Refractory central nervous system vasculitis and gastrocnemius myalgia syndrome in Crohn's disease successfully treated with anti-tumor necrosis factor-alpha antibody.

Abstract

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.

CONCLUSION:

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.

• Authors: Sebastian Ullrich, Susanne Schinke, Markus Both, Karl Christian Knop, Nancy Coula Kirkiles-Smith, Wolfgang Ludwig Gross, Peter Lamprecht
• Journal: Seminars in arthritis and rheumatism (Semin Arthritis Rheum) Vol. 38 Issue 5 Pg. 337-47 (Apr 2009) ISSN: 1532-866X [Electronic] United States
• PMID: 18304612 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't, Review)

Chemical References

• Anti-Inflammatory Agents
• Antibodies, Monoclonal
• Antibodies, Monoclonal, Humanized
• Tumor Necrosis Factor-alpha
• Adalimumab

Topics

• Adalimumab
• Adult
• Anti-Inflammatory Agents (administration & dosage)
• Antibodies, Monoclonal (administration & dosage)
• Antibodies, Monoclonal, Humanized
• Crohn Disease (complications, drug therapy)
• Drug Resistance
• Female
• Humans
• Middle Aged
• Muscle, Skeletal (blood supply)
• Muscular Diseases (drug therapy, etiology)
• Tumor Necrosis Factor-alpha (antagonists & inhibitors)
• Vasculitis, Central Nervous System (drug therapy, etiology)