RESUMEN
BACKGROUND: Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) and post-COVID condition can present similarities such as fatigue, brain fog, autonomic and neuropathic symptoms. METHODS: The study included 87 patients with post-COVID condition, 50 patients with ME/CFS, and 50 healthy controls (HC). The hemodynamic autonomic function was evaluated using the deep breathing technique, Valsalva maneuver, and Tilt test. The presence of autonomic and sensory small fiber neuropathy (SFN) was assessed with the Sudoscan and with heat and cold evoked potentials, respectively. Finally, a complete neuropsychological evaluation was performed. The objective of this study was to analyze and compare the autonomic and neuropathic symptoms in post-COVID condition with ME/CFS, and HC, as well as, analyze the relationship of these symptoms with cognition and fatigue. RESULTS: Statistically significant differences were found between groups in heart rate using the Kruskal-Wallis test (H), with ME/CFS group presenting the highest (H = 18.3; p ≤ .001). The Postural Orthostatic Tachycardia Syndrome (POTS), and pathological values in palms on the Sudoscan were found in 31% and 34% of ME/CFS, and 13.8% and 19.5% of post-COVID patients, respectively. Concerning evoked potentials, statistically significant differences were found in response latency to heat stimuli between groups (H = 23.6; p ≤ .01). Latency was highest in ME/CFS, and lowest in HC. Regarding cognition, lower parasympathetic activation was associated with worse cognitive performance. CONCLUSIONS: Both syndromes were characterized by inappropriate tachycardia at rest, with a high percentage of patients with POTS. The prolonged latencies for heat stimuli suggested damage to unmyelinated fibers. The higher proportion of patients with pathological results for upper extremities on the Sudoscan suggested a non-length-dependent SFN.
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COVID-19 , Síndrome de Fatiga Crónica , Síndrome de Taquicardia Postural Ortostática , Neuropatía de Fibras Pequeñas , Humanos , Síndrome de Fatiga Crónica/diagnóstico , Síndrome Post Agudo de COVID-19 , COVID-19/complicaciones , Síndrome de Taquicardia Postural Ortostática/diagnósticoRESUMEN
Objectives: To analyze the characteristics and the predictive factors of the use of rituximab and belimumab in daily practice in patients from the inception cohort Registro Español de Lupus (RELES). Material and methods: The study included 518 patients. We considered patients treated with biologics who received at least one dose of rituximab or belimumab, and possible indications of those manifestations registered at the same time or in the previous 2 months of the start of the therapy. Results: In our cohort, 37 (7%) patients received at least one biological treatment. Rituximab was prescribed in 26 patients and belimumab in 11. Rituximab was mainly prescribed for hemolytic anemia or thrombocytopenia (11 patients, 42%), lupus nephritis and neuropsychiatric lupus (5 patients each, 19%). Belimumab was mostly used for arthritis (8 patients, 73%). In the univariate analysis, the predictive factors at diagnosis for the use of biologic therapy were younger age (p = 0.022), a higher SLEDAI (p = 0.001) and the presence of psychosis (p = 0.011), organic mental syndrome (SOCA) (p = 0.006), hemolytic anemia (p = 0.001), or thrombocytopenia (p = 0.01). In the multivariant model, only younger age, psychosis, and hemolytic anemia were independent predictors of the use of biologics. Conclusions: Rituximab is usually given to patients with hematological, neuropsychiatric and renal involvement and belimumab for arthritis. Psychosis, hemolytic anemia and age at the diagnosis of lupus were independent predictive factors of the use of biological agents. Their global effects are beneficial, with a significant reduction in SLE activity and a low rate of side effects.
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Artritis , Productos Biológicos , Trombocitopenia , Humanos , Rituximab/uso terapéuticoRESUMEN
BACKGROUND: Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) is characterized by persistent physical and mental fatigue. The post-COVID-19 condition patients refer physical fatigue and cognitive impairment sequelae. Given the similarity between both conditions, could it be the same pathology with a different precipitating factor? OBJECTIVE: To describe the cognitive impairment, neuropsychiatric symptoms, and general symptomatology in both groups, to find out if it is the same pathology. As well as verify if the affectation of smell is related to cognitive deterioration in patients with post-COVID-19 condition. METHODS: The sample included 42 ME/CFS and 73 post-COVID-19 condition patients. Fatigue, sleep quality, anxiety and depressive symptoms, the frequency and severity of different symptoms, olfactory function and a wide range of cognitive domains were evaluated. RESULTS: Both syndromes are characterized by excessive physical fatigue, sleep problems and myalgia. Sustained attention and processing speed were impaired in 83.3% and 52.4% of ME/CFS patients while in post-COVID-19 condition were impaired in 56.2% and 41.4% of patients, respectively. Statistically significant differences were found in sustained attention and visuospatial ability, being the ME/CFS group who presented the worst performance. Physical problems and mood issues were the main variables correlating with cognitive performance in post-COVID-19 patients, while in ME/CFS it was anxiety symptoms and physical fatigue. CONCLUSIONS: The symptomatology and cognitive patterns were similar in both groups, with greater impairment in ME/CFS. This disease is characterized by greater physical and neuropsychiatric problems compared to post-COVID-19 condition. Likewise, we also propose the relevance of prolonged hyposmia as a possible marker of cognitive deterioration in patients with post-COVID-19.
