Gastrointestinal manifestations of vasculitis
- Michael D Apstein, MD
Michael D Apstein, MD
- Assistant Professor of Medicine
- Harvard Medical School
Vasculitis involving the gastrointestinal tract is usually part of a systemic process, although the signs and symptoms may initially be limited. (See "Overview of and approach to the vasculitides in adults".)
This topic review will cover the clinical manifestations and approach to the diagnosis of gastrointestinal vasculitis. The individual disorders, such as polyarteritis nodosa (PAN) and systemic lupus erythematosus (SLE), are discussed in detail elsewhere, as are systemic vasculitides without prominent gastrointestinal manifestations, such as granulomatosis with polyangiitis (Wegener’s) and mixed cryoglobulinemia. The latter disorder is of interest to gastroenterologists because it usually represents an extrahepatic manifestation of hepatitis C virus infection. (See "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)".)
The signs and symptoms of systemic vasculitis involving the gastrointestinal tract result from mesenteric ischemia and/or ischemic colitis. The presentation of patients with ischemic colitis secondary to vasculitis is similar to that of patients with ischemic colitis from other etiologies in that both present with rectal bleeding. In contrast to patients with chronic mesenteric ischemia due to a low flow state, who usually give a history of chronic abdominal pain, patients with vasculitis involving the gastrointestinal tract may present with acute abdominal pain. Similar to patients with chronic mesenteric ischemia, these patients frequently have pain that is out of proportion with findings on abdominal exam before intestinal infarction occurs. Clinicians should consider gastrointestinal vasculitis in this setting in the absence of a source of embolization. (See "Overview of intestinal ischemia in adults" and "Chronic mesenteric ischemia".)
In three series with a total of 351 patients, approximately one-third had gastrointestinal manifestations [1-3]. In one large series of patients with polyarteritis nodosa (PAN) or eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss), for example, the following frequency of gastrointestinal symptoms and findings was noted :
●Abdominal pain – 25 percent
Subscribers log in hereLiterature review current through: Aug 2017. | This topic last updated: Oct 26, 2016.References
- Guillevin L, Le Thi Huong Du, Godeau P, et al. Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in 165 patients. Br J Rheumatol 1988; 27:258.
- Scott DG, Bacon PA, Elliott PJ, et al. Systemic vasculitis in a district general hospital 1972-1980: clinical and laboratory features, classification and prognosis of 80 cases. Q J Med 1982; 51:292.
- Lopez LR, Schocket AL, Stanford RE, et al. Gastrointestinal involvement in leukocytoclastic vasculitis and polyarteritis nodosa. J Rheumatol 1980; 7:677.
- Pagnoux C, Mahr A, Cohen P, Guillevin L. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore) 2005; 84:115.
- Kuehne SE, Gauvin GP, Shortsleeve MJ. Small bowel stricture caused by rheumatoid vasculitis. Radiology 1992; 184:215.
- Vaglio A, Corradi D, Ronda N, et al. Large bowel obstruction heralding Churg-Strauss syndrome. Am J Gastroenterol 2004; 99:562.
- Ebert EC, Hagspiel KD, Nagar M, Schlesinger N. Gastrointestinal involvement in polyarteritis nodosa. Clin Gastroenterol Hepatol 2008; 6:960.
- Buffo GC, Deitch JS. Pneumatosis intestinalis in a patient with polyarteritis nodosa. Gastrointest Radiol 1986; 11:286.
- Lee EL, Smith HJ, Miller GL 3rd, et al. Ischemic pseudomembranous colitis with perforation due to polyarteritis nodosa. Am J Gastroenterol 1984; 79:35.
- Levine SM, Hellmann DB, Stone JH. Gastrointestinal involvement in polyarteritis nodosa (1986-2000): presentation and outcomes in 24 patients. Am J Med 2002; 112:386.
- Zizic TM, Classen JN, Stevens MB. Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodosa. Am J Med 1982; 73:525.
- Fauci AS, Katz P, Haynes BF, Wolff SM. Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Engl J Med 1979; 301:235.
- Kruger M, Böker KH, Zeidler H, Manns MP. Treatment of hepatitis B-related polyarteritis nodosa with famciclovir and interferon alfa-2b. J Hepatol 1997; 26:935.
- Carpenter MT, West SG. Polyarteritis nodosa in hairy cell leukemia: treatment with interferon-alpha. J Rheumatol 1994; 21:1150.
- Glasier CM, Siegel MJ, McAlister WH, Shackelford GD. Henoch-Schonlein syndrome in children: gastrointestinal manifestations. AJR Am J Roentgenol 1981; 136:1081.
- Goldman LP, Lindenberg RL. Henoch-schoenlein purpura. Gastrointestinal manifestations with endoscopic correlation. Am J Gastroenterol 1981; 75:357.
- Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, et al. Henoch-Schönlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum 1997; 40:859.
- Love PE, Santoro SA. Antiphospholipid antibodies: anticardiolipin and the lupus anticoagulant in systemic lupus erythematosus (SLE) and in non-SLE disorders. Prevalence and clinical significance. Ann Intern Med 1990; 112:682.
