Takayasu`s Arteritis and Inflammatory Bowel Diseases: Different Immune-Mediated Disorders or a Rare Extra-Intestinal Manifestation?
BACKGROUND: Takayasu's arteritis (TA) is a chronic granulomatous inflammatory vasculitis of unknown etiology that affects large arteries, especially the aortic arch and its branches, and is rarely associated with inflammatory bowel disease (IBD) (1). In most cases where this association is described in the literature, TA occurs after the diagnosis of IBD, with a time lapse of 10 to 36 months, and maintains a positive correlation with the activity of intestinal disease (2). This study aims to report a series of six cases of concomitant TA and IBD, determining its clinical features, diagnostic methods, their relationship with intestinal disease activity and responses according to therapeutic interventions. The authors also performed a review of the current literature in this field.
METHODS: Patients were identified at our IBD unit through electronic medical record searches including the keywords “Takayasu”, “arteritis” and “vasculitis”. Data regarding patient characteristics, disease phenotype and treatment were retrospectively collected from identified patients using a standardized chart review.
RESULTS: Six patients (50% male) with TA were identified in a total of 2,749 patients with IBD (46.8% CD, 53, 2% UC). Four patients had Crohn’s disease (CD) and 2 had ulcerative colitis (UC). The mean age of the patients at diagnosis was 33 years old (18–63). In 5 patients, TA occurred after the diagnosis of IBD, and 4 patients had another associated extra-intestinal manifestation. Vascular surgery was required in 2 patients due to extensive lesions and inflammatory activity. Most patients were clinically treated with corticosteroids, immunomodulators and anti-TNF agents. Simultaneous occurrence of both AT and CD is quite rare (1 in 10 billion) (3). However, the existence of common physiopathological pathways indicates that these conditions may be strongly associated 4. Moreover, since both diseases can be treated with the same agents, diagnostic delay may occur.
CONCLUSIONS: The report of this case series reinforce, based on the literature reviewed, that a linkage among TA and IBD may exist. In view of the limitations of the retrospective study design further investigations are needed. Moreover, the possible role of genetic links and immune factors (T helper 1 and T helper 17 cells, cytokines, chemokines) should be explored.