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COVID-19 , Síndrome de Fatiga Crónica , Humanos , Síndrome de Fatiga Crónica/complicaciones , COVID-19/complicaciones , Fatiga Mental , EncéfaloRESUMEN
OBJECTIVE: The objective of this report is to analyse retinal changes over a five-year period, assessed by spectral domain-optical coherence tomography (SD-OCT), in patients from the Lupus-Cruces cohort treated with hydroxychloroquine (HCQ). METHODS: SD-OCT screening was performed annually between 2012 and 2017. Average macular thickness (AMT), ganglion cell layer thickness (GCLT) and qualitative data of retinal pigment epithelium (RPE) and external retina (ExtR) were collected prospectively. We compared data from 2012 (first) and 2017 (second) SD-OCT. RESULTS: We studied 110 patients and 195 eyes. No cases of HCQ toxicity were detected. At the time of the second SD-OCT, 99% patients had taken a daily dose of HCQ ≤5 mg/kg/day. The median time on HCQ was 133 months. The mean AMT and GCLT were significantly lower in both eyes at the second SD-OCT; however, all the differences were clinically insignificant at less than 1%. Qualitative analysis of RPE and ExtR showed no significant changes. Similar results were found among patients with risk factors for retinopathy. The comparison of patients with and without risk factors showed no differences. CONCLUSIONS: This study shows clinically irrelevant retinal changes in an SLE cohort on HCQ treatment over a five-year follow-up. Our findings support the safety of long-term HCQ at doses ≤5 mg/kg/day.
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Antirreumáticos/efectos adversos , Hidroxicloroquina/efectos adversos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/patología , Retina/patología , Adulto , Anciano , Antirreumáticos/administración & dosificación , Estudios de Cohortes , Femenino , Estudios de Seguimiento , Hospitales Universitarios , Humanos , Hidroxicloroquina/administración & dosificación , Incidencia , Masculino , Persona de Mediana Edad , New Mexico/epidemiología , Estudios Prospectivos , Retina/efectos de los fármacos , Enfermedades de la Retina/inducido químicamente , Enfermedades de la Retina/patología , Estadísticas no Paramétricas , Tomografía de Coherencia Óptica , Población BlancaRESUMEN
Aim The aim of this study was to evaluate the clinical response to combined therapy with hydroxychloroquine and mepacrine in patients with systemic lupus erythematosus and refractory joint and/or skin disease. Methods Mepacrine was added to 46 systemic lupus erythematosus patients unresponsive to treatment with the following drug combinations: hydroxychloroquine + prednisone + immunosuppressive drugs ( n = 24), hydroxychloroquine + prednisone ( n = 16), hydroxychloroquine + prednisone + retinoids ( n = 2), hydroxychloroquine alone ( n = 1), hydroxychloroquine + one immunosuppressive drug ( n = 1), hydroxychloroquine + prednisone + one immunosuppressive drug + belimumab ( n = 1) or hydroxychloroquine + prednisone + belimumab ( n = 1). The outcome variable was the clinical response, either complete or partial, based on clinical judgement. The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score were additionally used. Results A total of 91% patients showed complete/partial response, with similar rates among those with joint or skin disease. In patients with cutaneous activity, a statistically significant decrease in the CLASI was seen. There also was a statistically significant decrease in the SLEDAI. The mean daily dose of prednisone decreased from 5.8 to 3.4 mg/d ( p = 0.001). Prednisone could be discontinued in 20% of patients. No serious adverse events were seen. Smoking was the only predictor of complete response. Conclusion In the setting of refractory skin and/or joint disease, the addition of mepacrine to previous therapy including hydroxychloroquine was safe and effective in reducing disease activity and decreasing prednisone doses. The fact that smokers responded better opens the door to further studying the combination of mepacrine-hydroxychloroquine as a first-line therapy in such patients.