- Cappell MS. Esophageal necrosis and perforation associated with the anticardiolipin antibody syndrome. Am J Gastroenterol 1994; 89:1241.
- Gaspari JC, Sande JR, Thomas CF Jr, et al. Lupus anticoagulant masquerading as an acute abdomen with multiorgan involvement. Am J Gastroenterol 1995; 90:825.
- Cappell MS. Atypical duodenal ulcer associated with the antiphospholipid antibody syndrome. Am J Gastroenterol 1997; 92:722.
- Richardson SC, Willis J, Wong RC. Ischemic colitis, systemic lupus erythematosus, and the lupus anticoagulant: case report and review. Gastrointest Endosc 2003; 57:257.
- Lockshin MD. Antiphospholipid antibody. Babies, blood clots, biology. JAMA 1997; 277:1549.
- Babian M, Nasef S, Soloway G. Gastrointestinal infarction as a manifestation of rheumatoid vasculitis. Am J Gastroenterol 1998; 93:119.
- Griffin JW Jr, Harrison HB, Tedesco FJ, Mills LR 4th. Behcet's disease with multiple sites of gastrointestinal involvement. South Med J 1982; 75:1405.
- Anti M, Marra G, Rapaccini GL, et al. Esophageal involvement in Behçet's syndrome. J Clin Gastroenterol 1986; 8:514.
- Powderly WG, Lombard MG, Murray FE, et al. Oesophageal ulceration in Behçet's disease presenting with haemorrhage. Ir J Med Sci 1987; 156:193.
- Stringer DA, Cleghorn GJ, Durie PR, et al. Behçet's syndrome involving the gastrointestinal tract--a diagnostic dilemma in childhood. Pediatr Radiol 1986; 16:131.
- Le Thi Huong D, Wechsler B, Dell'Isola B, et al. Acute pancreatitis in Behçet's disease. Dig Dis Sci 1992; 37:1452.
- Wakefield AJ, Ekbom A, Dhillon AP, et al. Crohn's disease: pathogenesis and persistent measles virus infection. Gastroenterology 1995; 108:911.
- Zlatanic J, Fleisher M, Sasson M, et al. Crohn's disease and acute leukocytoclastic vasculitis of skin. Am J Gastroenterol 1996; 91:2410.
- Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, et al. Takayasu's arteritis. Clinical study of 107 cases. Am Heart J 1977; 93:94.
- Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990; 33:1129.
- Jafri FM, Mendelow H, Shadduck RK, Sekas G. Jejunal vasculitis with protein-losing enteropathy after bone marrow transplantation. Gastroenterology 1990; 98:1689.
- Perlemuter G, Chaussade S, Soubrane O, et al. Multifocal stenosing ulcerations of the small intestine revealing vasculitis associated with C2 deficiency. Gastroenterology 1996; 110:1628.
- Gaburri PD, Assis RV, Ferreira LE. Intestinal ulcers due to drug-induced perinuclear anti-neutrophilic cytoplasmic antibody-positive vasculitis and antiphospholipid syndrome. Clin Gastroenterol Hepatol 2005; 3:A28.
- Khurshid Y, Kathula S, Hillman N, et al. A case of bleeding jejunal ulcer due to vasculitis from hydroxyurea. Am J Gastro 2000; 95:2616.
- Seibold F, Slametschka D, Gregor M, Weber P. Neutrophil autoantibodies: a genetic marker in primary sclerosing cholangitis and ulcerative colitis. Gastroenterology 1994; 107:532.
- Bansi DS, Fleming KA, Chapman RW. Importance of antineutrophil cytoplasmic antibodies in primary sclerosing cholangitis and ulcerative colitis: prevalence, titre, and IgG subclass. Gut 1996; 38:384.
- Mulder AH, Horst G, Haagsma EB, et al. Prevalence and characterization of neutrophil cytoplasmic antibodies in autoimmune liver diseases. Hepatology 1993; 17:411.
- Schmidt WA. Use of imaging studies in the diagnosis of vasculitis. Curr Rheumatol Rep 2004; 6:203.
- Harre RG, Conrad GR, Seabold JE. Colonic localization of indium-111 labeled leukocytes in active Behcet's disease. Clin Nucl Med 1988; 13:459.
- Stancanelli B, Vita A, Vinci M, et al. Bleeding of small bowel in Henoch-Schönlein syndrome: the successful diagnostic role of video capsule endoscopy. Am J Med 2006; 119:82.
- SPECIFIC DISORDERS
- Polyarteritis nodosa
- Eosinophilic granulomatosis with polyangiitis
- Henoch-Schönlein purpura (IgA vasculitis)
- Systemic lupus erythematosus
- - Antiphospholipid syndrome
- Rheumatoid vasculitis
- Behçet's syndrome
- Inflammatory bowel disease associated vasculitis
- Takayasu arteritis
- Jejunal vasculitis
- Drug-induced vasculitis
- EVALUATION OF THE PATIENT
- Imaging studies
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS