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Vasculitis syndromes

New insights into the molecular basis of systemic vasculitis

Gene variants have been discovered that cause a range of vascular inflammatory phenotypes with a characteristic onset in childhood. What might these exciting findings mean for the understanding, diagnosis and treatment of systemic vasculitis?

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References

  1. Zhou, Q. et al. Early-onset stroke and vasculopathy associated with mutations in ADA2. N. Engl. J. Med. 370, 911–920 (2014).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Navon Elkan, P. et al. Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. N. Engl. J. Med. 370, 921–931 (2014).

    Article  PubMed  Google Scholar 

  3. Jennette, J. C. et al. 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum. 65, 1–11 (2013).

    Article  CAS  PubMed  Google Scholar 

  4. Basu, N. et al. EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis. Ann. Rheum. Dis. 69, 1744–1750 (2010).

    Article  PubMed  Google Scholar 

  5. Lyons, P. A. et al. Genetically distinct subsets within ANCA-associated vasculitis. N. Engl. J. Med. 367, 214–223 (2012).

    CAS  PubMed  PubMed Central  Google Scholar 

  6. Hernández-Rodríguez, J., Alba, M. A., Prieto-González, S. & Cid, M. C. Diagnosis and classification of polyarteritis nodosa. J. Autoimmun. 48–49, 84–89 (2014).

    Article  PubMed  Google Scholar 

  7. Ozen, S. et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann. Rheum. Dis. 69, 798–806 (2010).

    Article  PubMed  Google Scholar 

  8. Zavialov, A. V., Gracia, E., Glaichenhaus, N., Franco, R., Zavialov, A. V. & Lauvau, G. Human adenosine deaminase 2 induces differentiation of monocytes and macrophages and stimulates proliferation of T helper cells and macrophages. J. Leukoc. Biol. 88, 279–290 (2010).

    Article  CAS  PubMed  Google Scholar 

  9. Watts, R. et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann. Rheum. Dis. 66, 222–227 (2007).

    Article  PubMed  Google Scholar 

  10. Pagnoux, C. et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 62, 616–626 (2010).

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

The authors are supported by Instituto de Salud Carlos III (PIE13/00033) and Ministerio de Economía y Competitividad (SAF11/30073).

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Correspondence to Maria C. Cid.

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M.C.C. has acted as a consultant for GSK. J.I.A. declares no competing interests.

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Cid, M., Aróstegui, J. New insights into the molecular basis of systemic vasculitis. Nat Rev Rheumatol 10, 323–324 (2014). https://doi.org/10.1038/nrrheum.2014.75

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