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Antimaláricos/uso terapéutico , Hidroxicloroquina/uso terapéutico , Inmunosupresores/uso terapéutico , Artropatías/tratamiento farmacológico , Lupus Eritematoso Cutáneo/tratamiento farmacológico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Quinacrina/uso terapéutico , Adulto , Antimaláricos/efectos adversos , Quimioterapia Combinada , Femenino , Humanos , Hidroxicloroquina/efectos adversos , Inmunosupresores/efectos adversos , Artropatías/diagnóstico , Artropatías/inmunología , Lupus Eritematoso Cutáneo/diagnóstico , Lupus Eritematoso Cutáneo/inmunología , Lupus Eritematoso Discoide/diagnóstico , Lupus Eritematoso Discoide/tratamiento farmacológico , Lupus Eritematoso Discoide/inmunología , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/inmunología , Masculino , Persona de Mediana Edad , Quinacrina/efectos adversos , Inducción de Remisión , Estudios Retrospectivos , Fumadores , Resultado del TratamientoRESUMEN
OBJECTIVES: Using data of patients from the inception cohort Registro Español de Lupus Eritematoso Sistémico (RELES), we aimed to analyse the incidence of severe infection in the first two years of follow-up and how predictors of infection change during the course of systemic lupus erythematosus (SLE). MATERIAL AND METHODS: The study included 282 patients. Markers of lupus activity, prednisone doses and immunosuppressive therapy were compared between patients with and without infections in the first and second year of the disease. Drug therapy administered during the first month of follow-up has been considered as a potential predictor of infections during the first year and medications administered during the first year have been considered potential predictors of infections during the second. RESULTS: Nineteen patients (6.4%) had a documented episode of major infection during the first year of follow-up and 16 patients (5.67%) during the second. The following variables were associated with infections during the first year: hypocomplementaemia at diagnosis ( p < 0.01), nephritis at diagnosis ( p = 0.03), SLEDAI score ( p < 0.01), prednisone >30 mg/day ( p = 0.01), methylprednisolone pulses ( p = 0.05) and mycophenolate use ( p = 0.02). The independent variables in the final model were hypocomplementaemia (odds ratio (OR) 4.41, 95% confidence interval (CI) 0.96-20.20, p = 0.05) and a dose of prednisone >30 mg/day (OR 6.60, 95% CI 1.34-32.42, p = 0.02). The following variables were associated with infections during the second year: dose of prednisone > 7.5 mg/day ( p = 0.05), methylprednisolone pulses ( p = 0.07), duration of therapy with antimalarials ( p = 0.09), therapy with mycophenolate ( p = 0.01), therapy with cyclophosphamide ( p = 0.05). The independent variables in the final model were a dose of prednisone >7.5 mg/day (OR 4.52, 95% CI 0.99-21, p = 0.054) and duration of therapy with antimalarials as a protective factor (OR 0.99, 95% CI 0.99-1.00, p = 0.053). CONCLUSIONS: The low incidence of early infections in the RELES cohort is partially explained by the extended use of antimalarials and by the general avoidance of prolonged high doses of prednisone. Patients with high baseline activity are at a higher risk of infection during the first months but therapy with medium-high doses of prednisone is the main predictor of infectious events. Thus, every effort should be made to limit oral glucocorticoid use from the very beginning of the SLE course.
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Antimaláricos/uso terapéutico , Inmunosupresores/uso terapéutico , Infecciones/epidemiología , Lupus Eritematoso Sistémico/tratamiento farmacológico , Prednisona/administración & dosificación , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Estudios de Cohortes , Ciclofosfamida/uso terapéutico , Quimioterapia Combinada , Femenino , Humanos , Incidencia , Infecciones/clasificación , Modelos Logísticos , Lupus Eritematoso Sistémico/complicaciones , Masculino , Metilprednisolona/uso terapéutico , Persona de Mediana Edad , Análisis Multivariante , Índice de Severidad de la Enfermedad , España/epidemiología , Adulto JovenRESUMEN
Objective A task force of scientists at the International Congress on Antiphospholipid Antibodies recognized that phosphatidylserine-dependent antiprothrombin antibodies (aPS/PT) might contribute to a better identification of antiphospholipid syndrome (APS). Accordingly, initial and replication retrospective, cross-sectional multicentre studies were conducted to ascertain the value of aPS/PT for APS diagnosis. Methods In the initial study (eight centres, seven countries), clinical/laboratory data were retrospectively collected. Serum/plasma samples were tested for IgG aPS/PT at Inova Diagnostics (Inova) using two ELISA kits. A replication study (five centres, five countries) was carried out afterwards. Results In the initial study ( n = 247), a moderate agreement between the IgG aPS/PT Inova and MBL ELISA kits was observed ( k = 0.598). IgG aPS/PT were more prevalent in APS patients (51%) than in those without (9%), OR 10.8, 95% CI (4.0-29.3), p < 0.0001. Sensitivity, specificity, positive (LR+) and negative (LR-) likelihood ratio of IgG aPS/PT for APS diagnosis were 51%, 91%, 5.9 and 0.5, respectively. In the replication study ( n = 214), a moderate/substantial agreement between the IgG aPS/PT results obtained with both ELISA kits was observed ( k = 0.630). IgG aPS/PT were more prevalent in APS patients (47%) than in those without (12%), OR 6.4, 95% CI (2.6-16), p < 0.0001. Sensitivity, specificity, LR + and LR- for APS diagnosis were 47%, 88%, 3.9 and 0.6, respectively. Conclusions IgG aPS/PT detection is an easily performed laboratory parameter that might contribute to a better and more complete identification of patients with APS.
