ABSTRACT
El reumatismo fibroblástico es una enfermedad de aparición excepcional, caracterizada por la asociación de una poliartritis agresiva y la presencia de nódulos cutáneos. La artritis se caracteriza por ser rápidamente progresiva, dando lugar a la pérdida de la función articular y, finalmente, a la aparición de la denominada por autores franceses main an griffe. Las lesiones cutáneas aparecen y desaparecen de forma espontánea, y se caracterizan por unos hallazgos histológicos singulares. Presentamos a una paciente con signos clínicos y hallazgos histológicos indicadivos de reumatismo fibroblástico, que tuvo una respuesta satisfactoria al tratamiento combinado con ciclofosfamida y prednisona, no descrito previamente en la bibliografía internacional (AU)
Subject(s)
Adult , Female , Male , Humans , Arthritis/diagnosis , Arthritis/therapy , Rheumatoid Nodule/complications , Rheumatoid Nodule/diagnosis , Rheumatic Diseases/complications , Rheumatic Diseases/diagnosis , Rheumatic Diseases/therapy , Skin/injuries , Histiocytosis, Non-Langerhans-Cell/diagnosis , Histiocytosis, Non-Langerhans-Cell/therapy , Cyclophosphamide/therapeutic use , Cyclophosphamide/administration & dosage , Biopsy/methods , Hand , Hand/pathology , Prednisone/therapeutic use , Prednisone/administration & dosage , Histiocytosis, Non-Langerhans-Cell/complications , Asthenia/complicationsABSTRACT
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.
Subject(s)
Familial Mediterranean Fever/complications , Multiple Myeloma/complications , Bone Marrow Examination , Humans , Immunoglobulin G , Immunoglobulin kappa-Chains , Male , Middle Aged , Multiple Myeloma/diagnosis , Paraproteinemias/complications , Paraproteinemias/immunologyABSTRACT
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.
