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      Activated STING in a vascular and pulmonary syndrome.

      Author(s): 1 , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , ,
      Publication date (Electronic):
      Journal: The New England journal of medicine

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          Abstract

          The study of autoinflammatory diseases has uncovered mechanisms underlying cytokine dysregulation and inflammation.

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          Autoimmunity initiates in nonhematopoietic cells and progresses via lymphocytes in an interferon-dependent autoimmune disease.

          Alevtina Gall, Piper R. Treuting, Keith B. Elkon … (2012)
          The type I interferon (IFN) response initiated by detection of nucleic acids is important for antiviral defense but is also associated with specific autoimmune diseases. Mutations in the human 3' repair exonuclease 1 (Trex1) gene cause Aicardi-Goutières syndrome (AGS), an IFN-associated autoimmune disease. However, the source of the type I IFN response and the precise mechanisms of disease in AGS remain unknown. Here, we demonstrate that Trex1 is an essential negative regulator of the STING-dependent antiviral response. We used an in vivo reporter of IFN activity in Trex1-deficient mice to localize the initiation of disease to nonhematopoietic cells. These IFNs drove T cell-mediated inflammation and an autoantibody response that targeted abundant, tissue-restricted autoantigens. However, B cells contributed to mortality independently of T cell-mediated tissue damage. These findings reveal a stepwise progression of autoimmune disease in Trex1-deficient mice, with implications for the treatment of AGS and related disorders. Copyright © 2012 Elsevier Inc. All rights reserved.
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            Antiphospholipid syndrome.

            Mark Crowther, Guillermo Ruiz-Irastorza, Ware Branch … (2010)
            The antiphospholipid syndrome causes venous, arterial, and small-vessel thrombosis; pregnancy loss; and preterm delivery for patients with severe pre-eclampsia or placental insufficiency. Other clinical manifestations are cardiac valvular disease, renal thrombotic microangiopathy, thrombocytopenia, haemolytic anaemia, and cognitive impairment. Antiphospholipid antibodies promote activation of endothelial cells, monocytes, and platelets; and overproduction of tissue factor and thromboxane A2. Complement activation might have a central pathogenetic role. Of the different antiphospholipid antibodies, lupus anticoagulant is the strongest predictor of features related to antiphospholipid syndrome. Therapy of thrombosis is based on long-term oral anticoagulation and patients with arterial events should be treated aggressively. Primary thromboprophylaxis is recommended in patients with systemic lupus erythematosus and probably in purely obstetric antiphospholipid syndrome. Obstetric care is based on combined medical-obstetric high-risk management and treatment with aspirin and heparin. Hydroxychloroquine is a potential additional treatment for this syndrome. Possible future therapies for non-pregnant patients with antiphospholipid syndrome are statins, rituximab, and new anticoagulant drugs. Copyright © 2010 Elsevier Ltd. All rights reserved.
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              Type I interferonopathies: a novel set of inborn errors of immunity.

              Yanick Crow (2011)
              The concept of grouping Mendelian disorders associated with an upregulation of type I interferon is not currently recognized in the medical literature. Here, we argue that such a concept has scientific validity and clinical utility. Specifically, we discuss a group of conditions, including Aicardi-Goutières syndrome, spondyloenchondrodysplasia, and cases of systemic lupus erythematosus with complement deficiency, in which an upregulation of type I interferons is apparently central to their pathogenesis. We believe that these diseases can usefully be considered to represent a novel set of inborn errors of immunity, and that the recognition of such diseases as type I interferonopathies will have significance in the development and use of targeted therapies. © 2011 New York Academy of Sciences.
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                Author and article information

                Journal
                Journal ID (iso-abbrev): N. Engl. J. Med.
                Title: The New England journal of medicine
                ISSN (Electronic): 1533-4406
                ISSN (Print): 0028-4793
                Publication date (Electronic): Aug 7 2014
                Volume: 371
                Issue: 6
                Affiliations
                [1 ] The authors' affiliations are as follows: National Institute of Arthritis and Musculoskeletal and Skin Diseases (Y.L., A.A.J., B.M., G.A.M.S., M.A.D., I.P., H.K., W.L.T., A.M.T., Y.H., N.P., D.C., M.G., A.C.S., P.T.W., S.R.B., Z.D., R.G-M.), National Cancer Institute (C.-C.R.L., E.W.C., J.J.D., M.R.), National Heart, Lung, and Blood Institute (D.Y., A. Biancotto, C.S.H., J.R.F., C.P.M., M.B.), Department of Laboratory Medicine (H.S.K., S.G., S.D.R., T.A.F.), National Human Genome Research Institute (D.L.S., F.C., D.L.K.), Department of Radiology and Imaging Services (S.H.), National Institute on Deafness and Other Communication Disorders (H.J.K.), and National Institute of Allergy and Infectious Diseases (S.M.H., S.M.) - all at the National Institutes of Health (NIH), and Walter Reed National Military Medical Center (O.Y.J., I.H.-S.) - both in Bethesda, MD; Dalhousie University, Halifax, NS, Canada (S.E.R., A.C.I.); Rheinisch-Westfaelische Technische Hochschule Aachen, Aachen (K.T.), and University Hospital of Muenster, Muenster (H.W., D.F., A. Barysenka) - both in Germany; Luis Calvo Mackenna Hospital, Santiago, Chile (B.G.); Merck Research Laboratories, Boston (J.D.H., H.M., J.M.); and Northwestern University Feinberg School of Medicine, Chicago (A.S.P.).
                Article
                Mid ID: NIHMS629092
                DOI: 10.1056/NEJMoa1312625
                PubMed ID: 25029335
                SO-VID: 7d13d1ab-e585-4348-bc38-8d35da4b19f1
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