Determinación de concentraciones séricas de 25(OH) D en niños con lupus eritematoso sistémico y artritis idiopática juvenil / Determination of 25(OH)D serum levels in children with systemic lupus erythematosus and juvenile idiopathic arthritis

Resumen:Introducción: Se sabe que la vitamina D (25(OH) D) tiene un efecto directo sobre la salud del hueso y músculo. Se ha relacionado también con enfermedades reumatológicas de origen autoinmune. Los estudios en niños con este tipo de enfermedades son escasos, sobre todo en artritis idiopática juvenil. El objetivo de este trabajo fue determinar las concentraciones de 25(OH) D en pacientes con lupus eritematoso sistémico (LES) y artritis idiopática juvenil (AIJ), y compararlas con las concentraciones en individuos sanos.Métodos: Se determinaron las concentraciones de 25(OH) D por medio de espectrometría de masas con cromatografía liquida por tándem (ID-LC-MS/MS), las concentraciones de la hormona paratiroidea por análisis inmunorradiométrico (IRMA), y las concentraciones de calcio, fósforo y fosfatasa alcalina por métodos colorimétricos en 37 pacientes con LES, 37 pacientes con AIJ y 79 controles sanos.Resultados: Las concentraciones séricas de 25(OH) D fueron de 18.9 ±7.92 ng/ml en LES, 21.97 ± 5.55 ng/ml en AIJ y 23.6 ± 3.07 ng/ml en controles sanos. Hubo una diferencia significativa al comparar las concentraciones de 25 (OH) D entre los pacientes con LES y los controles sanos (p <0.05). El 29.7% de pacientes con LES, el 35.1% con AIJ y el 31.6% de sujetos sanos cursaron con niveles deficientes de vitamina D en este estudio.Conclusiones: Una tercera parte de los niños estudiados en los tres grupos mostraron deficiencias de vitamina D. La más severa fue en los niños con LES.

Abstract:Background: It is well recognized that vitamin D has a direct effect in bone and muscle and has been associated as well with some rheumatologic diseases. Reports in children are scarce. The aim of this study was to determine the concentration level of 25(OH)D in a group of patients with systemic lupus erythematosus (SLE) and juvenile idiopathic arthritis (JIA) and compare them with healthy controls.Methods: Vitamin D (25(OH)D) was measured with isotope-dilution liquid chromatography-tandem mass spectrometry (ID-LC-MS/MS), PTH with immunoradiometric assay (IRMA), calcium, phosphorus and alkaline phosphatase by colorimetric assay in 37 patients with SLE, 37 patients with JIA and 79 healthy controls.Results: Mean 25(OH)D concentration levels were as follows: SLE 18.9 ± 7.92 ng/ml, JIA 21.97 ± 5.55 ng/ml and 23.6 ± 3.07 ng/ml in healthy controls. There was a significant difference between SLE patients vs. healthy controls (p <0.05); 29.7% of SLE patients, 35.1% of JIA patients and 31.6% of healthy controls had deficient levels of vitamin D.Conclusions: One third of the total sample of children in this study had deficient levels of vitamin D. Patients with SLE presented a significant difference compared with healthy controls.

Factors associated with mortality and infections in patients with systemic lupus erythematosus with diffuse alveolar hemorrhage.

OBJECTIVE: To evaluate factors associated with mortality and infections in patients with systemic lupus erythematosus (SLE) and diffuse alveolar hemorrhage (DAH). METHODS: A retrospective chart review was carried out for medical admissions of patients with a diagnosis of SLE and DAH in 9 hospitals. Clinical and laboratory data were recorded for each patient at DAH diagnosis. RESULTS: We included 57 episodes of DAH of 50 patients (7 recurrences), 49 women (86%), 14 juvenile SLE (24.6%); 24 had died (42.1%). In the chart review we detected infection in 22 episodes (38.6%): 8 invasive fungal infections, 16 bacterial infections, and 2 patients had both types. In the bivariate analysis, factors associated with mortality were high Acute Physiology and Chronic Health Evaluation II scores, requirement of mechanical ventilation (OR 15.0, 95% CI 1.9 to 662.2), infections (fungal or bacterial; OR 3.2, CI 0.9 to 11.1), renal failure (OR 4.9, CI 1.4 to 18.0), and thrombocytopenia (OR 4.3, CI 1.2 to 15.6). We found similar mortality between children and adults. Infections were associated with treatment for SLE, requirement of mechanical ventilation, hypocomplementemia, and high levels of C-reactive protein. CONCLUSION: Infection is a frequent finding in patients with DAH and SLE; we found similar mortality between adult SLE and juvenile SLE. Factors that we describe associated with infections may influence the therapeutic selection for these patients.

