Table 1 Vasculitides with detected complications on enterography
Vasculitis | Clinical clues | Imaging pearl for the radiologists | Mimickers on MRE/CTE |
|---|---|---|---|
Large-vessel vasculitis | Â | Â | Â |
Takayasu arteritis | Age at onset ≤ 40 y Claudication of the extremities Decreased brachial artery pulse Blood pressure difference > 10 mm Hg between the arms Bruit over the subclavian arteries or aorta Abdominal bruit in 14% | Involvement of the aorta and the origin of major visceral arteries, presenting with concentric mural thickening, transmural calcification, luminal stenosis, occlusion, aneurysmal changes, and collateral vessels formation Double ring vascular enhancement pattern Non-specific intestinal mural thickening, submucosal edema, and enhancement | Mesenteric ischemia due to other etiologies Atherosclerosis Connective tissue disorders  Marfan syndrome  Ehlers-Danlos syndrome  Loeys-Dietz syndrome |
Giant cell arteritis | Patient age > 50 years New-onset headache Temporal artery abnormality (tenderness or decreased pulsation) ESR ≥ 50 mm/h Abnormal temporal artery biopsy results Abdominal pain may indicate aorta aneurysm or dilation | Signs of mesenteric ischemia | |
Medium-vessel vasculitis | Â | Â | Â |
Polyarteritis nodosa | Disease-associated weight loss ≥ 4 kg Livedo reticularis Testicular pain or tenderness Myalgia, weakness, or leg tenderness Mono- or polyneuropathy Diastolic blood pressure > 90 mm Hg Elevated serum levels of creatinine or blood urea nitrogen Presence of hepatitis B reactants in serum Biopsy of a small- or medium-sized artery containing neutrophils or mixed leukocyte infiltrate | Involvement of mesenteric and visceral arteries, e.g., stenosis, vessel irregularity, aneurysmal dilation, and arterial mural thickening, with a predilection for superior mesenteric artery (SMA) branches Segmental bowel mural thickening Submucosal edema and mural hyperenhancement with a striated pattern Visceral infarction (liver, spleen, kidneys, and intestine) Spontaneous abdominal hemorrhage secondary to a ruptured aneurysm Heterogeneous liver or renal enhancement due to microvascular abnormalities | Segmental arterial mediolysis (SAM) Fibromuscular dysplasia (FMD) Mycotic aneurysm |
Small-vessel vasculitis | Â | Â | Â |
ANCA- associated vasculitis | Â | Â | Â |
Granulomatosis with polyangiitis (Wegener's granulomatosis) | Nasal and oral inflammation: oral ulcers or bloody nasal discharge Microhematuria Granulomatous inflammation of arterial walls or extravascular tissue | Intestinal ischemia presenting with multi-focal segmental circumferential mural thickening and abnormal hyperenhancement Abnormal chest radiographic findings: multiple and bilateral pulmonary nodules fixed infiltrates, or masses, or alveolar hemorrhage Renal pathologies, i.e., ischemia and interstitial nephritis Visceral infarction (liver, spleen, and kidneys) | Non-occlusive mesenteric ischemia Infectious enteritis Eosinophilic enteritis Inflammatory bowel disease Radiation and chemotherapy induced enteritis |
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) | Asthma Eosinophilia > 10% Mono- to polyneuropathy Nonfixed pulmonary infiltrates Paranasal sinus abnormality Extravascular eosinophils | Submucosal edema and mural hyperenhancement with a striated pattern with or without thickening (halo sign) Bowel dilatation, stenosis, or obstruction Hepatobiliary complications, i.e., cholestasis and liver infarction Pulmonary involvement (Non-Fixed parenchymal opacities) | |
Microscopic polyangiitis | Rapid progressive glomerulonephritis and/or alveolar hemorrhages Histopathologic findings of small vessel vasculitis or necrotizing glomerulonephritis Symptoms suggestive of small vessel involvement Skin lesions (commonly presented as palpable purpura) Neurological manifestations | Concentric mural thickening Post-contrast T1-weighted hyperenhancement Engorgement of mesenteric vessels Signs of bowel infarction or perforation Renal pathologies (striated pattern) Pulmonary pathologies | |
Immune complex-associated vasculitis | Â | Â | |
IgA vasculitis | Age ≤ 20 years at disease onset Palpable purpura Acute abdominal pain Biopsy showing granulocytes in the walls of small vessels (presence of 2 or more) Arthralgia and/or arthritis Glomerulonephritis | Signs of bowel ischemia and edema, e.g., segmental mural thickening and post-contrast focal mural hypo-enhancement Self-limiting mucosal and submucosal hemorrhage Signs of bowel perforation, obstruction, or irreducible intussusception Gallbladder wall thickening | |
Variable-vessel vasculitis | Â | Â | Â |
Behçet disease | Mandatory criteria Recurrent oral aphthosis Minor criteria Ocular lesions Recurrent genital aphthosis Skin lesions Positive pathergy test | Predominant ileocecal involvement Irregular circumferential mural thickening with homogeneous mural enhancement Deep penetrating ulcers and restricted diffusion on DWI in the involved section No specific predilection to the mesenteric side No surrounding mesenteric inflammatory changes | Non-occlusive mesenteric ischemia Infectious enteritis Eosinophilic enteritis Inflammatory bowel disease Radiation and chemotherapy-induced enteritis |
Vasculitis associated with systemic diseases | Â | Â | Â |
Systemic Lupus Erythematosus (SLE) | Synovitis or tenderness in at least two joints Cutaneous presentations, e.g., malar rash, discoid rash, and photosensitivity Oral ulcers Hematologic abnormalities, including thrombocytopenia, autoimmune hemolysis, and leukopenia Neurological involvement, such as seizure, delirium, or psychosis Proteinuria or lupus nephritis Low C3 and/or C4 Anti dsDNA antibody and/or Anti-Smith antibody positive | Multi-focal bowel wall thickening not confined to a single vascular territory Submucosal edema and mural hyper enhancement Target sign (diffuse circumferential wall thickening with submucosal edema) Evidence of serositis, e.g., ascites Comb sign (The hypervascular appearance of the mesentery) Genitourinary involvement, e.g., hydronephrosis, cystitis, and lupus nephritis Signs of solid-organ infarction, including wedge-shaped renal and splenic infarcts | Non-occlusive mesenteric ischemia Infectious enteritis Eosinophilic enteritis Inflammatory bowel disease Radiation and chemotherapy-induced enteritis |
Systemic sclerosis | Skin thickening of the fingers Fingertip lesions Telangiectasia Abnormal nailfold capillaries Pulmonary arterial hypertension and/or Interstitial lung Disease Raynaud's phenomenon anti-centromere and/or anti-topoisomerase antibody positive | Benign or sterile pneumatosis Hidebound sign (increased number of bowel folds stacked together despite luminal distention without an increase in interfold distance) Diffuse dilation of the small intestine (particularly jejunum) Intestinal mural thickening, indicating mural fibrosis Multiple wide-mouthed outpouchings involving all intestinal wall layers Subcutaneous calcifications |