SP0073 DIAGNOSIS OF GASTROINTESTINAL VASCULITIS
Authors: Maria C Cid
Journal: Annals of the Rheumatic Diseases (2019)

<h3></h3> The gastrointestinal vasculature can be involved by a variety of vasculitis affecting large, medium, small or variable vessel size according to the 2012 revised Chapel Hill consensus nomenclature. Gastrointestinal involvement (GI) is one of the most severe complications of vasculitis requiring intensive immunosuppressive therapy and sometimes revascularization techniques or emergency surgery and may affect a variety of organs including large or small intestines, liver, biliary tract, pancreas, and less frequently, stomach or oesophagus. It can complicate the course of patient with known or with overt suspicion of vasculitis or be the main or even the only manifestation of these diseases. Clinical manifestations range from abdominal pain, sometimes colicky, that usually worsens after eating to overt signs of bowel ischaemia or perforation with diffuse and constant abdominal pain, reduced peristaltism, abdominal defence and distension, and signs of peritoneal irritation. GI bleeding, frequently in the form of occult blood loss or haematochezia and less frequently melena or rectorragia, is common. Non-specific symptoms such as nausea, vomiting or diarrhoea may be observed. Pancreatitis, cholecystitis or appendicitis may also occur. In large-vessel vasculitis, including giant-cell arteritis (GCA) and Takayasu disease (TAK), inflammation usually affects the proximal portion of the celiac trunk, hepatic or mesenteric arteries. GI involvement is rare in GCA with only isolated cases or small series having been reported. About 30% of patients with GCA have elevated hepatic enzymes as part of the acute phase response and rarely reflect involvement of the hepatic vasculature. Imaging techniques disclose stenotic or occlusive lesions in 25% of patients with TAK but clinically manifest involvement occur in about 16% of patients. Abdominal pain suggestive of vascular insufficiency is present in about 16% of patients; abdominal bruits can be perceived in 14% but only 4% present with overt signs of mesenteric ischemia. Due to the involvement of the proximal part of the GI branches, the ischemic territories may be extensive but usually progression of vascular occlusion is not abrupt, and due to the size of vessels involved, patients may benefit from percutaneous revascularization procedures if immunosuppressive treatments are not sufficient. Interestingly 5% of patients with TAK also suffer from inflammatory bowel disease, particularly Crohn"s disease. In medium sized vasculitis, such as polyarteritis nodosa (PAN) or Kawasaki disease (KD), multiple muscular artery branches of the GI vasculature can be involved. Abdominal pain is frequent in PAN (35-95% of cases). GI involvement is more frequent in HBV-associated PAN than in idiopathic PAN. About 61% of patient with KD may have GI symptoms but these are severe in 4.6% only. Gallbladder hydrops and appendicular vasculitis may occur. The presence of prominent GI manifestations is more frequent in patients with coronary aneurysms indicating more severe vasculitic disease. In small-vessel vasculitis, including anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)(granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA], and eosinophilic granulomatosis with polyangiitis [EGPA]) and immune-complex-mediated vasculitis (IgA vasculitis [Henoch-Shönlein purpura] and cryoglobulinemic vasculitis) the microvasculature is segmentally affected and the severity of involvement may be variable in different areas. Among AAV, GI involvement is more frequent in patients with EGPA (35-50%), than MPA (5-30%) or GPA (5-11%). In EGPA, GI involvement can be vascular as in other vasculitis or may be in the form of eosinophilic gastroenteritis or esophagitis. In GPA, in addition to vascular involvement, granulomatous hepatitis or pancreatitis may occasionally occur. Among immune-complex vasculitis, GI involvement is typical in IgA vasculitis, occurring in 50-75% of patients. Although it is usually mild and self-limited, complications including bleeding, ischaemia, perforation or intussusception may appear in 3-5% of patients. Hepatobiliary involvement is uncommon (1.8%). GI involvement is rare in cryoglobulinemic vasculitis but is life-threatening when it occurs. Among the category of variable vessel size vasculitis, GI involvement in Behçet"s disease (BD) may consist of mucosal ulcers, frequently in the distal ileum, similar to inflammatory bowel