An eight-year-old girl was admitted in the pediatric neurology ward of Imam Hossein Children's Hospital, Isfahan University of Medical Sciences, due to a sudden visual loss in both eyes and headache in the occipital area. The patient had no fever, and she did not have any symptoms of meningitis, including neck stiffness and Brudzinski and Kernig signs, in physical examinations. Cranial nerve examination was normal, visual acuity of the right eye was hand motion of 50 cm, and the left eye was 100 cm. The patient had visual impairment in recognizing green and red colors. On ophthalmic examination, anterior and posterior chambers and funduscopy of both eyes were completely normal. The patient had a history of recurrent oral aphthous ulcers (5-6 times a year), genital ulcers, chronic and vague abdominal pain, diarrhea, and intermittent constipation. She had been treated with intravenous immunoglobulin due to Kawasaki disease when she was three years old.
The results of laboratory tests including complete blood count, erythrocyte sedimentation rate, C-reactive protein, serum electrolytes, infectious diseases' serology for toxoplasmosis, cytomegalovirus, tuberculosis, and rheumatologic tests including antinuclear antibody, angiotensin-converting enzyme, antiphospholipid antibodies, and serum complement levels were normal. The result of Mantoux test was negative. Chest X-ray and brain magnetic resonance imaging (MRI) without contrast results were normal either. The result of cerebrospinal fluid analysis including cell, sugar, protein, smear, culture, oligoclonal bands, neuromyelitis optica (NMO) antibody, and myelin oligodendrocyte glycoprotein (MOG) was normal. Fecal calprotectin level was 252 mug/mg (NL < 50). GI endoscopy and colonoscopy results were normal. The patient was transferred to the pediatric rheumatology ward. The result of the pathergy skin test, on the forearm area performed with intradermal needle 21 gauge, was positive. Both human leukocyte antigen (HLA) B5 and HLA-B51 tests were positive.
According to the criteria of the International Study Group for Behcet Disease, the patient was recognized as a case of BD-related bilateral retrobulbar optic neuritis, and the treatment started by prescribing pulse methylprednisolone 30 mg/kg/d (max 1 gr) for three days. Then, in the fourth day of treatment, intravenous infusion with infliximab 8 mg/kg was given. The patient was discharged on the fifth day of hospitalization with oral prednisolone 2 mg/kg/d, azathioprine 2 mg/kg/d, and colchicine 1 mg/d. After a one-week follow-up, the patient had no color vision impairment, and both eyes could see two meters away. Ocular exam through slit lamp and funduscopy was normal. She did not have headache and nausea. After a one-month follow-up, the patient had no color vision impairment, and ocular exam through slit lamp and funduscopy was also normal. Visual acuity of both was around 20/40. Infusion of infliximab 8 mg/kg was given, and oral prednisolone 1 mg/kg/d with azathioprine and colchicine with similar doses was continued. Three months after discharge, the patient did not have any complain about aphthous ulcers and GI system problems. Visual acuity of both eyes was 20/20, and ocular exam was normal. Azathioprine, colchicine, and infliximab infusion continued monthly. Oral prednisolone was tapered to 0.2 mg/kg/d.