BASICS
DESCRIPTION
• Inflammatory bowel disease (IBD) is an idiopathic inflammatory disease of the intestinal tract that is associated with a number of extra-intestinal manifestations. IBD can be classified as Crohn’s disease (CD) or ulcerative colitis (UC) based on the pathologic location of the diseased areas within the gut. Ocular manifestations of IBD are variable and can be evident as inflammation in all of the ocular tissues. The most common ocular associations include non-granulomatous anterior uveitis (e.g., iritis), episcleritis, scleritis, keratitis, retinal vasculitis, and posterior uveitis.
• Unless indicated, CD and UC will be discussed as part of the same clinical spectrum (IBD) in regard to the eye.
EPIDEMIOLOGY
Prevalence
• IBD has a prevalence of 70–150 cases per 100,000 persons.
• 3.5–11.8% of patients with IBD will have ocular inflammation at some point.
• Ophthalmic manifestations are infrequently the presenting complaint leading to a diagnosis of IBD.
• UC appears to have less associated ocular inflammation than CD.
• Episcleritis is the most common extra-intestinal manifestation of IBD (up to 29%) and is reported to be closely correlated to disease activity, but is not often seen by clinicians due to its relatively asymptomatic nature.
• Anterior uveitis is the most common extra intestinal manifestation of IBD seen by clinicians and is more common in women (1).
• Approximately 2% of all uveitis cases are reported to be secondary to IBD (2).
RISK FACTORS
• Active intestinal IBD is associated with, but not required for, ocular manifestations.
• Presence of other extra-intestinal manifestations, such as arthritis or erythema nodosum, are associated with a higher risk of ocular involvement (3).
• Genetic (see below)
Genetics
• IBD is familial but displays non-Mendelian inheritance and variable expressivity, likely reflecting a multifactorial genetic influence.
• HLA-B27 is present in approximately 8% of the Caucasian population and is associated with higher rates of acute anterior uveitis in general.
• Patients with IBD and HLA-B27 are at increased risk for ocular disease manifestations than IBD patients without HLA-B27 positivity.
– 50% of patients with IBD and anterior uveitis will be HLA-B27 positive.
– Ocular inflammation in IBD is also strongly associated with HLA-B58 and HLA-DRB10103 (3).
GENERAL PREVENTION
Control of IBD flares and chronic disease activity.
PATHOPHYSIOLOGY
An imbalance of inflammatory mediators leads to nonspecific inflammatory infiltrates in affected tissues, resulting in edema, local destruction, and tissue dysfunction.
ETIOLOGY
• Idiopathic
– IBD is generally thought to be secondary to an environmental trigger in genetically predisposed individuals that results in a pro-inflammatory response.
COMMONLY ASSOCIATED CONDITIONS
• HLA-B27 arthropathies
• Malabsorption syndromes
• Chronic anemia
• Osteoporosis
• Colon cancer
• Primary sclerosing cholangitis
• Erythema nodosum
• Depression
DIAGNOSIS
HISTORY
• Episcleritis
– Epibulbar injection, foreign body sensation. No pain or blurred vision
• Anterior uveitis
– Aching pain, photophobia, blurred vision
• Scleritis
– Deep boring pain, ocular tenderness, headache, photophobia, blurred vision
• Keratitis
– Sharp, superficial pain, photophobia. No other source of keratitis by history (contact lenses, trauma)
• Retinal vasculitis/posterior uveitis
– Aching pain, blurred vision. No infectious source (prior surgery, trauma, endogenous)
PHYSICAL EXAM
Slit-lamp examination
• Episcleritis
– Epibulbar injection that blanches with 2.5% phenylephrine. No anterior chamber cell and flare
• Anterior uveitis
– Ciliary flush (vascular injection adjacent to the limbus), anterior chamber cell and flare
• Scleritis
– Diffuse or nodular epibulbar injection (not conjunctival, usually does not blanch with phenylephrine 2.5%), severe tenderness to ocular palpation, anterior chamber cell and flare. Occasionally can be necrotizing and painless. Posterior scleritis can lead to an exudative retinal detachment
• Keratitis
– Conjunctival injection, corneal epithelial defect, corneal stromal infiltrates, anterior chamber cell and flare
• Retinal vasculitis/posterior uveitis
– Posterior chamber cell, vitreous snowballs or snowbanks, retinal vascular sheathing, macular edema
DIAGNOSTIC TESTS & INTERPRETATION
Lab
No lab tests are useful in the diagnosis of ocular manifestations in IBD.
Imaging
• If posterior scleritis is suspected, B-scan ultrasound of the eye may demonstrate a T-sign, caused by posterior peribulbar edema.
• If retinal vasculitis is suspected, a fluorescein angiogram preformed by a retina specialist may be useful.