Dermatomiositis juvenil y calcinosis extensa. Tratamiento con metilprednisolona y metotrexato / Juvenile dermatomyositis and extensive calcinosis. Treatment with methylprednisolone and methotrexate

La dermatomiositis juvenil (DMJ) es una enfermedad multisistémica de etiología incierta, que resulta en una inflamación crónica no supurativa del músculo estriado, la piel y el tracto gastrointestinal. Las calcificaciones distróficas ocurren en un 30-70% de los niños con DMJ. Presentamos el caso de una paciente de 4 años de edad, con diagnóstico de DMJ según criterios de Bohan y Peter, en una edad muy temprana de presentación, con calcinosis extensas que le impedían sentarse, sin flexión de articulación de rodillas, con clase funcional 3. Recibió tratamiento con pulsos intravenosos de metilprednisolona cada 14 días, además de metotrexato vía oral, con mejoría clínica. A pesar de que la calcinosis es frecuente en enfermedades del tejido conectivo y puede llevar a discapacidad severa, no se han desarrollado protocolos terapéuticos para su manejo. El uso simultáneo de metilprednisolona y metotrexato permite un control más rápido de la enfermedad, con mejoría en la fuerza muscular y el eritema y regresión de las calcinosis, sin efectos colaterales importantes (AU)

Juvenile dermatomyositis (JDM) is considered a multisystemic disease of uncertain etiology. The clinical manifestation is a non-suppurative inflammation of the striated muscle, gastrointestinal tract and skin. Dystrophic calcifications are present in 30%-70% of children with JDM. The clinical case we are presenting is a 4 years old female with diagnosis of JDM in accordance to the Bohan and Peters criteria (very early presentation age) with extensive calcinosis, classified as functional class III, without being able to sit down or flex her knees. She was treated with IV methylprednisolone (MPS) bolus every 14 days and oral methotrexate, with improvement of her clinical condition. Even though calcinosis is a frequent finding in connective tissue disease and can cause severe disability, there are no treatment protocols at this time. The simultaneous use of IV MPS and oral methotrexate allows for a faster control of the disease, improvement in muscular force, reduction of erythema and regression of the calcinosis without important collateral effects (AU)

Lupus eritematoso sistémico en una lactante de 6 meses / Systemic lupus erythematosus in a 6 year old female child

El lupus eritematoso sistémico (LES) es poco frecuente, particularmente en la primera década de la vida; su inicio antes de 1 año de edad es muy raro y conlleva un diagnóstico difícil de documentar, ya que las manifestaciones pueden ser muy variables y parecerse a las de una gran variedad de enfermedades. En la edad pediátrica la incidencia de LES se estima en 0,36-0,9/100.000 niños, con prevalencia mayor en mujeres que en varones (aproximadamente 3:1 en menores de 12 años y 10:1 en niños mayores). Sin embargo, el inicio de LES antes de los 4 años ocurre con muy baja incidencia. Presentamos el caso de una lactante de la que se documentaron claramente más de cuatro criterios diagnósticos de LES desde los 6 meses de edad. Por lo revisado en la literatura, podría ser el caso de la paciente más pequeña que se haya publicado. Los médicos deben estar atentos al mayor riesgo de complicaciones sistémicas en niños con LES que se inicia a una edad temprana y realizar un diagnóstico oportuno de esta enfermedad (AU)

Systemic lupus erythematosus (SLE) is an uncommon disease, particularly in the first decade of life. Finding it before the first year of life is very rare and it entails a difficult diagnosis to document because the clinical manifestations can be very variable and can simulate a great variety of diseases In the pediatric age, incidence of SLE is 0.36-0.9/100 000 children, with greater prevalence in females 3:1 under 12 years of age and 10:1 in patients over 12 years. There is a very low incidence of SLE before 4 years of age. We present the clinical case of a 9 month-old female in whom four criteria of SLE were clearly documented at 6 months of age. In a review of literature, this is the youngest patient reported with SLE. Physicians must be very alert of the higher risk of systemic complications in children that are diagnosed with SLE at an early age (AU)

[Juvenile dermatomyositis and extensive calcinosis. Treatment with methylprednisolone and methotrexate]. / Dermatomiositis juvenil y calcinosis extensa. Tratamiento con metilprednisolona y metotrexato.

