Comparación de pacientes con lupus eritematoso sistémico tratados en centros públicos versus centros privados de salud / Comparison of systemic lupus erythematosus patients treated in the public system of health versus private centers

Estudios recientes sobre lupus eritematoso sistémico (LES) han comunicado desenlaces más favorables gracias al mejor conocimiento de la enfermedad, al manejo más experto del mismo y al uso racional de los tratamientos. En este estudio se identificaron 301 pacientes con lupus eritematoso sistémico (LES), vistos entre 1988 y 2019. Doscientos veintiocho fueron tratados en el sistema público de salud y 73 en el sistema privado. Ambos grupos fueron comparados, observando que los primeros tenían menor edad al momento de la primera consulta y al inicio del LES y menor tiempo de evolución hasta la primera consulta que los pacientes atendidos en el sistema privado de salud de manera estadísticamente significativa. También mostraron mayor frecuencia de leucopenia, con presencia de anticuerpo Sm y compromiso renal en la primera consulta, como así también mayor uso de corticoides EV. Los pacientes hospitalarios presentaron mayor daño acumulado en los primeros 10 años de evolución, pero el desenlace muerte fue similar en ambos grupos. Ambos grupos de pacientes (tanto los del sistema público como los del sector privado) fueron atendidos por especialistas, realizando un cuidadoso seguimiento de estos pacientes.

Recent studies on systemic lupus erythematosus (SLE) have reported more favorable outcomes thanks to better knowledge of the disease, more expert management of it, and rational use of treatments. In this study we identified 301 SLE patients, seen between 1988 and 2019. Two hundred and twenty eight patients were treated in the public health system and 73 in private practice. In comparing both groups, we discovered that patients in the public health system were younger at first consultation and at SLE onset and that the mean duration of their disease prior to first consultation was shorter in a statistically significant way. Also, they showed more frequence of leucopenia, Sm antibody, renal involvement and received I.V. corticosteroids. Patients treated in the public system of health showed more accrual damage over the 10 first years of the disease than patients seen in the private system of health, but death in both groups was similar. Patients from both public and private groups were attended by medical specialist practices who made close follow-ups.

Evaluación de estrés oxidativo en pacientes con Lupus Eritematoso Sistémico y su posible relación con la exposición ambiental a agroquímicos / Assessment of oxidative stress in patients with Systemic Lupus Erythematosus and its possible relationship with environmental exposure to agrochemicals

Los agroquímicos son un método efectivo para controlar especies perjudiciales a los intereses del hombre, pero aplicados indiscriminadamente provocan diversos impactos a nivel ambiental y en la salud. Uno de los mecanismos más importantes por el cual los agroquímicos se ponen en contacto directo con las poblaciones de zonas periurbanas es mediante la deriva. El Lupus Eritematoso Sistémico (LES) es una enfermedad autoinmune que posee un amplio rango de presentaciones clínicas y severidad. Si bien su etiología es desconocida, se la asocia con diversas causas ambientales dentro de las que podrían estar presentes los agroquímicos. El objetivo de este estudio fue evaluar, el posible daño oxidativo generado por exposición ambiental a mezclas de agroquímicos en pacientes con LES y personas sanas que residen en zonas rurales expuestas a mezclas de plaguicidas y contrastar los resultados con pacientes y personas sanas de zonas urbanas no expuestas. Se evaluaron 44 pacientes con diagnóstico de LES y 58 personas sanas (controles), se utilizaron como marcadores: catalasa (CAT), superóxido dismutasa (SOD), la relación glutation oxidado/reducido (GSH/GSSG) y lipoperoxidación (TBARS). Se encontraron diferencias estadísticamente signifcativas para TBARS y SOD al comparar los resultados del grupo LES urbano con su control y el grupo LES rural con el suyo (p<0,01 en ambos). Al realizar las comparaciones entre LES urbano y LES rural se halló un incremento en TBARS del 18,3% (p=0,014). Esta investigación demuestra la importancia de la utilización de biomarcadores de daño oxidativo en el seguimiento clínico de pacientes con LES, con el fn de establecer pautas de tratamiento adecuadas y considerar la relación entre la exposición ambiental a plaguicidas y el aumento de daño oxidativo en pacientes con LES.

