Correspondence and reprintrequests to:Dr. M.B. Gorroño-EchebarríaDept. OftalmologíaHospital Universitario ‘Príncipe deAsturias’Carretera Alcalá-Meco s/n. Alcalá deHenares28805 MadridSpainTel: 34-918878100, ext.2189Fax: 34-918801825E-mail: mbgech@netscape.net

Abstract Several ocular manifestations have been found in Crohn’sdisease patients, most often affecting the anterior segment. This paperpresents the case of a young woman with pars plana exudates in whomCrohn’s disease was later diagnosed. To the authors’ knowledge, this isonly the second report of Crohn’s disease and concomitant pars planaexudates.

Key words Inflammatory bowel disease; Crohn’s disease; anterioruveitis; pars plana exudates

Introduction Uveitides comprise a heterogeneous group of inflam-matory ocular diseases with varying etiologies and prognoses, whichmay be classified according to the anatomic location of the inflamma-tion (anterior, intermediate, posterior, or panuveitis) or by the evolu-tion of symptoms (acute or chronic). Uveitis can occur as the soleclinical manifestation of a disease, during the evolution of an extra-ocular disease, or as the initial sign of a systemic disease.

Intermediate uveitis is characterized by vitreal inflammation withassociated inflammation of the vitreous base and peripheral anteriorretina and choroid. It may be found as an isolated and idiopathic condition or in association with systemic disorders such as multiplesclerosis and sarcoidosis. A subset of these intermediate uveitides iscalled pars planitis when a white opacity occurs over the pars plana and ora serrata.

Inflammatory bowel disease (IBD) includes two disorders, Crohn’sdisease (CD) and ulcerative colitis (UC). Both conditions can affectthe colon and rectum and display similar extraintestinal manifestations

Crohn’s disease associated with pars plana exudates 65

Ocular Immunology and Inflammation0927-3948/02/US$ 16.00

Ocular Immunology and Inflammation –2002, Vol. 10, No. 1, pp. 65–68© Swets & Zeitlinger 2002

Accepted 3 April 2002

Inflammatory bowel disease (Crohn’sdisease) in a Spanish patient with pars

plana exudates: Report of a new case andreview of the literature

Marina B. Gorroño-Echebarría1

Fernando Albarrán2

Ama Marcos1

Melchor Alvarez-Mon2

Departments of 1Ophthalmology and 2Internal Medicine, Hospital Universitario ‘Príncipe de Asturias’, Alcalá de Henares,

Madrid, Spain

Case report

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involving the liver, skin, joints, and eye. The immune system is thoughtto play an important role in the pathogenesis of these disorders.Extraintestinal immune-related inflammatory manifestations havebeen reported in 25–36% of CD patients, most commonly peripheralarthritis and erythema nodosum.1

The eye is affected in 4–10% of patients with IBD, with a wide spectrum of ocular manifestations reported.2,3 Acute episcleritis, acuteanterior uveitis, and marginal keratitis have been most frequentlydescribed. Posterior segment manifestations are less common (<1%)and include central serous choroidopathy, panuveitis, choroiditis,ischemic optic neuropathy, retinal vasculitis, neuroretinitis, and periph-eral uveitis. Two patients with concomitant intermediate uveitis andIBD,3,4 one of whom with pars plana exudates, have been described.The ocular manifestations of CD can appear either before or after theintestinal manifestations of the disease.

We report here an unusual case of a Spanish woman with pars planaexudates, who was later diagnosed with CD. To our knowledge, thisassociation has been reported only once previously.4

Case report A 27-year-old woman with a four-year history of recurrent bilateral anterior uveitis had been treated previously withtopical steroids and mydriatics. She had recently been diagnosed withpars planitis and was referred to our department for evaluation.Upon presentation, her visual acuity was 20/25 in each eye. Slit-lampexamination revealed 1+ anterior chamber cells and rare small whitenongranulomatous keratic precipitates in each eye, with posteriorsynechiae for almost 360° in the right eye. Intraocular pressure was 10mmHg in each eye, and remained in the normal range in subsequentexaminations. Fundus examination of each eye revealed moderate vitreous cells, many snowballs in the inferior vitreous, and a snowbankin the inferior pars plana, characteristic of pars planitis.

The patient related several outbreaks in the previous three years oflow back pain and mild dull mesogastric pain without diarrhea or signsof gastrointestinal bleeding. The patient’s physical examination wasunremarkable except for right lower quadrant abdominal tendernessto palpation without rebound or palpable mass. There was no familyhistory of autoimmune diseases. Laboratory evaluation revealed anormal differential white cell count, with normal platelets. Mild macro-cytic anemia was present with a hemoglobin level of 12.3g/dl (normalvalues 12–16g/dl). General biochemistry and urine analysis werenormal except for albumin (3.2g/dl, normal values 3.6–5.3g/dl), a1globulin (0.58g/dl, normal values 0.15–0.35g/dl), and a2 globulin (1.1g/dl, normal values 0.4–0.9g/dl). Immunoglobulins were normal,and antinuclear antibodies, rheumatoid factor, and HLA-B27 werenegative. Mantoux and serologic tests for syphilis, CMV, EBV, and tox-oplasma were negative. Serum folic acid and vitamin B12 were normal,although in the low normal range. Ferritin was 4ng/ml (normal values15–90ng/ml). A Schilling test revealed a mild vitamin B12 malabsorp-tion, and parietal cell antibodies were negative.The ophthalmic disease,laboratory findings, and abdominal pain were consistent with an under-lying IBD. Further radiologic and endoscopic exploration revealed a

66 M.B. Gorroño-Echebarría et al.

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terminal ileum stricture. Barium studies showed a 20-cm stenotic areaat the terminal ileum and another stenotic area affecting the cecum andascending colon. Colonoscopy revealed a stenotic ascending colon withadjacent ulcers. Biopsy of the ulcers revealed nonspecific mixed inflam-matory changes. A bone scan showed bilateral mild sacroileitis.