Juvenile dermatomyositis (JDM) is considered a multisystemic disease of uncertain etiology. The clinical manifestation is a non-suppurative inflammation of the striated muscle, gastrointestinal tract and skin. Dystrophic calcifications are present in 30%-70% of children with JDM. The clinical case we are presenting is a 4 years old female with diagnosis of JDM in accordance to the Bohan and Peters criteria (very early presentation age) with extensive calcinosis, classified as functional class III, without being able to sit down or flex her knees. She was treated with IV methylprednisolone (MPS) bolus every 14 days and oral methotrexate, with improvement of her clinical condition. Even though calcinosis is a frequent finding in connective tissue disease and can cause severe disability, there are no treatment protocols at this time. The simultaneous use of IV MPS and oral methotrexate allows for a faster control of the disease, improvement in muscular force, reduction of erythema and regression of the calcinosis without important collateral effects.

[Systemic lupus erythematosus in a 6 year old female child]. / Lupus eritematoso sistémico en una lactante de 6 meses.

Systemic lupus erythematosus (SLE) is an uncommon disease, particularly in the first decade of life. Finding it before the first year of life is very rare and it entails a difficult diagnosis to document because the clinical manifestations can be very variable and can simulate a great variety of diseases. In the pediatric age, incidence of SLE is 0.36-0.9/100 000 children, with greater prevalence in females 3:1 under 12 years of age and 10:1 in patients over 12 years. There is a very low incidence of SLE before 4 years of age. We present the clinical case of a 9 month-old female in whom four criteria of SLE were clearly documented at 6 months of age. In a review of literature, this is the youngest patient reported with SLE. Physicians must be very alert of the higher risk of systemic complications in children that are diagnosed with SLE at an early age.

Miositis osificante progresiva con buena respuesta a tratamiento con esteroide intravenoso / Progressive myositis ossificans with good response to intravenous steroid treatment

Introducción. La miositis osificante progresiva es una causa rara de miositis en la infancia, se trata de una alteración que se hereda de forma autosómica dominante, caracterizada por inflamación muscular seguida de fibrosis y calcificación. Material y métodos. Dos pacientes con diagnóstico de miositis osificante cuyas manifestaciones fueron: acortamiento de pulgares y primeros ortejos, así como endurecimiento de tejidos blandos y calcificaciones en cuello y tórax. Recibieron tratamiento convencional con difosfonatos e isotretinoína, con pobre respuesta. Se decidió el empleo de metilprednisolona, se aplicaron tres dosis iniciales a 30 mg por kg de peso por dosis por vía intravenosa, y posteriormente cada mes. Resultados. Los pacientes presentaron mejoría clínica tanto en la clase funcional como en la sintomatología, la cual se ha mantenido a largo plazo. Conclusión. Se propone como alternativa de tratamiento la utilización de metilprednisolona por sus efectos inmunosupresores y antiinflamatorios, sobre todo en aquellos pacientes que no han tenido respuesta al manejo farmacológico convencional.

Introduction. Progressive myositis ossificans is a rare cause of myositis in childhood, is an autosomal dominant inflammatory disorder that results in swelling of muscle followed by fibrosis and calcification. Material and methods. We present 2 cases in which, the main clinical manifestations were congenitally short great toes and thumbs; and hardening of the soft tissues; in both cases calcifications of the neck and the thorax were demonstrated by roentgenograms. The 2 patients received conventional management with diphosphonates and isotretinoine, with poor response to these therapies; because of this we decided to use 3 initial doses of intravenous methylprednisolone (30 mg/kg/doses), and after that, we continued the treatment with monthly applications of methylprednisolone. Results. With this therapy our patients improved; and this was evidenciated by diminishing of calcifications and by improvement in their functional status. Conclusion. In this paper we propose an alternative pharmacological therapy with intravenous methylprednisolone, looking for its antiinflammatory and immunosuppressive effects of steroids; mainly in patients with poor response to conventional treatments.