An exacerbation of Behçet's syndrome presenting with bilateral papillitis

M. D. Tarzi, S. Lightman1 and H. J. Longhurst

Department of Immunopathology, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE and 1 Moorfields Eye Hospital, 162 City Road, London EC1 2PD, UK

Correspondence to: M. D. Tarzi. E-mail: mike.tarzi{at}bartsandthelondon.nhs.uk

SIR, A 32-yr-old white British man presented with acutely blurred vision. He was under follow-up for Behçet's syndrome, diagnosed 4 yr previously on the basis of orogenital ulceration, erythema nodosum, pustular skin lesions and recurrent testicular swelling, and had entered prolonged remission following treatment with methotrexate, tacrolimus and dapsone. At presentation he was taking tacrolimus 3 mg and dapsone 50 mg daily. The visual disturbance developed over a period of hours and was not associated with orbital pain or conjunctival injection. He described general malaise and a mild headache, but there was no history of fever, vomiting or neurological deficits. He was afebrile and fully orientated, with no neck stiffness. Several oral ulcers were present, as well as pustular lesions on the face and arms. Visual acuity was 6/6 in the right eye and 6/9 in the left eye. A central scotoma was present in the left eye on confrontation testing, and ophthalmoscopy revealed bilateral swelling of the optic nerve heads, more marked on the left (Fig. 1). The remainder of the examination was normal.



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FIG. 2. This figure may be viewed in colour as supplementary data at Rheumatology Online.

 
He was admitted for urgent investigation. The erythrocyte sedimentation rate and serum levels of C-reactive protein were within normal limits. Magnetic resonance imaging of the brain was unremarkable, with no space-occupying lesions or meningeal enhancement, no demyelination, normal appearances of the optic nerve and no evidence of venous thrombosis on magnetic resonance venography. A lumbar puncture demonstrated an opening pressure of 14 cm cerebrospinal fluid (CSF). The CSF sample was acellular, with glucose and protein concentration within the normal range, and later proved negative by polymerase chain reaction for herpes simplex virus, cytomegalovirus and varicella-zoster virus.

We diagnosed bilateral optic neuritis secondary to Behçet's syndrome, and commenced treatment with intravenous methylprednisolone at a dose of 1 g daily. Clinical response was noted within 72 h, with visual acuity returning to 6/6 in the left eye and partial resolution of papillitis on ophthalmoscopy. Tacrolimus was discontinued, and he was discharged on mycophenolate 500 mg twice daily and a reducing dose of prednisolone. At follow-up 6 months later, the visual acuity was 6/6 in both eyes, with full visual fields and normal ophthalmoscopic appearances, but ongoing orogenital ulceration and pustular skin lesions have so far responded incompletely to mycophenolate dose escalation and the addition of dapsone, and he remains on a maintenance dose of prednisolone.

Isolated optic neuritis is an exceedingly rare presentation of Behçet's syndrome. By contrast, inflammatory eye disease affects up to 70% of patients, with young males at particular risk [1]. The most frequent ocular manifestation is bilateral pan-uveitis, with or without retinal vasculitis. Once established, eye disease tends to become chronic and relapsing, permanent visual loss historically affecting 25% of such patients. The prognosis has been improved by the use of cytotoxic agents such as azathioprine, cyclosporin and tacrolimus, with azathioprine in particular confirmed to reduce ocular relapse and preserve visual acuity [2]. In addition, biological treatments may be effective for inflammatory eye disease: drugs directed against anti-tumour necrosis factor {alpha} are increasingly used [3] and there are encouraging results from an open study of interferon {alpha}2a in refractory ocular Behçet's syndrome [4].

Neuropapillitis without inflammatory eye disease, which should be considered as part of the spectrum of neuro-Behçet's, appears to be rare. Gallinaro et al. reported an acute presentation with neuropapillitis in a 34-yr-old woman with Behçet's syndrome [5], who later developed a sixth nerve palsy. More recently, Mitra and Koul published a report of a 10-yr-old girl with acute uniocular visual loss associated with papilloedema [6]. The authors describe several separate presentations over the course of 3 yr with manifestations of neuro-Behçet's, including various cranial nerve lesions, hemiparesis and sensory disturbances, associated with active cerebrospinal fluid and magnetic resonance imaging abnormalities. A brisk response to systemic corticosteroids was noted, in keeping with the experience of our patient.

The important differential diagnoses of bilateral papilloedema in the context of Behçet's syndrome are cerebral venous thrombosis and central nervous system infection. The latter, of particular concern in the immunosuppressed patient, may be excluded by imaging and lumbar puncture. Cerebral venous sinus thrombosis is well described in Behçet's syndrome [7, 8] and is usually characterized by severe headache; the diagnosis can be made by magnetic resonance angiogram.

The authors have declared no conflicts of interest.

References

  1. Verity DH, Wallace GR, Vaughan RW, Stanford MR. Behçet's disease: from Hippocrates to the third millennium. Br J Ophthalmol 2003;87:1175–83.[Abstract/Free Full Text]
  2. Yazici H, Pazlarli H, Barnes CG et al. A controlled trial of azathioprine in Behçet's syndrome. N Engl J Med 1990;322:281–5.[Abstract]
  3. Sfikakis PP. Behçet's disease: a new target for anti-tumour necrosis factor treatment. Ann Rheum Dis 2002;61(s2):51–3.
  4. Kötter I, Eckstein AK, Stübiger N, Zierhut M. Treatment of ocular symptoms of Behçet's disease with interferon alpha 2a: a pilot study. Br J Ophthalmol 1998;82:488–94.[Abstract/Free Full Text]
  5. Gallinaro C, Robinet-Combes A, Sale Y, Richard P, Saraux A, Collin J. Neuropapillitis in Behçet's disease: a case. J Fr Ophthalmol 1995;18:147–50.[ISI][Medline]
  6. Mitra S, Koul RL. Paediatric neuro-Behçet's disease presenting with optic nerve head swelling. Br J Opthalmol 1999;83:1096–7.[Free Full Text]
  7. Celebisoy N, Secil Y, Akyurekli O. Pseudotumor cerebri: etiological factors, presenting features and prognosis in the western part of Turkey. Acta Neurol Scand 2002;106:367–70.[CrossRef][ISI][Medline]
  8. Fujikado T, Imagawa K. Dural sinus thrombosis in Behçet's disease—a case report. Jpn J Ophthalmol 1994;38:411–6.[ISI][Medline]
Accepted 8 March 2005





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