The agrochemicals are an effective method to control harmful species of interests to humans, but when they are applied indiscriminately, they can cause environmental and health damage to people. Systemic Lupus Erythematosus (SLE) is an autoimmune disease that has a wide range of clinical manifestations and severity. While the etiology of SLE is unknown, it is associated with various environmental causes, being one possibility the exposure to agrochemicals. The aim of this study was to evaluate the possible oxidative damage generated by environmental exposure to agrochemical mixtures in SLE patients and healthy people living in rural areas and compare them with patients and healthy people living in urban, unexposed areas. Forty four patients diagnosed with SLE and 58 healthy individuals (controls) were evaluated, using the following markers: Catalase (CAT), superoxide dismutase (SOD), the oxidized/reduced glutathione ratio (GSH / GSSG) and lipid peroxidation (TBARS). Statistically signifcant differences were found in TBARS and SOD by comparing the results of the urban SLE group with its control and rural SLE group with its control (p <0.01 for both cases). When making comparisons between urban SLE group and the rural SLE group, an increase of 18.3% was found in TBARS (p = 0.014). This research demonstrates the importance of using biomarkers of oxidative stress in the clinical monitoring of patients with SLE, in order to establish guidelines for appropriate treatment and consider the relationship between environmental exposure to pesticides and increased oxidative damage in patients with SLE.

Osteonecrosis en lupus eritematoso sistémico / Osteonecrosis in systemic lupus erythematosus

Objetivos: Definir la proporción de osteonecrosis (ON) en nuestra población lúpica, identificar factores asociados a su desarrollo y realizar un análisis descriptivo de las ON. Materiales y métodos: Estudio retrospectivo observacional. Se incluyó a 158 pacientes con lupus eritematoso sistémico (criterios ACR 1982), comparando datos demográficos, de laboratorio, manifestaciones clínicas, SLICC, SLEDAI, tratamiento citotóxico y esteroideo. En pacientes con ON se analizaron el tiempo de evolución y la edad al diagnóstico de ON, la forma de presentación, la articulación comprometida, el método diagnóstico, la clasificación Ficat y Arlet y el tratamiento realizado. Se utilizó la prueba de la t o la prueba de Mann-Whitney para la comparación de medias y para igualdad de proporciones o independencia, la prueba de la chi al cuadrado o exacta de Fisher, según correspondiera. Resultados: La ON ocurrió en 15/158 pacientes (9,5%), 13 mujeres y 2 hombres. Edad al diagnóstico de ON (mediana): 30 años (r: 16-66) y el tiempo de evolución hasta el diagnóstico de ON: 35 meses (r: 1-195). En los 15 pacientes hubo 34 articulaciones con ON, 23 sintomáticas y 22 diagnosticadas por RM. Veintiséis ON fueron en caderas (24 bilaterales), 4 en rodillas y 4 en hombros. En 13 pacientes la ON afectó a 2 o más articulaciones. Al inicio, 28 articulaciones estaban en estadio i - ii , uno en estadio iii y 5 sin datos y al final, 14 en estadio iii - iv , 5 en estadio i-ii y 15 sin datos. Veintinueve se trataron con reposo y 8 caderas requirieron reemplazo articular. La ON se asoció a aspecto Cushing (p = 0,014), OR 4,16 (IC 95% 1,4-12,6) y SLICC 2.° año (p = 0,042). No hubo relación con manifestaciones clínicas, datos de laboratorio, tratamiento citotóxico o dosis de esteroides ni actividad. Todos los pacientes con ON recibieron antipalúdicos, a diferencia de un 77% de aquellos sin ON. Conclusiones: La proporción de ON fue del 9,5%, la mayoría fue en mujeres, el 76% en caderas (26) y el 92% bilateral. Se asociaron significativamente a aspecto Cushing y daño acumulado al segundo año. No se halló relación con el resto de las variables evaluadas (AU)

Objectives: To define the proportion of osteonecrosis (ON) in our patient population with lupus and to identify factors associated with the development of ON in systemic lupus erythematosus, as well as to carry out a descriptive analysis of ON cases. Materials and methods: Observational retrospective study of 158 patients with SLE (ACR 1982 criteria). Demographic and laboratory data, clinical manifestations, SLICC, SLEDAI, cytotoxic and steroid treatments were compared. In patients with ON, we analyzed time of disease progression and age at ON diagnosis, form of presentation, joints involved, diagnostic methods, Ficat-Arlet classification, and treatment. To compare the means, t-test or Mann-Whitney’s test were employed and the cHi-2 test or Fisher’s exact test, as appropriate, were used to measure the equality of proportions. Results: ON was present in 15 out 158 patients (9.5%), 13 women and 2 men, with a mean age of 30 (r: 16-66) at diagnosis and 35 months of evolution until diagnosis (r: 1-195). Among the 15 patients, 34 joints presented ON, 23 were symptomatic and 22 were diagnosed by magnetic resonance images. Twenty-six occurred in hips (24 bilateral), 4 in knees and 4 in shoulders. In 13 patients, ON involved 2 or more joints. At onset, 28 joints were in stage i-ii, one in stage iii and 5 had no data and; in the end, 14 were in stage iii-iv, 5 in stage i-ii and 15 had no data. Twenty-nine underwent conservative treatment with rest and 8 hips required joint replacement. ON progression was associated with Cushing’s syndrome (P=0.014) OR 4.16 (95% CI 1.4-12.6) and 2nd year SLICC (P=0.042). No relation with clinical manifestations, lab results, cytotoxic treatment, steroid treatment (total accumulated dose, mean daily dose and duration) metilprednisolone pulses, nor activity was found. All patients with ON received antimalarials, in contrast to 77% of those without ON. Conclusions: The proportion of ON was 9.5%, mainly in women, 76% in hips (26) and 92% bilaterally. They were associated significantly with Cushing’s syndrome and accumulated damage at second year (AU)

