Overview of gastrointestinal manifestations of vasculitis
- Michael D Apstein, MD
Michael D Apstein, MD
- Assistant Professor of Medicine
- Harvard Medical School
- Section Editor
- Peter A Merkel, MD, MPH
Peter A Merkel, MD, MPH
- Section Editor — Vasculitis
- Chief, Division of Rheumatology
- University of Pennsylvania
- Deputy Editors
- Monica Ramirez Curtis, MD, MPH
Monica Ramirez Curtis, MD, MPH
- Deputy Editor — Rheumatology
- Instructor of Medicine, Part-time
- Harvard Medical School
- Shilpa Grover, MD, MPH, AGAF
Shilpa Grover, MD, MPH, AGAF
- Deputy Editor — Gastroenterology/Hepatology
- Assistant Professor of Medicine, Part-time
- Harvard Medical School
The vasculitides are defined by the presence of inflammatory leukocytes in vessel walls with reactive damage to mural structures. Symptomatic involvement of the gastrointestinal tract may occur either in isolation or in combination with multiple organs. Vasculitis should be considered in patients when the history, physical exam, laboratory, and/or radiologic data indicate that multiple organ systems are involved. However, the diagnosis may be confirmed only after pathologic examination of excised tissue due to ischemia, infarction, or perforation of the involved portion of the gastrointestinal tract.
This topic will review the clinical features and evaluation of patients with gastrointestinal vasculitis. The major vasculitides that may present with gastrointestinal involvement are also described. The diagnosis and treatment of major vasculitides are discussed in detail separately. (See "Overview of and approach to the vasculitides in adults" and "Clinical manifestations and diagnosis of polyarteritis nodosa in adults" and "Gastrointestinal manifestations of systemic lupus erythematosus" and "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis" and "Clinical manifestations and diagnosis of the mixed cryoglobulinemia syndrome (essential mixed cryoglobulinemia)".)
While several vasculitides may be associated with gastrointestinal involvement, it is most prevalent in patients with immunoglobulin A vasculitis (Henoch-Schönlein purpura), antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, polyarteritis nodosa (PAN), and Behçet's syndrome . In three series with a total of 351 patients with systemic vasculitis, approximately one-third had gastrointestinal manifestations [2-4].
MAJOR VASCULITIDES WITH GASTROINTESTINAL INVOLVEMENT
The frequency and type of gastrointestinal manifestations vary among the vasculitides. Symptoms of vasculitis involving the gastrointestinal tract result from ischemia to the affected organ. Although vasculitis is frequently classified by the size of the vessels involved, the symptoms from either large-, medium-, or small-vessel vasculitis can overlap. As such, abdominal pain, nausea, vomiting, diarrhea, and/or gross or occult blood in the stool is common. Gross bleeding is more commonly seen in vasculitis involving the colon compared with that involving the small bowel, but there is considerable overlap of symptoms so it is difficult to determine clinically which portion of the gastrointestinal tract is involved.
The major vasculitides with gastrointestinal involvement are discussed below, using the international Chapel Hill Consensus Conference (CHCC) classification categories. (See "Overview of and approach to the vasculitides in adults".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- MAJOR VASCULITIDES WITH GASTROINTESTINAL INVOLVEMENT
- Large-vessel vasculitis
- - Takayasu arteritis
- - Giant cell (temporal) arteritis
- Medium-vessel vasculitis
- - Polyarteritis nodosa
- Small-vessel vasculitis
- - ANCA-associated vasculitis
- - Immunoglobulin A vasculitis (Henoch-Schönlein purpura)
- - Cryoglobulinemic vasculitis
- Variable-vessel vasculitis
- - Behçet's syndrome
- Single-organ vasculitis
- Vasculitis associated with systemic disease
- - Rheumatoid vasculitis
- - Systemic lupus erythematosus
- CLINICAL PRESENTATION
- DIFFERENTIAL DIAGNOSIS
- DIAGNOSTIC EVALUATION
- Our approach
- Laboratory testing
- Vascular imaging
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS