Refractory central nervous system vasculitis and gastrocnemius myalgia syndrome in Crohn's disease successfully treated with anti-tumor necrosis factor-alpha antibody.
BACKGROUND
Secondary vasculitis represents a rare extraintestinal manifestation of Crohn's disease (CD). Appropriate and prompt diagnosis is often delayed by uncertainties about the relationship of the vasculitic manifestations and CD.


OBJECTIVE
To describe our experience with vasculitis in CD and review the literature with respect to different manifestations and pathophysiological aspects of extraintestinal vasculitic manifestations of CD.


METHODS
We report 2 new cases of CD with secondary small-vessel vasculitis. We also extensively review the literature (1960-2007) using a broad range of key words related to secondary vasculitis in CD. Relevant publications were evaluated for the number of reported patients and manifestations of vasculitis.


RESULTS
Vasculitis is a rare extraintestinal manifestation of CD. Different types of vasculitis affect large-, medium-, and small-sized vessels associated with CD. Common immunologic features include intestinal inflammation as well as an infiltration of gammadelta-T-cells and/or Th1-type cells into vessel walls. The 2 new cases of secondary vasculitis in CD reported here reflect 2 major types of CD-related inflammatory vascular disorders. The first involves the central nervous system, while the second represents circumscribed Musculus gastrocnemius involvement (so-called "gastrocnemius myalgia syndrome"). Successful treatment of refractory secondary vasculitis in CD with an anti-tumor necrosis factor-alpha antibody is shown for the first time.


CONCLUSIONS
Vasculitis secondary to CD is an uncommon finding. Therefore, it has to be carefully differentiated from other forms of primary or secondary vasculitis with intestinal involvement. Treatment with an anti- tumor necrosis factor-alpha antibody may prove a treatment option in vasculitis as an extraintestinal manifestation of CD.
Ullrich
Sebastian
S
Department of Anatomy II, Center for Experimental Medicine, University Hospital of Hamburg, Hamburg, Germany. hsullrich@aol.com
Schinke
Susanne
S
Both
Markus
M
Knop
Karl Christian
KC
Kirkiles-Smith
Nancy Coula
NC
Gross
Wolfgang Ludwig
WL
Lamprecht
Peter
P
D000068879:Adalimumab; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D061067:Antibodies, Monoclonal, Humanized; D003424:Crohn Disease / Q000150:complications / Q000188:drug therapy*; D004351:Drug Resistance; D005260:Female; D006801:Humans; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000098:blood supply; D009135:Muscular Diseases / Q000188:drug therapy* / Q000209:etiology; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*; D020293:Vasculitis, Central Nervous System / Q000188:drug therapy* / Q000209:etiology
D000893:Anti-Inflammatory Agents; D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D014409:Tumor Necrosis Factor-alpha; D000068879:Adalimumab