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Anticuerpos Antifosfolípidos/sangre , Síndrome Antifosfolípido/diagnóstico , Lupus Eritematoso Sistémico/complicaciones , Fosfatidilserinas/inmunología , Complicaciones del Embarazo/diagnóstico , Trombosis/diagnóstico , Adolescente , Adulto , Anciano , Síndrome Antifosfolípido/sangre , Estudios Transversales , Femenino , Humanos , Cooperación Internacional , Masculino , Persona de Mediana Edad , Embarazo , Complicaciones del Embarazo/sangre , Estudios Retrospectivos , Sensibilidad y Especificidad , Adulto JovenRESUMEN
Objective There is a decreased breast cancer risk in systemic lupus erythematosus (SLE) versus the general population. We assessed a large sample of SLE patients, evaluating demographic and clinical characteristics and breast cancer risk. Methods We performed case-cohort analyses within a multi-center international SLE sample. We calculated the breast cancer hazard ratio (HR) in female SLE patients, relative to demographics, reproductive history, family history of breast cancer, and time-dependent measures of anti-dsDNA positivity, cumulative disease activity, and drugs, adjusted for SLE duration. Results There were 86 SLE breast cancers and 4498 female SLE cancer-free controls. Patients were followed on average for 7.6 years. Versus controls, SLE breast cancer cases tended to be white and older. Breast cancer cases were similar to controls regarding anti-dsDNA positivity, disease activity, and most drug exposures over time. In univariate and multivariate models, the principal factor associated with breast cancers was older age at cohort entry. Conclusions There was little evidence that breast cancer risk in this SLE sample was strongly driven by any of the clinical factors that we studied. Further search for factors that determine the lower risk of breast cancer in SLE may be warranted.
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Neoplasias de la Mama/epidemiología , Lupus Eritematoso Sistémico/complicaciones , Adulto , Factores de Edad , Estudios de Cohortes , Femenino , Humanos , Cooperación Internacional , Persona de Mediana Edad , Análisis Multivariante , Modelos de Riesgos Proporcionales , Factores de RiesgoRESUMEN
Autoantibodies to dense fine speckles 70 (DFS70) are purported to rule out the diagnosis of SLE when they occur in the absence of other SLE-related autoantibodies. This study is the first to report the prevalence of anti-DFS70 in an early, multinational inception SLE cohort and examine demographic, clinical, and autoantibody associations. Patients were enrolled in the Systemic Lupus International Collaborating Clinics (SLICC) inception cohort within 15 months of diagnosis. The association between anti-DFS70 and multiple parameters in 1137 patients was assessed using univariate and multivariate logistic regression. The frequency of anti-DFS70 was 7.1% (95% CI: 5.7-8.8%), while only 1.1% (95% CI: 0.6-1.9%) were monospecific for anti-DFS70. In multivariate analysis, patients with musculoskeletal activity (Odds Ratio (OR) 1.24 [95% CI: 1.10, 1.41]) or with anti-ß2 glycoprotein 1 (OR 2.17 [95% CI: 1.22, 3.87]) were more likely and patients with anti-dsDNA (OR 0.53 [95% CI: 0.31, 0.92]) or anti-SSB/La (OR 0.25 [95% CI: 0.08, 0.81]) were less likely to have anti-DFS70. In this study, the prevalence of anti-DFS70 was higher than the range previously published for adult SLE (7.1 versus 0-2.8%) and was associated with musculoskeletal activity and anti-ß2 glycoprotein 1 autoantibodies. However, 'monospecific' anti-DFS70 autoantibodies were rare (1.1%) and therefore may be helpful to discriminate between ANA-positive healthy individuals and SLE.
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Proteínas Adaptadoras Transductoras de Señales/inmunología , Autoanticuerpos/inmunología , Lupus Eritematoso Sistémico/inmunología , Factores de Transcripción/inmunología , beta 2 Glicoproteína I/inmunología , Adulto , Estudios de Cohortes , Femenino , Humanos , Modelos Logísticos , Masculino , Análisis Multivariante , PrevalenciaRESUMEN
Throughout the 20th century, a remarkable improvement in the prognosis of systemic lupus erythematosus (SLE) has been seen, mostly due to a better understanding of the disease and the advent of new therapies. However, a plateau seems to have been reached. Whilst outcomes related to active disease have greatly improved, damage accrual tends to be slowly increasing especially damage secondary to high-dose glucocorticoids, which could lead not only to increased morbidity but also to a worse long-term prognosis. Cardiovascular disease and infections still account for the majority of deaths yet both could potentially be improved, again, by the use of high-dose glucocorticoids. In addition, antimalarials have also demonstrated many beneficial effects on patients with lupus. Thus, the universal use of hydroxychloroquine, a more rational prescription of glucocorticoids and preventive strategies for cardiovascular disease can be used as measures with major impacts on the future prognosis of patients with SLE.
