Young Investigator: A Rare Case of Crohnʼs Disease-Associated Aortitis

Abstract

RESULTS: A 33 year-old woman with history of Crohn's disease complicated by enteropathic arthropathies presented with symptoms of complete gastrointestinal obstruction. She had history of ongoing ileocolitis despite therapy with certolizumab. Due to persistent obstruction symptoms, she underwent an uneventful laparoscopic ileocecectomy. On post-operative day number two, she developed fever, chest pain, tachycardia and shortness of breath. Her WBC was elevated to 10 K/microL, with CRP 10 mg/L, total IgG and IgG2 noted to be elevated with normal IgG4 levels. Interferon release assay for tuberculosis was negative. A chest CT revealed ascending aortitis, which was later confirmed with MR angiography. She was started on high-dose corticosteroids and ultimately transitioned to infliximab (5 mg/kg) and subcutaneous methotrexate with resolution of aortitis on follow-up imaging. While her aortitis has remained in remission, her intestinal Crohn's symptoms and arthralgias are recurring despite recently increasing infliximab dose to 10mg/kg every 6 weeks, with therapeutic levels and no evidence of anti-infliximab antibodies. CONCLUSION(S): Extraintestinal manifestations (EIM) of inflammatory bowel disease are commonly found in approximately one-third of patients. They typically manifest as peripheral or axial arthropathies, dermatologic manifestations including pyoderma gangrenosum and erythema nodosum, ocular involvement including uveitis or episcleritis, and primary sclerosing cholangiopathy. While very uncommon, there also appears to be some degree of overlap between Crohn's disease and large vessel vasculitides. Initial evaluation should exclude diagnoses of Takayasu's arteritis, Behcet's disease, sarcoidosis, ankylosing spondylitis, and connective tissue disorders. Infectious etiologies such as tuberculosis and syphilis should also be eliminated. Upon exclusion of these disease processes causing aortic inflammation, the diagnosis of a Crohn's-related EIM should be considered. Only a few cases of aortitis associated with Crohn's disease have been reported in the literature but this appears to be a very rare phenomenon. Severe cases of aortitis carry the risk of aortic rupture when inflammation is left untreated. Fortunately, aortitis appears to be readily responsive to high dose corticosteroid therapy, as was the case in our patient. If significant valvular disease is present (i.e. severe aortic regurgitation), this may require valvular replacement surgery. Interestingly, there have been reports of Crohn's-related aortitis developing in the setting of anti-TNF therapy, again requiring corticosteroid therapy for resolution. As such, we will need to monitor our patient closely to detect any signs of recurrent aortitis.