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      Fenómeno de Raynaud secundario y vasculitis cutánea asociados al uso de interferón Translated title: Secondary Raynaud's phenomenon and cutaneous vasculitis associated with the use of interferon

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          Abstract

          Resumen Se presenta el caso de un hombre adulto con fenómeno de Raynaud secundario y refractario al manejo médico asociado a vasculitis cutánea, que previamente venía recibiendo interferón beta para tratamiento de esclerosis múltiple. La agresividad del proceso requiere la interrupción de la medicación,la utilización de vasodilatadores, inhibidores de endotelina 1, simpatectomía bilateral por videotoracoscópia, aplicación de toxina botulínica periarterial interdigital en manos y terapia inmunosupresora con corticoide a altas dosis y ciclofosfamida obteniendo detención del proceso isquémico, pero con pérdida anatómica asociada. (Acta Med Colomb 2014; 39: 81-84).

          Translated abstract

          Abstract The case of an adult male with secondary Raynaud's phenomenon refractory to medical management and associated with cutaneous vasculitis who previously had been receiving interferon beta for multiple sclerosis treatment, is presented. The aggressiveness of the process required the interruption of the medication, the use of vasodilators, inhibitors of endothelin-1, bilateral thoracoscopic sympathectomy by video, peri-arterial interdigital botulinum toxin application in hands and immunosuppressive therapy with high dose corticosteroids and cyclophosphamide, obtaining arrest of the ischemic process, but with associated anatomical loss. (Acta Med Colomb 2014; 39: 81-84).

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          Interferons as pathogenic effectors in autoimmunity.

          Interferons (IFNs) type-1 (IFN alpha/beta) and type-II (IFN-gamma) are the most pleiotropic molecules in the intricate cytokine network. This dominance arises from three crucial factors: (i) initiation of IFN-alpha/beta and IFN-gamma production at the inception of most innate immune responses, which primes for the ensuing adaptive immune responses, primarily through the sine qua non upregulation of major histocompatibility complex and costimulatory molecules; (ii) magnification of their production and signaling by cross-talk between themselves, and synergistic or antagonistic effects on other cytokines; and (iii) direct or indirect initiation of transcription of hundreds of immunologically relevant genes. Considering that aberrant immune responses against self-molecules seem to depend on the same constituents and pathways as those against exogenous antigens, it follows that IFNs are also major effectors in the pathogenesis of autoimmunity. Here, we review the diverse biological effects of IFNs on the immune system, discuss findings pertaining to the nature of exogenous and endogenous stimuli that might induce IFN production through the engagement of Toll-like receptors, and summarize the detrimental and, in some instances, beneficial effects of IFNs in systemic and organ-specific autoimmune diseases.
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            Vascular events associated with alpha interferon therapy.

            Alpha Interferon (IFN) is a biological agent used for the therapy of an increasing number of diseases, either as an established effective therapeutic tool or in the context of clinical trials. The use of IFN may be complicated by serious adverse reactions. We describe here the clinical course of a variety of vasculopathic complications in association with IFN-therapy in 12 patients with the diagnosis of chronic myeloid leukemia and 1 patient with malignant melanoma treated at our institute. Vascular manifestations in these patients include Raynaud's phenomena, digital ulcerations and gangrene, pulmonary vasculitis, pulmonary hypertension and thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS). These reactions occurred after 3 months to 3 years of 3-10 million units (MU) daily IFN therapy. Concomitant administration of hydroxyurea (HU) was noted in 5 patients. Discontinuation of IFN and initiation of immunosuppressive therapy brought about a complete resolution or arrested progression of these reactions. IFN-therapy may be complicated by severe vasculopathic/vasospastic complications that usually improve after its discontinuation. Possible underlying mechanisms for these complications are discussed. The early diagnosis of these complications may be vital and IFN should be immediately discontinued when early signs of these complications become evident.
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              Type I interferon therapy and its role in autoimmunity.

              Interferons (IFNs) display a pleiotropic effect on different cell types of both the innate and the adaptive immunities being able to affect the immune responses. The ability of IFNs, and in particular of type I IFN, to activate dendritic cells and to modulate the expression of major histocompatibility classes I and II molecules supports their potential role also in the development and maintenance of tolerance. When tolerance breakdown has occurred, immunocomplexes generated by the reaction of nuclear antigens and specific autoantibodies can further induce type I IFN production. Accordingly, high acid-labile type I IFN plasma levels, overexpression of IFNalpha-induced transcripts and the association with genes closely related to type I IFN response represent the rationale for the so-called IFN signature in systemic lupus erythematosus, a prototypical autoimmune disease. The role of IFNs in autoimmunity is further supported by their direct and deleterious impact on target tissues. The therapeutic use of IFNs is based on their antiviral and antiproliferative effect. Type I IFN administration, in particular, is associated with the appearance of autoimmunity, although less frequently than expected. Such an event takes place mostly in patients with previous autoimmune manifestations and can be characterized by the appearance of autoantibodies only or of a clinically overt disease. IFN therapy cessation is usually, but not always, required for controlling the autoimmune disorders.
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                Author and article information

                Journal
                amc
                Acta Medica Colombiana
                Acta Med Colomb
                Asociacion Colombiana de Medicina Interna (Bogotá, Distrito Capital, Colombia )
                0120-2448
                January 2014
                : 39
                : 1
                : 81-84
                Affiliations
                [03] Bogotá, D.C orgnameHospital Universitario Fundación Santa Fe de Bogotá Colombia somerson18hotmail.com, somerson18@ 123456gmail.com
                [02] Bogotá, D.C orgnameMedicina Interna Colombia
                [01] Bogotá, D.C orgnameMedicina Interna Colombia
                Article
                S0120-24482014000100017 S0120-2448(14)03900117
                9cf5eeea-5513-44ba-8c1a-ce0b27df2568

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : 16 October 2013
                : 09 October 2012
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 10, Pages: 4
                Product

                SciELO Colombia

                Self URI: Texto completo solamente en formato PDF (ES)
                Categories
                Presentación de casos

                cutaneous vasculitis,autoimmunity,interferon,Raynaud's phenomenon,vasculitis cutánea,autoinmunidad,interferón,fenómeno de Raynaud

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