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Lupus Eritematoso Sistémico/diagnóstico por imagen , Humanos , Inmunosupresores/efectos adversos , Inmunosupresores/uso terapéutico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Pronóstico , Análisis de Supervivencia , Resultado del TratamientoRESUMEN
OBJECTIVE: Anti-C1q has been associated with systemic lupus erythematosus (SLE) and lupus nephritis in previous studies. We studied anti-C1q specificity for SLE (vs rheumatic disease controls) and the association with SLE manifestations in an international multicenter study. METHODS: Information and blood samples were obtained in a cross-sectional study from patients with SLE (n = 308) and other rheumatologic diseases (n = 389) from 25 clinical sites (84% female, 68% Caucasian, 17% African descent, 8% Asian, 7% other). IgG anti-C1q against the collagen-like region was measured by ELISA. RESULTS: Prevalence of anti-C1q was 28% (86/308) in patients with SLE and 13% (49/389) in controls (OR = 2.7, 95% CI: 1.8-4, p < 0.001). Anti-C1q was associated with proteinuria (OR = 3.0, 95% CI: 1.7-5.1, p < 0.001), red cell casts (OR = 2.6, 95% CI: 1.2-5.4, p = 0.015), anti-dsDNA (OR = 3.4, 95% CI: 1.9-6.1, p < 0.001) and anti-Smith (OR = 2.8, 95% CI: 1.5-5.0, p = 0.01). Anti-C1q was independently associated with renal involvement after adjustment for demographics, ANA, anti-dsDNA and low complement (OR = 2.3, 95% CI: 1.3-4.2, p < 0.01). Simultaneously positive anti-C1q, anti-dsDNA and low complement was strongly associated with renal involvement (OR = 14.9, 95% CI: 5.8-38.4, p < 0.01). CONCLUSIONS: Anti-C1q was more common in patients with SLE and those of Asian race/ethnicity. We confirmed a significant association of anti-C1q with renal involvement, independent of demographics and other serologies. Anti-C1q in combination with anti-dsDNA and low complement was the strongest serological association with renal involvement. These data support the usefulness of anti-C1q in SLE, especially in lupus nephritis.
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Anticuerpos Antinucleares/sangre , Complemento C1q/inmunología , ADN/inmunología , Lupus Eritematoso Sistémico/inmunología , Adulto , Estudios de Casos y Controles , Proteínas del Sistema Complemento/deficiencia , Estudios Transversales , Femenino , Humanos , Lupus Eritematoso Sistémico/etnología , Nefritis Lúpica/etnología , Nefritis Lúpica/inmunología , Masculino , Persona de Mediana Edad , Proteinuria/sangre , Enfermedades Reumáticas/inmunología , Sensibilidad y Especificidad , Adulto JovenRESUMEN
OBJECTIVES: To compare the effectiveness and safety of medium-dose (MD) and high-dose (HD) prednisone regimens and to identify factors related to remission with a target maintenance dose of prednisone in patients with giant cell arteritis (GCA). METHODS: Retrospective cohort study conducted in an autoimmune diseases unit. Patients received ≤ 30 mg (MD group) or >30 mg (HD group) of daily prednisone as monotherapy or combined with methylprednisolone pulses and/or methotrexate, at the discretion of the physician. The primary endpoint was time to clinical and biological remission receiving a prednisone maintenance dose ≤ 7.5 mg/day. Factors related to the primary endpoint were identified by Cox regression analysis. RESULTS: Overall, 103 patients (MD=53, HD=50) were followed for a median (95%CI) of 2.85 (2.57-3.52) years. Both groups exhibited similar baseline features except for ocular ischaemic manifestations (MD=21%, HD=48%, p=0.004). Patients in the MD group had a shorter time to the primary endpoint (MD=186 [147-223], HD=236 [177-276] days, HR=1.70 [1.12-2.57], p=0.01) with no increase in relapses (MD=39%, HD=50%, p=0.29) or GCA complications (MD=11%, HD=16%, p=0.49). Cumulative prednisone doses at 6 months were 2.47 ± 0.70 g for MD patients and 3.86 ± 1.85 g for HD patients (p<0.001). Adverse effects were more frequent among HD recipients (MD=43%, HD=66%, p=0.02). The only independent factor associated with the primary endpoint was the use of methylprednisolone pulses (HR=2.21 [1.31-3.71], p=0.003). CONCLUSIONS: MD prednisone regimen may be an effective and safe alternative to HD prednisone regimen in GCA. Induction with methylprednisolone pulses predicts a better response, allowing for a less intensive prednisone regimen.
