• Satoshi YoshidaEmail author


The vasculitides span a wide range of disease severity, extending from illnesses that rarely produce death to those almost universally fatal before the introduction of effective therapy. Clinical manifestations of specific vasculitic disorders are diverse and depend on the size of the involved vessels and the organs affected by ischemia. Most damage results when inflammation narrows vessels and causes tissue necrosis.

Immunosuppressive and cytotoxic agents are used to treat many vasculitic diseases. Although such approaches can be effective, long-term treatment may be complicated by chronic sequelae from organ damage, disease relapses, and medication side effects. Recent investigations have focused on understanding the pathophysiology of these diseases, which may lead to more efficacious and less toxic therapeutic options.

Two cases related to the presentation and management of vasculitis are discussed.


Vasculitis Wegener’s granulomatosis Necrotizing sialometaplasia Churg-Strauss syndrome Antineutrophil cytoplasmic antibodies 


  1. 1.
    Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg, J, Gross WL. Nomenclature of systemic vasculitides: proposal of an international consensus conference. Arthritis Rheum 1994;37:187–92.PubMedCrossRefGoogle Scholar
  2. 2.
    Langford CA. Vasculitis. J Allergy Clin Immunol 2003;111:S602–12.PubMedCrossRefGoogle Scholar
  3. 3.
    Carlson DL. Necrotizing sialometaplasia: a practical approach to the diagnosis. Arch Pathol Lab Med 2009;133(5):692–8.PubMedGoogle Scholar
  4. 4.
    Wechsler ME, Garpestad E, Flier SR, Kocher O, Weiland DA, Polito AJ, Klinek MM, Bigby TD, Wong GA, Helmers RA, Drazen JM. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA 1998;279(6):455–7.PubMedCrossRefGoogle Scholar
  5. 5.
    Wechsler ME. Pulmonary eosinophilic syndromes. Immunol Allergy Clin North Am 2007;27(3):477–92.PubMedCrossRefGoogle Scholar
  6. 6.
    Churg A, Brallas M, Cronin SR, Churg J. Formes frustes of Churg-Strauss syndrome. Chest 1995;108(2):320–3.PubMedCrossRefGoogle Scholar
  7. 7.
    Chen KR, Carlson JA. Clinical approach to cutaneous vasculitis. Am J Clin Dermatol 2008;9(2):71–92.PubMedCrossRefGoogle Scholar
  8. 8.
    Murrell DF, Dick S, Ahmed AR, Amagai M, Barnadas MA, Borradori L, Bystryn JC, Cianchini G, Diaz L, Fivenson D, Hall R, Harman KE, Hashimoto T, Hertl M, Hunzelmann N, Iranzo P, Joly P, Jonkman MF, Kitajima Y, Korman NJ, Martin LK, Mimouni D, Pandya AG, Payne AS, Rubenstein D, Shimizu H, Sinha AA, Sirois D, Zillikens D, Werth VP. Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus. J Am Acad Dermatol 2008;58(6):1043–6.PubMedCrossRefGoogle Scholar
  9. 9.
    Jabbari A, Cheung W, Kamino H, Soter NA. Interstitial granulomatous dermatitis with arthritis. Dermatol Online J 2009;15(8):22.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Department of Allergy and ImmunologyJohns Hopkins Medicine International,Tokyo Midtown Medical CenterTokyoJapan

Personalised recommendations