Osteonecrosis in systemic lupus erythematosus.

OBJECTIVES: To define the proportion of osteonecrosis (ON) in our patient population with lupus and to identify factors associated with the development of ON in systemic lupus erythematosus, as well as to carry out a descriptive analysis of ON cases. MATERIALS AND METHODS: Observational retrospective study of 158 patients with SLE (ACR 1982 criteria). Demographic and laboratory data, clinical manifestations, SLICC, SLEDAI, cytotoxic and steroid treatments were compared. In patients with ON, we analyzed time of disease progression and age at ON diagnosis, form of presentation, joints involved, diagnostic methods, Ficat-Arlet classification, and treatment. To compare the means, t-test or Mann-Whitney's test were employed and the cHi-2 test or Fisher's exact test, as appropriate, were used to measure the equality of proportions. RESULTS: ON was present in 15 out 158 patients (9.5%), 13 women and 2 men, with a mean age of 30 (r: 16-66) at diagnosis and 35 months of evolution until diagnosis (r: 1-195). Among the 15 patients, 34 joints presented ON, 23 were symptomatic and 22 were diagnosed by magnetic resonance images. Twenty-six occurred in hips (24 bilateral), 4 in knees and 4 in shoulders. In 13 patients, ON involved 2 or more joints. At onset, 28 joints were in stage i-ii, one in stage iii and 5 had no data and; in the end, 14 were in stage iii-iv, 5 in stage i-ii and 15 had no data. Twenty-nine underwent conservative treatment with rest and 8 hips required joint replacement. ON progression was associated with Cushing's syndrome (P=0.014) OR 4.16 (95% CI 1.4-12.6) and 2nd year SLICC (P=0.042). No relation with clinical manifestations, lab results, cytotoxic treatment, steroid treatment (total accumulated dose, mean daily dose and duration) metilprednisolone pulses, nor activity was found. All patients with ON received antimalarials, in contrast to 77% of those without ON. CONCLUSIONS: The proportion of ON was 9.5%, mainly in women, 76% in hips (26) and 92% bilaterally. They were associated significantly with Cushing's syndrome and accumulated damage at second year.

Encefalitis con estatus convulsivo localizado en una paciente con lupus eritematoso sistémico / Encephalitis and status epilepticus in a patient with systemic lupus erythematosus

Describimos a una paciente de 42 años de edad con diagnóstico de lupus eritematoso sistémico (LES) inactivo al momento de la visita. Se presentó con desórdenes del movimiento atípicos y epilepsia parcial continua (EPC). Una biopsia cerebral excluye procesos tumorales y vasculitis. Discutimos diagnósticos diferenciales de estatus epiléptico en una paciente con LES (AU)

We describe a 42-year old woman with inactive systemic lupus erythematosus (SLE) at the time of her visit. She presented with an atypical movement disorder and partial continuous epilepsy. A brain biopsy excluded cerebral vasculitis and tumoral processes. We discuss the differential diagnosis of status epilepticus in a patient with systemic lupus erythematosus (AU)

Encephalitis and status epilepticus in a patient with systemic lupus erythematosus.

We describe a 42-year old woman with inactive systemic lupus erythematosus (SLE) at the time of her visit. She presented with an atypical movement disorder and partial continuous epilepsy. A brain biopsy excluded cerebral vasculitis and tumoral processes. We discuss the differential diagnosis of status epilepticus in a patient with systemic lupus erythematosus.

Micofenolato mofetil en el tratamiento del Rhupus / Mycophenolate mofetil in the treatment of Rhupus

Micofenolato mofetil (MMF) es un agente inmunosupresor que se comenzó a utilizar en pacientes trasplantados. Posteriormente, MMF se ha utilizado en una variedad de enfermedades autoinmunes. La mayor experiencia en el lupus eritematoso sistémico (LES) se ha centrado en la glomerulonefritis lúpica. Tras su éxito en el tratamiento de dicha patología, MMF ya está siendo utilizado para el control de otras manifestaciones, tales como las manifestaciones hematológicas y cutáneas resistentes a los esquemas habituales. En este caso clínico, se presenta una paciente con Rhupus, cuya artropatía no respondió a más de tres FARME, y presentó mejoría clínica y radiológica con micofenolato mofetil (MMF).