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Antiinflamatorios/administración & dosificación , Arteritis de Células Gigantes/tratamiento farmacológico , Inmunosupresores/uso terapéutico , Metotrexato/uso terapéutico , Metilprednisolona/uso terapéutico , Prednisona/administración & dosificación , Anciano , Anciano de 80 o más Años , Estudios de Cohortes , Diabetes Mellitus/inducido químicamente , Quimioterapia Combinada , Femenino , Humanos , Hipercolesterolemia/inducido químicamente , Hipertensión/inducido químicamente , Masculino , Persona de Mediana Edad , Fracturas Osteoporóticas/inducido químicamente , Inducción de Remisión , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Both acute pancreatitis and diffuse alveolar haemorrhage are rare conditions associated with systemic lupus erythematosus (SLE). In this case report, a 23-year-old female with SLE was diagnosed with lupus-associated pancreatitis and, within a few days and despite initial therapy with pulse methyl-prednisolone, subsequently suffered an acute respiratory failure due to a diffuse alveolar haemorrhage. The patient was admitted to the intensive care unit and treatment was intensified with cyclophosphamide and rituximab, which shortly induced the complete remission of SLE with resolution of both clinical conditions. She completed treatment with six pulses of cyclophosphamide followed by azathioprine, hydroxychloroquine and prednisone at initial doses of 20 mg/d with rapid tapering to 5 mg/d, without relapse of the disease during the following year. This case can illustrate that, even in severe, life-threatening SLE flares, it is possible to avoid high-dose prednisone, which has been associated with severe side effects, including infections. Acute pancreatitis and diffuse alveolar haemorrhage are rare conditions caused by SLE. DAH can be a life-threatening complication, with an early mortality of at least 50%. When facing such severe SLE activity, there is a general tendency to use high doses of prednisone as the initial therapy, maintaining such high doses for long periods of time, even after the clinical situation has subsided. We report a case of a young woman with SLE, suffering from acute pancreatitis and diffuse alveolar haemorrhage, who was successfully treated with pulse methyl-prednisolone, hydroxychloroquine, cyclophosphamide and rituximab, combined with medium doses of prednisone.
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Anticuerpos Monoclonales de Origen Murino/administración & dosificación , Ciclofosfamida/administración & dosificación , Inmunosupresores/administración & dosificación , Lupus Eritematoso Sistémico/tratamiento farmacológico , Pancreatitis/tratamiento farmacológico , Prednisona/administración & dosificación , Insuficiencia Respiratoria/tratamiento farmacológico , Enfermedad Aguda , Adulto , Anticuerpos Monoclonales de Origen Murino/efectos adversos , Ciclofosfamida/efectos adversos , Progresión de la Enfermedad , Quimioterapia Combinada , Femenino , Hemorragia/inmunología , Humanos , Inmunosupresores/efectos adversos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/inmunología , Pancreatitis/inmunología , Prednisona/efectos adversos , Inducción de Remisión , Insuficiencia Respiratoria/inmunología , Rituximab , Índice de Severidad de la Enfermedad , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
OBJECTIVE: Evidence points to a decreased breast cancer risk in systemic lupus erythematosus (SLE). We analyzed data from a large multisite SLE cohort, linked to cancer registries. METHODS: Information on age, SLE duration, cancer date, and histology was available. We analyzed information on histological type and performed multivariate logistic regression analyses of histological types according to age, SLE duration, and calendar year. RESULTS: We studied 180 breast cancers in the SLE cohort. Of the 155 cases with histology information, 11 were referred to simply as 'carcinoma not otherwise specified'. In the remaining 144 breast cancers, the most common histological type was ductal carcinoma (n = 95; 66%) followed by lobular adenocarcinoma (n = 11; 8%), 15 cancers were of mixed histology, and the remaining ones were special types. In our regression analyses, the independent risk factors for lobular versus ductal carcinoma was age [odds ratio (OR) 1.07, 95% confidence interval (CI) 1.01-1.14] and for the 'special' subtypes it was age (OR 1.06, 95% CI 1.01-1.10) and SLE duration (OR 1.05, 95% CI 1.00-1.11). CONCLUSIONS: Generally, up to 80% of breast cancers are ductal carcinomas. Though our results are not definitive, in the breast cancers that occur in SLE, there may be a slight decrease in the ductal histological type. In our analyses, age and SLE duration were independent predictors of histological status.
