Case Presentation:
We present our case, which is a 37-year-old male middle eastern with a
past medical history of Haemophilia A. He presented to our outpatient
ophthalmology clinic with a right eye sudden decrease in vision for four
days. On evaluation of the eye, he had features of unilateral
pan-uveitis with para-macular retinitis, mild vitritis, peripheral
punctuate retinal hemorrhage, and macular exudative retinal detachment,
findings highly suggestive of Behçet’s disease. He was started on a
tapering dose of oral prednisolone and referred urgently to the
Rheumatology clinic. Upon enquiring, he mentioned a history of recurrent
painful oral and genital ulcers for several years for which he did not
seek medical attention before. On review of systems, he had no history
of joint pain or swelling, no history of skin rash, respiratory or
gastrointestinal symptoms, and no history of deep vein thrombosis.
On examination, no oral ulcers or scars were present, but he had one
papule on the scrotum. Review of other systems were unremarkable. Blood
tests (table.1) at this point were unremarkable, including autoimmune
screening apart from slightly elevated C-reactive protein (CRP, 17
mg/dl). HLA typing for HLA-B51 was negative. Pathergy test was negative.
A clinical diagnosis of Behçet’s disease was made based on history of
recurrent painful oral ulcers, genital ulcers and panuveitis. Hence,
Immunosuppressive therapy was started with prednisolone, azathioprine
and infliximab.
After a few weeks of treatment, his vision improved with a resolution of
signs of uveitis. He was maintained on azathioprine and infliximab, with
the latter stopped after 15 months.
The patient was referred after seven months to an audiology clinic for
evaluation of tinnitus and hearing loss. Symptoms of hearing loss had
started in left ear almost at the time of panuveitis but it stabilized
after starting immunosuppression and later involved the right ear, few
months after stopping Infliximab. Physical examination of the ears were
normal, with intact tympanic membrane bilaterally.
A pure tone audiogram showed
bilateral sensorineural hearing loss more on high frequencies (Figure
1). Magnetic resonance imaging (MRI) of the head and internal auditory
meatus was done and were normal (Figure 2). All vestibular tests
including video-nystagmography, oculomotors and post headshake test,
computerized dynamic posturography, caloric testing, vestibular evoked
myogenic potential and video head impulse test were unremarkable. Based
on the previous diagnosis of Behçet’s disease, the sensorineural hearing
loss was attributed to cochlear involvement by autoimmune inner ear
disease related to Behçet’s disease. The patient was kept under close
follow-up by Rheumatology, Ophthalmology, and Audiology. He was started
on oral steroid prednisolone 70 mg per day for one month in addition to
4 doses of intratympanic steroid injections, which partially relieved
symptoms. However, he continued to have repeated hearing loss episodes
whenever steroid doses were tapered. Infliximab was resumed as a
steroid-sparing agent with a dose of 5mg/kg every six weeks, along with
azathioprine 100 mg per day and it helped to successfully taper and stop
steroid. The patient was followed for the next two years, and his
symptoms were controlled with stable audiogram levels.