The uveitis was treated with 60mg oral prednisone on alternate daysand 125mg cyclosporine A every 12 hours (5mg/kg/d), as well as topicalsteroids and mydriatics.

Three years after initial presentation, the patient was asymptomatic.She is currently being maintained on 10mg oral prednisone on alter-nate days to treat the ocular disease and salazopyrine (20mg every 12hours) to treat the intestinal disease. Her present visual acuity is 20/20in each eye without correction, with mild vitreous inflammation andpars plana exudates.

Discussion We report the case of a 27-year-old woman, who pre-sented with pars plana exudates prior to developing intestinal mani-festations of CD. The association of ocular inflammation with IBD has been well documented. Ocular complications occur in 4–10% ofpatients with IBD. Posterior segment manifestations are less common(<1%), possibly due to the frequent use of corticosteroids in treatingthe underlying intestinal disease and the rapid resolution of posteriorsegment manifestations with this therapy. Patients with pars plana exu-dates show a mild anterior uveitis with a pupil that dilates adequately,although posterior synechiae may be present. The synechiae in ourpatient (covering almost 360° in the right eye) may have been causedby inadequate therapy due to a delayed diagnosis. Alternatively, thefirst episode of the disease (before the patient was referred to us) mighthave been associated with a more severe iridocyclitis.The risk of devel-oping ocular inflammation in CD may be related to the site of bowelinvolvement (colitis or ileocolitis) and to the presence of arthritis.5 Ourpatient manifested both colitis and mild sacroileitis.

IBD and uveitis are both characterized by a dysregulation of the immune system, and a common immunologic disturbance mayunderlie both conditions. Possible autoimmune mechanisms includegenetic susceptibility, cytokine imbalance, antigenic display of anautoantigen, aberrant self-recognition, and molecular mimicry (i.e.,organ-specific cellular antigen(s) shared by the colon and extracolonicorgans).

Microbes may play an important role in molecular mimicry. Anti-colon antibodies that bind epithelial cells have been demonstrated in56% of patients with UC and 67% of patients with CD.6 These anti-bodies have been found to cross-react with Escherichia coli 014, sug-gesting that anticolon antibodies initially induced against E. coli 014antigens might cross-react and contribute to the cell damage observedin IBD. Interestingly, it has been shown that S-antigen, a soluble retinalprotein which can induce experimental autoimmune uveitis (EAU) insusceptible animals, shares an amino acid motif with peptides derivedfrom E. coli.7

The possibility that an immune response initiated against a bacterialantigen could, by means of molecular mimicry, cause IBD and uveitis

Crohn’s disease associated with pars plana exudates 67

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References1 Greenstein AJ, Janowitz HD, Sachar

DB. The extra-intestinalcomplications of Crohn’s diseaseand ulcerative colitis: A study of 700patients. Medicine (Baltimore). 1976;55:401–412.

2 Hopkins DJ, Horan E, Burton IL,Clamp SE, De Dombal FT, GoligherJC. Ocular disorders in a series of332 patients with Crohn’s disease. BrJ Ophthalmol. 1974;58:732–737.

3 Boskovich SA, Lowder CY, MeislerDM, Gutman FA. Systemic diseasesassociated with intermediate uveitis.Cleveland Clin J Med. 1993;60:460–465.

4 Lyons JL, Rosenbaum JT. Uveitisassociated with inflammatory bowel disease compared with uveitis associated with

spondyloarthropathy. ArchOphthalmol. 1997;115:61–64.

5 Salmon JF, Wright JP, Murray AD.Ocular inflammation in Crohn’sdisease. Ophthalmology. 1991;98:480–484.

6 Lagercrantz R, Hammarstrom S,Perlmann P, Gustafsson BE.Immunological studies in ulcerativecolitis. 3. Incidence of antibodies tocolon-antigen in ulcerative colitisand other gastro-intestinal diseases.Clin Exp Immunol. 1966;1:263–276.

7 Singh VK, Yamaki K, Abe T,Shinohara T. Molecular mimicrybetween uveitopathogenic site ofretinal S-antigen and Escherichiacoli protein: Induction ofexperimental autoimmune uveitisand lymphocyte cross-reaction. CellImmunol. 1989;122:262–273.

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in susceptible individuals is intriguing. A better understanding of thepathogenesis of the ocular manifestations of IBD is needed.

In summary, our findings suggest that a patient with pars plana exu-dates may have an associated IBD and attention should be focused onsuch a link.

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