Asunto(s)
Neoplasias de la Mama/etiología , Carcinoma Ductal de Mama/etiología , Carcinoma Lobular/etiología , Lupus Eritematoso Sistémico/complicaciones , Adulto , Anciano , Neoplasias de la Mama/patología , Carcinoma Ductal de Mama/patología , Carcinoma Lobular/patología , Estudios de Cohortes , Susceptibilidad a Enfermedades/etiología , Susceptibilidad a Enfermedades/patología , Femenino , Humanos , Modelos Logísticos , Persona de Mediana Edad , Análisis Multivariante , Oportunidad Relativa , Factores de RiesgoRESUMEN
Infection is one of the leading causes of morbidity and mortality in systemic lupus erythematosus (SLE). Bacterial infections are most frequent, followed by viral and fungal infections. The impaired cellular and humoral immune functions seen in patients with SLE are predisposing conditions, whilst disease activity, prednisone doses over 7.5-10 mg/day, high doses of methylprednisolone or cyclophosphamide are well-recognised risk factors for infection. The first six months after rituximab treatment and the use of more than three courses are also associated with an increased susceptibility for infection. It has not been established whether belimumab, azathioprine and mycophenolate mofetil increase the risk of serious infections. Most vaccines are effective and safe in SLE patients, although vaccination should be avoided during periods of active disease. Live virus vaccines are contraindicated for immunosuppressed patients. Influenza and pneumococcal vaccines are universally recommended. Tuberculosis prophylaxis should be considered in selected cases. Therefore, it is advisable not to exceed doses of 5 mg/day of prednisone in chronic treatment. Methylprednisolone and cyclophosphamide should be used in low-dose regimens. Antimalarials have a well-known protective role against infection, in addition to other beneficial properties, thus, hydroxychloroquine is recommended for all SLE patients where no contraindication exists.
Asunto(s)
Infecciones Bacterianas/etiología , Lupus Eritematoso Sistémico/complicaciones , Virosis/etiología , Infecciones Bacterianas/epidemiología , Infecciones Bacterianas/prevención & control , Humanos , Inmunidad Celular , Inmunidad Humoral , Huésped Inmunocomprometido , Inmunosupresores/efectos adversos , Inmunosupresores/uso terapéutico , Lupus Eritematoso Sistémico/mortalidad , Lupus Eritematoso Sistémico/fisiopatología , Micosis/epidemiología , Micosis/etiología , Micosis/prevención & control , Infecciones Oportunistas/epidemiología , Infecciones Oportunistas/etiología , Infecciones Oportunistas/prevención & control , Factores de Riesgo , Vacunas/administración & dosificación , Virosis/epidemiología , Virosis/prevención & controlRESUMEN
Systemic lupus erythematosus (SLE) is a systemic autoimmune inflammatory disease of unknown cause, with heterogeneity in its clinical presentation, as well as variability in its clinical course and prognosis. The current goal of treatment is to achieve disease remission or a state of low activity, and thereby improve the patient's quality of life. Biological therapy in lupus, unlike other entities, although it has not been fully established, in recent years it has burst onto the scene with important therapeutic novelties. This review aims to update the therapeutic tools for the treatment of SLE focusing on the new molecules that have achieved the objectives of their clinical trials.
Asunto(s)
Lupus Eritematoso Sistémico , Calidad de Vida , Humanos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Pronóstico , Terapia Biológica , Inmunosupresores/uso terapéuticoRESUMEN
Patients with systemic lupus erythematosus are exposed to a remarkably high number of maternal-fetal complications during pregnancy. Knowledge regarding the reciprocal influence between lupus and pregnancy is the starting point to assure that these patients are correctly monitored. It is also important to carry out comprehensive preconception evaluation to individually evaluate the risk of each patient. The immunological profile, history of nephritis, presence of chronic damage and disease activity are the basic data that will determine the specific individual risk profile. Finally, correct drug management must be assured during this period, based on the safety profile of the different treatments during pregnancy and lactation.