DIFFERENTIAL DIAGNOSIS
• Infectious or allergic conjunctivitis
• Idiopathic anterior uveitis, episcleritis, scleritis, posterior uveitis, retinal vasculitis or optic neuritis; or one of the above associated with an alternate systemic inflammatory condition (sarcoidosis, tuberculosis, etc.)
• Infectious keratitis
TREATMENT
MEDICATION
First Line
• Episcleritis
– Artificial tears 1 drop q.i.d escalating to prednisolone acetate 1% 1drop q.i.d if needed
• Anterior uveitis
– Prednisolone acetate 1% 1 drop every hour, taper as inflammation is controlled. Scopolamine 0.25% or cyclopentolate 1% 1 drop twice a day for cycloplegia and synechiae prevention
• Scleritis
– Oral NSAIDs (ibuprofen 800 mg PO t.i.d or indomethacin 25 mg PO t.i.d).
– Topical steroids are generally not effective and may predispose to scleral thinning.
• Keratitis
– Epithelium intact – prednisolone acetate 1% frequency dependent on severity
– Epithelial loss – prednisolone acetate 1%, topical antibiotics, and possibly oral steroids
• Posterior uveitis/retinal vasculitis
– Oral steroids, intravitreal or sub-tenons steroid
Second Line
• Anterior uveitis, scleritis, keratitis, posterior uveitis, and retinal vasculitis.
– Oral steroids (4)
– Azathioprine, cyclosporine, methotrexate
– Anti-TNF agents (5)
Pregnancy Considerations
Patients who are pregnant should practice punctual occlusion when instilling topical eye drops to minimize systemic effects. Immunosuppressive therapy should be used with extreme caution in pregnant patients.
ADDITIONAL TREATMENT
Issues for Referral
Referral to a comprehensive ophthalmologist should be strongly considered for all suspected ophthalmic manifestations of IBD.
SURGERY/OTHER PROCEDURES
Intravitreal or sub-Tenons steroid injection may be considered by a retinal specialist for recalcitrant ocular inflammation.
IN-PATIENT CONSIDERATIONS
Patients with IBD associated ocular inflammation do not usually require hospital admission.
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
• Patients with ocular manifestations of IBD should be seen by an ophthalmologist until the inflammation is resolved.
• There is no evidence supporting screening patients with IBD for ocular manifestations.
Patient Monitoring
• Patients with uveitis and/or with long-term high dose oral corticosteroid therapy are at risk for developing cataracts (a reversible cause of visual loss) and glaucoma (a nonreversible cause of visual loss).
• There are no evidence-based recommendations regarding glaucoma screening in patients on long-term oral corticosteroids. The risk of developing glaucoma increases with treatment duration (especially >1 year), treatment dose, and pre-treatment morbidity (age, preexisting glaucoma or ocular hypertension and connective tissue disorders) (6)[C].
PATIENT EDUCATION
• Crohn’s and Colitis Foundation of America – Patient fact sheet on eye diseases
– http://www.ccfa.org/frameviewer/?url=/media/pdf/FactSheets/eyes.pdf
• A family history of IBD is an independent risk factor for the development of idiopathic uveitis, scleritis, and keratitis (7).
PROGNOSIS
• Good
– Most patients with ocular manifestations of IBD are treatment-responsive at some level and maintain their visual acuity.
COMPLICATIONS
• Glaucoma and cataracts secondary to long-term oral/topical steroids or prolonged intraocular inflammation
• Macular edema secondary to inflammation
• Phthisis bulbi in a chronically inflamed eye
REFERENCES
1. Bernstein CN, Blanchard JF, Rawsthorne P, et al. The prevalence of extraintestinal diseases in inflammatory bowel disease: A population-based study. Am J Gastroenterol 2001;96(4):1116–1122.
2. McCannel CA, Holland GN, Helm CJ, et al. Causes of uveitis in the general practice of ophthalmology. Am J Ophthalmol 1996;121:35–46.
3. Orchard TR, Chua CN, Ahmad T, et al. Uveitis and erythema nodosum in inflammatory bowel disease: Clinical features and the role of HLA genes. Gastroenterology 2002;123(3):714–718.
4. Ghanchi FD, Rembacken BJ. Inflammatory bowel disease and the eye. Surv Ophthalmol 2003;48:663–676.
5. Barrie B, Regueiro M. Biologic therapy in the management of extraintestinal manifestations of inflammatory bowel disease. Inflamm Bowel Dis 2007;13:1424–1429.
6. Chadhas V, Cruikshank I, Swingler R, et al. Advanced glaucomatous visual loss and oral corticosteroids. BMJ 2008;337:670.
7. Lin P, Tessler HH, Goldstein DA. Family history of inflammatory bowel disease in patients with idiopathic ocular inflammation. Am J Ophthalmol 2006;141(6):1097–1104.
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