Hepatitis autoinmune, síndrome seco y poliartritis conrespuesta satisfactoria al tratamiento combinado decorticoides y ciclosporina / Autoimmune hepatitis, Sjögren's syndrome and polyarthritis with satisfactory response to combined treatment with corticosteroids and cyclosporin

La hepatitis autoinmune es una entidad poco frecuente. Puede presentarse como un proceso multisistémico, asociado a artritis, manifestaciones cutáneas e incluso glomerulonefritis, siendo en estos casos difícil de delimitar con una enfermedad del tejido conectivo. El tratamiento estándar son los corticoides aislados o bien asociados a azatioprina. En casos refractarios, esta entidad puede responder al tratamiento con fármacos inmunomoduladores. Presentamos a un paciente con síndrome seco, poliartritis y hepatitis autoinmune que respondió satisfactoriamente al tratamiento combinado de esteroides y ciclosporina (AU)

El reumatólogo consultory la piedra filosofal / The consultant rheumatologist and the philosopher's stone

No disponible

[Familial Mediterranean fever and myeloma: an undescribed association]. / Fiebre mediterránea familiar y mieloma: una asociación no descrita.

An atypical case of familial mediterranean fever is presented in a 55 year old male with neither family antecedents nor ethnic determinants. The patient presented isolated articular involvement and positive response in the metaraminol provocation and colchicine suppression test. It was associated with monoclonal type IgG kappa gammopathy which evolved over one year until obtaining criteria, although asymptomatic, for myeloma. The increase of the monoclonal component and the infiltration of the bone medulla by plasmatic cells were considered as signs of progression inducing the initiation of treatment despite the lack of symptoms. Both entities are discussed and a mechanism justifying their association is proposed: interleukin-6 produced by macrophages in the inflammatory articular foci due to the deficiency of the C5a inhibitor existing in familial mediterranean fever, may act on a plasmatic cell clone in which receptors for IL-6 exist as a paracrine growth factor.

[Vasculitis associated with proliferation of large granular lymphocytes]. / Vasculitis asociada con proliferación de linfocitos grandes granulares.

We report a patient with the syndrome of large granular lymphocytes in whom the initial clinical features were polyarthritis, hepatosplenomegaly and neutropenia. Relative lymphocytosis was also demonstrated at the expense of a subpopulation with morphology and surface markers characteristic of large granular lymphocytes (CD2+, CD8+, CD16+ and HNK-1+). After 6 months of asymptomatic course, without changes in clinical or laboratory data, the patient died from an acute abdomen with mesenteric ischemia of different likely causes as suggested by necropsy data (multivisceral diffuse infiltrate by large granular lymphocytes, systemic vasculitis and Clostridium sepsis). The association between this syndrome and systemic vasculitis is discussed.