Asunto(s)
Antiinflamatorios/uso terapéutico , Inmunosupresores/uso terapéutico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Atención Posnatal/métodos , Complicaciones del Embarazo/tratamiento farmacológico , Atención Prenatal/métodos , Adulto , Lactancia Materna , Femenino , Humanos , Embarazo , Complicaciones del Embarazo/etiología , Complicaciones del Embarazo/prevención & controlRESUMEN
OBJECTIVE: Neuropsychiatric events occur unpredictably in systemic lupus erythematosus (SLE) and most biomarker associations remain to be prospectively validated. This study examined a disease inception cohort of 1047 SLE patients to determine which autoantibodies at enrolment predicted subsequent neuropsychiatric events. METHODS: Patients with a recent SLE diagnosis were assessed prospectively for up to 10 years for neuropsychiatric events using the American College of Rheumatology case definitions. Decision rules of graded stringency determined whether neuropsychiatric events were attributable to SLE. Associations between the first neuropsychiatric event and baseline autoantibodies (lupus anticoagulant (LA), anticardiolipin, anti-ß(2) glycoprotein-I, anti-ribosomal P and anti-NR2 glutamate receptor) were tested by Cox proportional hazards regression. RESULTS: Disease duration at enrolment was 5.4 ± 4.2 months, follow-up was 3.6 ± 2.6 years. Patients were 89.1% female with mean (±SD) age 35.2 ± 13.7 years. 495/1047 (47.3%) developed one or more neuropsychiatric event (total 917 events). Neuropsychiatric events attributed to SLE were 15.4% (model A) and 28.2% (model B). At enrolment 21.9% of patients had LA, 13.4% anticardiolipin, 15.1% anti-ß(2) glycoprotein-I, 9.2% anti-ribosomal P and 13.7% anti-NR2 antibodies. LA at baseline was associated with subsequent intracranial thrombosis (total n=22) attributed to SLE (model B) (HR 2.54, 95% CI 1.08 to 5.94). Anti-ribosomal P antibody was associated with subsequent psychosis (total n=14) attributed to SLE (model B) (HR 3.92, 95% CI 1.23 to 12.5, p=0.02). Other autoantibodies did not predict neuropsychiatric events. CONCLUSION: In a prospective study of 1047 recently diagnosed SLE patients, LA and anti-ribosomal P antibodies are associated with an increased future risk of intracranial thrombosis and lupus psychosis, respectively.
Asunto(s)
Autoanticuerpos/sangre , Biomarcadores/sangre , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/inmunología , Trastornos Mentales/diagnóstico , Adulto , Métodos Epidemiológicos , Femenino , Humanos , Trombosis Intracraneal/epidemiología , Trombosis Intracraneal/etiología , Inhibidor de Coagulación del Lupus/sangre , Lupus Eritematoso Sistémico/diagnóstico , Masculino , Trastornos Mentales/epidemiología , Trastornos Mentales/etiología , Persona de Mediana Edad , Pronóstico , Trastornos Psicóticos/epidemiología , Trastornos Psicóticos/etiología , Proteínas Ribosómicas/inmunología , Adulto JovenRESUMEN
The antiphospholipid syndrome (APS) is defined by the presence of thrombosis and/or pregnancy morbidity in combination with the persistent presence of circulating antiphospholipid antibodies: lupus anticoagulant, anticardiolipin antibodies and/or anti-ß2-glycoprotein I antibodies in medium to high titers. The management of thrombosis in patients with APS is a subject of controversy. This set of recommendations is the result of an effort to produce guidelines for therapy within a group of specialist physicians in Cardiology, Neurology, Hematology, Rheumatology and Internal Medicine, with a clinical and research focus on APS.
Asunto(s)
Anticuerpos Antifosfolípidos/sangre , Síndrome Antifosfolípido/sangre , Síndrome Antifosfolípido/inmunología , Trombosis/prevención & control , Trombosis/terapia , Comités Consultivos , Anticuerpos Antifosfolípidos/efectos adversos , Síndrome Antifosfolípido/complicaciones , Ensayos Clínicos como Asunto , Congresos como Asunto , Femenino , Humanos , Embarazo , Texas , Trombosis/sangre , Trombosis/etiologíaRESUMEN
BACKGROUND: Antimalarial drugs (AMs), chloroquine (CQ) and hydroxychloroquine (HCQ), are frequently withdrawn in patients with lupus with either severe or remitting disease. However, additional effects beyond immunomodulation have been recently described. The aim of the present work was to analyse all the published evidence of the beneficial and adverse effects of AM therapy in systemic lupus erythematosus (SLE). METHODS: A systematic review of the English literature between 1982 and 2007 was conducted using the MEDLINE and EMBASE databases. Randomised controlled trials (RCTs) and observational studies were selected. Case reports were excluded except for toxicity reports. The GRADE system was used to analyse the quality of the evidence. RESULTS: A total of 95 articles were included in the systematic review. High levels of evidence were found that AMs prevent lupus flares and increase long-term survival of patients with SLE; moderate evidence of protection against irreversible organ damage, thrombosis and bone mass loss. Toxicity related to AMs is infrequent, mild and usually reversible, with HCQ having a safer profile. In pregnant women, high levels of evidence were found that AMs, particularly HCQ, decrease lupus activity without harming the baby. By contrast, evidence supporting an effect on severe lupus activity, lipid levels and subclinical atherosclerosis was weak. Individual papers suggest effects in preventing the evolution from SLE-like to full-blown SLE, influencing vitamin D levels and protecting patients with lupus against cancer. CONCLUSIONS: Given the broad spectrum of beneficial effects and the safety profile, HCQ should be given to most patients with SLE during the whole course of the disease, irrespective of its severity, and be continued during pregnancy.