disease, or involvement of mesenteric artery branches as in other vasculitis. Venous thrombosis involving the liver vasculature or GI tract may also occur in BD. Vasculitis involvement may be restricted to different portions of the GI tract (gallbladder, appendix, intestines) in patients with no obvious evidence of systemic vasculitis. Gallbladder is the more frequent location and is usually incidentally discovered after surgery. Overt systemic vasculitis may develop in up to 25% of patients during follow-up. Diagnosis of GI involvement in systemic vasculitis requires clinical suspicion. Imaging (computed tomography [CT]-angiography[CTA] or magnetic resonance angiography [MRA] are crucial in large vessel vasculitis evidencing concentric thickening with contrast enhancement of the vessel wall, along with lumen reduction. In medium vessel vasculitis CT may disclose hepatic, spleen or renal infarcts that reinforce clinical suspicion of vascular disease. However, CTA or MRA may not have enough resolution to evidence involvement of medium-sized branches and conventional angiography is usually needed, revealing vascular stenosis an occlusions as well as microaneurysms. In small-vessel vasculitis, and in all vasculitis conveying intestinal ischemia, CT may reveal thickening of the intestine wall, dilatation, and, in severe cases, pneumatosis of the intestine wall as well as gas in the portal system. Pneumo-peritoneum can be seen in the case of perforation. Ascites is not uncommon in medium and small vessel vasculitis. Occasionally, hemorrhage in the peritoneal cavity due to rupture of aneurysms can be observed. It is important to remark that imaging findings provide strong support to the clinical suspicion of vasculitis based on additional clinical, blood test or serologic findings or evidence of vasculitis elsewhere but similar findings can be observed in the context of a variety of vasculopathies or thrombophilic/embolic disorders that may mimic vasculitis. Endoscopy is also a useful exploration in some settings. It may detect intestinal purpura, usually in IgA vasculitis, mucosal ulcers or mucosal inflammation. Endoscopic biopsies may reveal eosinophilic inflammation in EGPA but may not be deep enough to disclose inflamed blood vessels and its absence does not exclude vasculitis. In case of surgical complications, a throughout pathologic examination of the removed specimen should be requested in order to evidence of vascular inflammation. GI involvement in systemic vasculitis is frequently a severe and potentially life-threating complication. Prompt detection and intense immunosuppressive, revascularization or surgical treatment in the appropriate settings are crucial for a successful recovery and prevention of irreversible damage. MCC and GEF are supported by Ministerio de Ciencia (SAF 2017/88275-R) and Instituto de salud Carlos III/FEDER (PI18/00461) respectively. <h3>References:</h3> [1] Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013 Jan;65(1):1-11. [2] Soowamber M, Weizman AV, Pagnoux C. Gastrointestinal aspects of vasculitides. Nat Rev Gastroenterol Hepatol. 2017 Mar;14(3):185-194. [3] Chetty R, Serra S. A pragmatic approach to vasculitis in the gastrointestinal tract. J Clin Pathol. 2017 Jun;70(6):470-475 [4] Schmidt J, Kermani TA, Bacani AK, Crowson CS, Cooper LT, Matteson EL, et al. Diagnostic features, treatment, and outcomes of Takayasu arteritis in a US cohort of 126 patients. Mayo Clin Proc. 2013 Aug;88(8):822-30 [5] Kronzer VL, Larson DP, Crowson CS, Warrington KJ, Ytterberg SR, Makol A, et al. Occurrence and aetiology of gastrointestinal perforation in patients with vasculitis. Clin Exp Rheumatol. 2019 Jan 9. [Epub ahead of print] [6] Fabi M, Corinaldesi E, Pierantoni L, Mazzoni E, Landini C, Bigucci B, et al. Gastrointestinal presentation of Kawasaki disease: A red flag for severe disease? PLoS One. 2018 Sep 4;13(9):e0202658. [7] Retamozo S, Díaz-Lagares C, Bosch X, Bové A, Brito-Zerón P, Gómez ME, et al. Life-threatening cCryoglobulinemic patients with hepatitis C: clinical description and outcome of 279 patients. Medicine (Baltimore). 2013 Sep;92(5):273-284. [8] Hernández-Rodríguez J, Hoffman GS. Updating single-organ vasculitis. Curr Opin Rheumatol. 2012 Jan;24(1):38-45. 9) [9] Ghodasara N, Liddell R, Fishman EK, Johnson PT. High-value multidetector CT angiography of the superior mesenteric artery: what emergency medicine physicians and interventional radiologists need to know. Radiographics. 2019 Mar-Apr;39(2):559-577. <h3>Disclosure of Interests:</h3> None declared