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Reviews

Pharmacological approaches to CNS vasculitis: where are we at now?

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References

  • = papers of interest
  • ••= papers of particular interest
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••The largest cohort of adult patients with PACNS to date.

  • de Boysson H, Zuber M, Naggara O, Bousser MG, et al. Primary angiitis of the central nervous system: description of the first fifty-two adults enrolled in the French cohort of patients with primary vasculitis of the central nervous system. Arthritis Rheumatol. 2014;66(5):1315–1326.

••Another large and independent cohort of adult patients with PACNS.

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••The largest cohort of children with PACNS to date.

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••The princeps article on PACNS and its diagnostic criteria.

•A comprehensive review, emphasizing the differences between adult and children PACNS.

  • Hajj-Ali RA. Primary angiitis of the central nervous system: differential diagnosis and treatment. Best Pract Res Clin Rheumatol. 2010;24(3):413–426.
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  • Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1–11.
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  • Salvarani C, Brown RD Jr., Calamia KT, et al. Primary central nervous system vasculitis with prominent leptomeningeal enhancement: a subset with a benign outcome. Arthritis Rheum. 2008;58(2):595–603.
  • Salvarani C, Brown RD Jr., Calamia KT, et al. Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy. Rheumatology (Oxford). 2008;47(11):1671–1677.
  • Guillevin L, Pagnoux C, Seror R, et al. The five-factor score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French vasculitis study group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19–27.
  • Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359(26):2790–2803.
  • Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349(1):36–44.
  • Monach PA, Arnold LM, Merkel PA. Incidence and prevention of bladder toxicity from cyclophosphamide in the treatment of rheumatic diseases: a data-driven review. Arthritis Rheum. 2010;62(1):9–21.
  • Yilmaz N, Emmungil H, Gucenmez S, et al. Incidence of cyclophosphamide-induced urotoxicity and protective effect of mesna in rheumatic diseases. J Rheumatol. 2015;42(9):1661–1666.
  • De Boysson H, Arquizan C, Guillevin L, et al. Rituximab for primary angiitis of the central nervous system: report of 2 patients from the French COVAC cohort and review of the literature. J Rheumatol. 2013;40(12):2102–2103.
  • Salvarani C, Brown RD Jr., Calamia KT, et al. Efficacy of tumor necrosis factor alpha blockade in primary central nervous system vasculitis resistant to immunosuppressive treatment. Arthritis Rheum. 2008;59(2):291–296.
  • Batthish M, Banwell B, Laughlin S, et al. Refractory primary central nervous system vasculitis of childhood: successful treatment with infliximab. J Rheumatol. 2012;39(11):2227–2229.
  • Liu Y, Jesus AA, Marrero B, et al. Activated STING in a vascular and pulmonary syndrome. N Engl J Med. 2014;371:507–518.
  • Navon Elkan P, Pierce SB, Segel R, et al. Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. N Engl J Med. 2014;370(10):921–931.
  • Cellucci T, Tyrrell PN, Sheikh S, et al. Childhood primary angiitis of the central nervous system: identifying disease trajectories and early risk factors for persistently higher disease activity. Arthritis Rheum. 2012;64(5):1665–1672

•The analysis of a pediatric cohort of PACNS suggests the existence of different clinical patterns. Patients with angiography-negative disease and seizures at presentation experience higher disease activity.

  • Salvarani C, Hunder GG, Morris JM, et al. A-beta-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis. Neurology. 2013;81(18):1596–1603.

•This report compared the characteristics and outcomes of 78 patients with cerebral vascular Abeta deposition and 118 with PACNS without such deposits. Patients with Abeta-related angiitis (ABRA) were older, had more frequent altered cognition, seizures, gadolinium leptomeningeal enhancement, intracerebral hemorrhage, but a lower rate of MRI evidence of cerebral infarction. Their treatments and outcomes, however, were similar.

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