia de HTA, diabetes e insuficiencia renal fue elevada o muy elevada en comparación con la población general emparentada, todos FRCV determinantes para el pronóstico de estos pacientes. Conclusiones: La edad y la función renal basal son predictores de mortalidad y la edad y la HTA de aparición precoz son predictores independientes de tener ECV. Se recomienda el cribado de FRCV en pacientes con vasculitis ANCA.(AU)
Subject(s)
Humans , Male , Female , Cardiovascular Diseases/mortality , Hypertension , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Spain , Cohort Studies , Risk FactorsABSTRACT
BACKGROUND: Cardiovascular disease (CVD) is one of the principal causes of death in antineutrophil cytoplasmic antibody-(ANCA)-associated vasculitis (AAV). OBJECTIVES: To evaluate the mortality and it's causes and CVD and its vascular risk factors (VRFs) in AAV patients in Andalusia. METHODS: A multicenter cohort of 220 AAV patients followed-up from 1979 until June 2020 was studied in Andalussia, south of Spain. The information, including socio-demographic and clinical data was recorded retrospectively through chart review. Data was analysed using Chi2, ANOVA and Cox proportional hazards regresion as uni and multivariate test with a 95% confidence interval (CI). RESULTS: During a mean ± standard deviation follow-up of 96.79 ± 75.83 months, 51 patients died and 30 presented at least one CVE. Independent prognostic factors of mortality were age (HR 1.083, p=0.001) and baseline creatinine (HR 4.41, p=0.01). Independent prognostic factors of CVE were age [hazard ratio (HR) 1.042, p=0.005] and the presence of hypertension (HTN) six months after diagnosis (HR 4.641, p=0.01). HTN, diabetes and renal failure, all of these important VRFs, are more prevalent in AAV patients than it is described in matched general population. CONCLUSIONS: Age and baseline renal function, but not CVEs, are predictors of mortality and age and early HTN are independent predictors for having a CVE. CVD screening in AAV patients is demanded.
ABSTRACT
Introducción El efecto de la dexametasona en la fase inicial de la infección por SARS-CoV-2 y su influencia sobre la COVID-19 no está bien definido. Describimos las características clínico-radiológicas, los parámetros de tormenta de citoquinas y la evolución clínica de una serie de pacientes tratados con dexametasona en la fase inicial de la enfermedad. Método Estudio de 8 pacientes que recibieron dexametasona previo al desarrollo de la COVID-19. Evaluamos variables clínicas, pruebas de imagen, parámetros de liberación de citoquinas, tratamiento empleado y su evolución. Resultados Todos los pacientes recibieron una dosis de 6mg/día con una duración media de 4,5 días previos al ingreso. La mayoría de los pacientes presentaron una extensión grave en la tomografía computarizada de alta resolución (TCAR) y una elevación leve de los parámetros de liberación de citoquinas; 3 pacientes requirieron oxigenoterpia nasal de alto flujo (ONAF) por insuficiencia respiratoria, y ningún paciente requirió intubación orotraqueal ni falleció. Conclusión La dexametasona en las fases iniciales de la infección por SARS-CoV-2 parece asociarse con una COVID-19 grave (AU)
Introduction The effect of dexamethasone in the initial phase of infection by SARS-CoV-2 and its influence on COVID-19 is not well defined. We describe clinical-radiological characteristics, the cytokine storm parameters, and the clinical evolution of a series of patients treated with dexamethasone in the disease's initial phase. Method A study of 8 patients who received dexamethasone before the development of COVID-19. We evaluate clinical variables, imaging tests, cytokine release parameters, treatment used and patient evolution. Results All patients received a 6mg/day dose with a mean duration of 4.5 days before admission. High resolution computed tomography (HRCT) revealed that most of them presented a severe extension; most patients had a slightly elevated level of cytokine release parameters. Three patients required high-flow oxygen therapy due to respiratory failure; none required orotracheal intubation or died. Conclusion Dexamethasone in the early stages of SARS-CoV-2 infection appears to be associated with severe COVID-19 (AU)
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Dexamethasone/administration & dosage , Anti-Inflammatory Agents/administration & dosage , /drug therapy , Severity of Illness Index , Retrospective StudiesABSTRACT
INTRODUCTION: The effect of dexamethasone in the initial phase of infection by SARS-CoV-2 and its influence on COVID-19 is not well defined. We describe clinical-radiological characteristics, the cytokine storm parameters, and the clinical evolution of a series of patients treated with dexamethasone in the disease's initial phase. METHOD: A study of 8 patients who received dexamethasone before the development of COVID-19. We evaluate clinical variables, imaging tests, cytokine release parameters, treatment used and patient evolution. RESULTS: All patients received a 6 mg/day dose with a mean duration of 4.5 days before admission. High resolution computed tomography (HRCT) revealed that most of them presented a severe extension; most patients had a slightly elevated level of cytokine release parameters. Three patients required high-flow oxygen therapy due to respiratory failure; none required orotracheal intubation or died. CONCLUSION: Dexamethasone in the early stages of SARS-CoV-2 infection appears to be associated with severe COVID-19.