Micofenolato mofetil en el tratamiento del Rhupus / Mycophenolate mofetil in the treatment of Rhupus

Micofenolato mofetil (MMF) es un agente inmunosupresor que se comenzó a utilizar en pacientes trasplantados. Posteriormente, MMF se ha utilizado en una variedad de enfermedades autoinmunes. La mayor experiencia en el lupus eritematoso sistémico (LES) se ha centrado en la glomerulonefritis lúpica. Tras su éxito en el tratamiento de dicha patología, MMF ya está siendo utilizado para el control de otras manifestaciones, tales como las manifestaciones hematológicas y cutáneas resistentes a los esquemas habituales. En este caso clínico, se presenta una paciente con Rhupus, cuya artropatía no respondió a más de tres FARME, y presentó mejoría clínica y radiológica con micofenolato mofetil (MMF).(AU)

Rhupus: descripción de 4 casos / Rhupus: report of 4 cases

Presentamos las características clínicas y serológicas de cuatro pacientes con diagnóstico de rhupus (definición de Simon). Presentaban criterios ACR tanto para LES como para AR, factor antinuclear (FAN) positivo a títulos desde 1/80 a 1/5.250 y anti-DNAn positivo, predominando como manifestación clínica una poliartritis simétrica erosiva. Se halló anti-CCP positiva y a títulos altos en 3 de los 4 pacientes y anticuerpos antifosfolípidos en 2 (anticardiolipina y anticoagulante lúpico), sin manifestación de síndrome antifosfolípido. Sólo un paciente presentó compromiso renal y 2 nódulos subcutáneos. En 3 de los pacientes el diagnóstico de AR precedió a las manifestaciones de LES en un promedio de 7,7 años. La mitad fue refractaria a fármacos antirreumáticos modificadores de la enfermedad (FAME) convencionales en combinación y requirió biológicos y micofenolato mofetil (AU)

We present the clinical and serological characteristics of four patients with rhupus (Simon’s definition). The 4 patients with rhupus presented ACR criteria for SLE as well as for RA, ANA positive with titers ranging from 1/80 to 1/5,250, and positive anti-DNA, with the predominance of symmetrical erosive polyarthritis. We found anti-CCP positivity and high titers in 3 of the 4 patients, and positive antiphospholipid antibodies in 2 (anticardiolipin and LA), without manifestation of antiphospholipid syndrome. One patient presented renal affection, and 2 subcutaneous nodules. The 3 patients with RA preceded the manifestations of SLE by an average of 7.7 years. Two patients were refractory to conventional DMAR in combination, requiring biologic and mycophennolate mofetil (AU)

Neuromyelitis Optica-AQP4: an update.

Devic disease (neuromyelitis optica [NMO]) is an idiopathic inflammatory demyelinating and necrotizing disease characterized by optic neuritis and transverse myelitis, either simultaneously or in isolation. NMO is often idiopathic but may also be associated with systemic autoimmune disease. The prognosis of NMO is severe, especially in those with early and recurrent relapses. MRI studies have revealed that most frequently, there is a long spinal cord lesion that extends through three or more vertebral segments in length. NMO-IgG is the first antibody marker for any inflammatory central nervous system disorder and is both sensitive and specific for NMO. The identification of NMO-IgG in patients with recurrent optic neuritis or longitudinally extensive myelitis and its ability to predict subsequent relapse support the concept of a spectrum of NMO disorders. Treatment in the acute phase includes intravenous steroids and plasma exchange therapy. Immunosuppressive agents are recommended for the prophylaxis of relapses.

[Rhupus: report of 4 cases]. / Rhupus: descripción de 4 casos.

We present the clinical and serological characteristics of four patients with rhupus (Simon's definition). The 4 patients with rhupus presented ACR criteria for SLE as well as for RA, ANA positive with titers ranging from 1/80 to 1/5,250, and positive anti-DNA, with the predominance of symmetrical erosive polyarthritis. We found anti-CCP positivity and high titers in 3 of the 4 patients, and positive antiphospholipid antibodies in 2 (anticardiolipin and LA), without manifestation of antiphospholipid syndrome. One patient presented renal affection, and 2 subcutaneous nodules. The 3 patients with RA preceded the manifestations of SLE by an average of 7.7 years. Two patients were refractory to conventional DMAR in combination, requiring biologic and mycophennolate mofetil.