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Resumen El lupus eritematoso sistemico es una enfermedad compleja, con un espectro clinico amplisimo y, por tanto, con un tratamiento que ha de cumplir requisitos dificiles: prevenir las reactivaciones y complicaciones a medio y largo plazo, controlar la inflamacion de los organos afectados de una forma eficaz para evitar en lo posible el dano residual, sin que la inmunosupresion necesaria para ello conlleve un exceso de riesgo de infeccion u otros efectos a largo plazo, como el aumento del riesgo de cancer. Cumplir todas estas premisas no es posible con el conocimiento actual, pero es indudable que el pronostico de la enfermedad ha mejorado considerablemente en las ultimas decadas, y que el conocimiento de la patogenia ha contribuido enormemente al desarrollo de nuevas estrategias y de nuevos farmacos que sin duda cambiaran en los proximos anos las perspectivas de esta enfermedad potencialmente muy grave. En esta actualizacion se revisan los esquemas terapeuticos actualizados, haciendo especial enfasis en las nuevas terapias.

PUNTOS CLAVE Concepto. Las alteraciones de dos fenomenos fisiologicos (autorreactividad y tolerancia inmunologica) estan en la base de las enfermedades autoinmunes (EAI). Etiopatogenia. Se desconoce la etiologia de la mayor parte de las EAI, aunque se acepta que son enfermedades de etiologia multifactorial con una base genetica importante sobre la que influyen diversos factores ambientales de tipo fisico-organico. Mecanismos de autoinmunidad. Dos tipos basicos: mediados por anticuerpos y mediados por celulas. Clasificacion de las EAI. Dos categorias principales: EAI organoespecificas y EAI no-organoespecificas.

Caso clinico Mujer de origen colombiano de 37 anos de edad, sin antecedentes personales de interes, que ingresa por cuadro de unos tres meses de evolucion de lumbalgia inflamatoria, malestar general y febricula mantenida, con ocasionales picos de hasta 39 °C. La paciente referia un episodio autolimitado de fiebre y malestar general unos 6 meses antes del ingreso. Posteriormente permanece asintomatica hasta un mes antes del ingreso que comienza con malestar general y dolor en columna lumbar, irradiado a fosas iliacas de caracteristicas inflamatorias. Todo ello se acompanaba de intensa astenia, sin perdida de peso, y de picos febriles de hasta 39 °C. El dolor lumbar no aumentaba con maniobras de Valsalva, ni se acompanaba de alteraciones esfinterianas. La anamnesis por aparatos no demostraba ningun otro dato concluyente. Los unicos hallazgos de laboratorio de interes mostraban una velocidad de sedimentacion globular (VSG) de 55 mm/h y la proteina C reactiva de 45,7; el resto del estudio inmunologico dio negativo. Serologia a virus de la hepatitis C, hepatitis B, virus de la inmunodeficiencia humana (VIH), Brucella, citomegalovirus, Leishmania, sifilis y Mantoux todo negativo. En el examen fisico, solo se apreciaba un soplo sistolico II/VI en el foco aortico y soplo audible en la zona epigastrica, junto con dolor a la presion profunda en ambos flancos abdominales sin signos de defensa. Todos los pulsos perifericos se encontraban presentes. La tension arterial en miembro superior derecho era de 125/63 y en miembro superior izquierdo de 127/65. Las maniobras sacroiliacas, asi como la espinopercusion dorsolumbar, eran negativas. La exploracion neurologica fue normal.

Background Raynaud9s phenomenon (RP) is characterized by transient episodes of vasoconstriction of the arteries and arterioles of the extremities in response to cold or emotional stimuli. Depending on the severity of the vascular insult, it can cause superficial ulceration or deep-tissue necrosis. Pharmacological treatments aim to enhance blood flow but their efficacy is not uniform. Objectives To evaluate the efficacy of botulinum toxin-A in the treatment of severe RP. Methods We present a series of 7 patients with RP with bad response to conventional pharmacological therapy that have been treated with local botulinum neurotoxin-A. Exclusion criteria included botulinum toxin allergy, active infection at the site of injection, previous digital sympathectomy and pregnancy. 4 to 8 units of botulinum toxin-A were administered per point of injection depending on the degree of severity. Points of injection were located on the lateral and medial aspect of the base of the fingers, except the thumb. Prior to infiltration, obstructive pathology was ruled out by Doppler ultrasound; also, a nailfold capillaroscopy test was performed before and after the infiltration. Variables such as the number of episodes per day, pain during the episodes, recuperation time, finger color and presence of digital ulceration or necrosis have been studied baseline, 30 minutes, one week and one month after the infiltration. Follow up of 6 patients was performed with a 9 weeks median, being 18 weeks the maximum time registered. Patient 1 died due to a peritonitis 3 days after the study started. Results 30 minutes after infiltration, three patients felt no improvement, two assessed slight improvement and two very important improvement. At the patients9 one-week and thirty-days follow-up visits two patients did not perceive any change and four experienced great amelioration. Patients that did not register any change where those with fewer subjective clinical complaints and normal Doppler ultrasound and capillaroscopy tests. The variable with the most remarkable response was pain, with important pain decrease in all of the cases. Three patients presented digit ulcers at baseline visit; ulceration healing was noted in all of them, two of them one week after the injection and the other one, one month after. Three patients reported mild “weakness” after being injected and one reported slight thenar-eminence pain that lasted a few days. None of the patients suffered any systemic complications related to the toxin. Conclusions Botulinum toxin-A is a safe and effective therapeutic option for patients with severe RP that have failed to conventional treatment. Disclosure of Interest None declared

The role of systemic lupus erythematosus (SLE) in the development of acute pancreatitis is a matter of controversy since, in many cases, this complication has been attributed to the drugs administered. In this study, we present a patient diagnosed as having SLE who developed acute pancreatitis with no apparent cause aside from her basic disease, and in whose necropsy was observed vascular damage consisting of severe intimal proliferation. Pancreatic vascular lesions in previously reported patients with lupus are reviewed.