Acute loss of Vision due to Severe Bilateral Uveitis as the Presenting Symptom of Crohn's Disease

Marc Girardin; Kevin A Waschke; Ernest G Seidman


Nat Clin Pract Gastroenterol Hepatol. 2007;4(12):695-698. 

In This Article

Discussion of Diagnosis

Extraintestinal manifestations occur in about 25% of IBD patients,[1] but ocular symptoms are observed in only 5-10% of all IBD cases. The most frequent ocular symptoms are episcleritis (29%), scleritis (18%) and uveitis (17%; Figure 1).[2] Ocular manifestations are more frequently associated with Crohn's disease than ulcerative colitis: uveitis has been reported in 6.4% of cases of Crohn's disease, compared with 4.6% of patients with ulcerative colitis.[1] Extraintestinal manifestations of IBD are reported to be the first symptoms at presentation in only 10-20% of cases overall.[3] Several cases of ocular manifestations have been reported as a first symptom of Crohn's disease, but none includes uveitis. In the present case, the impairment of vision owing to bilateral anterior uveitis occurred before symptoms of the primary intestinal disease. Interestingly, uveitis has been reported to be the first symptom in 41% of cases of spondyloarthritis.[4] This finding is notable because 5-8% of patients with spondyloarthropathies also have IBD.[5]

(A) Representative case of diffuse episcleritis. Diffuse inflammation with dilatation of the vessels of the blood-rich episcelra, the layer directly beneath the conjunctiva. (B) Representative case of scleritis, a more severe disorder that might impair vision. The vascular irregularities and distortion are deeper and the sclera between vessels typically appears pink, rather than white. (C) Representative case of anterior uveitis, similar to that observed in the reported case. The superficial conjunctival injection extends to the limbus (ciliary flush), indicative of intraocular inflammation. Inflamm Bowel Dis 10: 135-139. Copyright © 2004 Mintz R et al. Reprinted with permission of John Wiley & Sons, Inc.

Uveitis is defined as inflammation of the vascular coat of the eye—anteriorly in the iris (iritis) and ciliary body (cyclitis), and posteriorly in the vitreous (vitritis), choroid and retina. Uveitis is acute, anterior, low grade and recurrent in 60% of cases, 30% of cases have panuveitis (inflammation of the anterior and posterior segments) and only 10% of cases have an isolated episode. Whether uveitis is related to active bowel disease is unclear. The condition has been described as occurring during active bowel disease, during quiescent periods and preceding the diagnosis of IBD.[6] In the largest study published to date, Veloso et al.[1] reported uveitis in 5.6% of 792 IBD patients followed up prospectively. In 78.5% of these cases, symptoms occurred simultaneously with active intestinal disease. Uveitis is independent of the site and extent of bowel involvement[7] and reported more commonly in women than men.[8] In IBD patients, ocular inflammation is predominantly anterior. This pattern is in contrast to that of other inflammatory disorders, such as Behçet's disease, in which the inflammation is predominantly posterior.[9]

Patients presenting with uveitis are more likely to manifest other extraintestinal symptoms, such as arthritis or pyoderma gangrenosum, although these manifestations were not observed in the present case. Uveitis and ankylosing spondyloarthritis have both been associated with the HLA-B27 genotype.[9] In both these conditions, the clinical course of the IBD might be independent of the extraintestinal disorder, similar to the present case, in which a relapse of the patient's Crohn's disease was unassociated with uveitis.[6,9] One hypothesis to explain these intestinal and extraintestinal associations is that of a common antigen in the colon, eye and joints that triggers an autoimmune reaction. The uvea is a highly vascularized tissue, rich in antigen-presenting cells.[10] The presence of polymorphisms of the tumor necrosis factor gene on chromosome 6 in proximity to the HLA genes has been linked to erythema nodosum.[11] Mutations in this region might, therefore, explain the simultaneous manifestations within and outside the intestine.


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