Subject(s)
COVID-19 Drug Treatment , Cytokine Release Syndrome , Dexamethasone , Humans , SARS-CoV-2ABSTRACT
INTRODUCTION: The effect of dexamethasone in the initial phase of infection by SARS-CoV-2 and its influence on COVID-19 is not well defined. We describe clinical-radiological characteristics, the cytokine storm parameters, and the clinical evolution of a series of patients treated with dexamethasone in the disease's initial phase. METHOD: A study of 8 patients who received dexamethasone before the development of COVID-19. We evaluate clinical variables, imaging tests, cytokine release parameters, treatment used and patient evolution. RESULTS: All patients received a 6 mg/day dose with a mean duration of 4.5 days before admission. High resolution computed tomography (HRCT) revealed that most of them presented a severe extension; most patients had a slightly elevated level of cytokine release parameters. Three patients required high-flow oxygen therapy due to respiratory failure; none required orotracheal intubation or died. CONCLUSION: Dexamethasone in the early stages of SARS-CoV-2 infection appears to be associated with severe COVID-19.
Subject(s)
Angiography , Early Diagnosis , Susac Syndrome/diagnostic imaging , Tomography, Optical Coherence , Adrenal Cortex Hormones/administration & dosage , Adult , Diagnosis, Differential , Female , Hearing Loss/etiology , Humans , Susac Syndrome/drug therapy , Susac Syndrome/pathology , Tinnitus/etiologyABSTRACT
Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, Pâ<â0.0001). The dcSSc subset, male sex, age at disease onset older than 65 years, digital ulcers, interstitial lung disease (ILD), PH, heart involvement, scleroderma renal crisis (SRC), presence of antitopoisomerase I and absence of anticentromere antibodies, and active capillaroscopic pattern showed reduced survival rate. In a multivariate analysis, older age at disease onset, dcSSc, ILD, PH, and SRC were independent risk factors for mortality. In the present study involving a large cohort of SSc patients, a high prevalence of disease-related causes of death was demonstrated. Older age at disease onset, dcSSc, ILD, PH, and SRC were identified as independent prognostic factors.
Subject(s)
Registries , Scleroderma, Systemic/mortality , Adult , Aged , Cause of Death , Female , Humans , Male , Middle Aged , Retrospective Studies , Risk Factors , Spain/epidemiologyABSTRACT
Antiphospholipid syndrome is considered a high risk factor for any kind of surgery. Considering that all solid organ transplants are critically dependent on the patency of vascular anastomosis, there is much concern about the consequences this pro-thrombotic condition may have on transplantation. Relatively little information is available in the literature assessing the real risk that antiphospholipid syndrome or the presence of antiphospholipid antibodies represent in solid organ transplantation. The aim of this article is to review the literature related to transplantation of solid organs in patients diagnosed with antiphospholipid syndrome or patients with positive antiphospholipid antibodies.
Subject(s)
Antibodies, Antiphospholipid/immunology , Antiphospholipid Syndrome/diagnosis , Organ Transplantation , Antibodies, Anticardiolipin/immunology , Antiphospholipid Syndrome/immunology , Antiphospholipid Syndrome/surgery , Female , Humans , Lupus Coagulation Inhibitor/immunology , Middle Aged , Risk Factors , Thrombophilia/diagnosis , Thrombophilia/etiology , Thrombophilia/therapy , Thrombosis/immunology , Thrombosis/physiopathology , Transplantation Immunology , Treatment OutcomeABSTRACT
OBJECTIVE: To analyze the role of the antiphospholipid autoantibodies (aPL) on the neuropsychological deficits in systemic lupus erythematosus (SLE) patients, comparing groups of patients with antiphospholipid syndrome (APS; n = 15), SLE with aPL (n = 12), and SLE without aPL (n = 27), and a healthy control group (n = 31). METHODS: Patients fulfilled the American College of Rheumatology SLE classification criteria or the Sydney criteria for APS. All participants were woman, and groups were matched on age and education. A standardized cognitive examination classified patients as cognitively declined or impaired according to the American College of Rheumatology. RESULTS: Differences between the groups were found in all of the studied variables, comprising attention and executive functions (sustained and selective attention, fluency, and inhibition), and memory (verbal and visual). Post-hoc analyses showed cognitive performance was equivalent between APS and SLE with aPL. Differences between SLE without aPL and control groups were found only in four of the 10 studied variables, while differences in all but two memory variables were found between SLE without aPL and control groups. Furthermore, cognitive deficit was three times more frequent in APS and SLE with aPL patients than for the control group (80%, 75%, and 16%, respectively), and two times more frequent compared to SLE patients without aPL (48%). CONCLUSIONS: Our results support the relationship between aPL and cognitive symptoms in SLE. Also, almost half of the patients with SLE and no aPL showed cognitive problems, pointing to the multifactorial causes of cognitive problems in SLE. Future research with larger sample size is guaranteed to replicate our results.
Subject(s)
Antibodies, Antiphospholipid/immunology , Antiphospholipid Syndrome/immunology , Cognition Disorders/epidemiology , Lupus Vasculitis, Central Nervous System/immunology , Lupus Vasculitis, Central Nervous System/psychology , Adult , Attention , Executive Function , Female , Humans , Memory , Middle AgedABSTRACT
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.
Subject(s)
Immunosuppressive Agents/therapeutic use , Lupus Erythematosus, Systemic/complications , Pyoderma Gangrenosum/etiology , Adult , Female , Humans , Male , Middle Aged , Pyoderma Gangrenosum/drug therapy , Pyoderma Gangrenosum/pathologyABSTRACT
Melanoma is the most lethal form of skin malignancy because of its aggressive behaviour. In advanced disease, interferon alfa can be used as adjuvant therapy. However, this therapy is not free of side effects. We present a case of severe Raynaud syndrome and digital necrosis induced by interferon alfa in a patient with melanoma. Pathogenic mechanisms are discussed.
