54. Binocular sight loss: uncommon, yet a devastating complication of GCA

Abstract

Introduction: GCA (giant cell arteritis) affects cranial branches of the arteries from aortic arch, especially the superficial temporal artery and vessels supplying the eye. Ophthalmic manifestations of GCA are characterised by the vasculitis of the ophthalmic artery and its branches. We present a case of a patient who developed the binocular sequential permanent vision loss secondary to GCA due to the delayed diagnosis. Case description: A seventy-six year-old lady withabackgroundof type two diabetes mellitus, hypertension and dyslipidemia and hemicolectomy for appendix tumour two years ago (no recurrence) was admitted with blurring of vision in the eye. She did not complain about other symptoms, however, on direct questioning she admitted six weeks history of weight loss (2.5 stones), severe tiredness and occasional night sweats, mild left sided temporal headache. She was initiated on 60mg of prednisolone. One week prior to admission she had right eyeCRAOdiagnosed by the ophthalmology team with preceding three weeks history of right eye blurring of vision. CRPat that time was 12 g/L andGCAwas not considered likely. Following her permanent right sided vision loss, she was referred to stroke team for further management and was given aspirin. Her CT and MRI brain scans were unremarkable, USS carotid arteries revealing diffuse intimalthickeningin allthearteriesoftheneck. Onassessmentin therheumatologyfast track clinicshedeniedanyassociated jaw/tongue/arms claudication and no proceeding or historical PMRsymptoms. She had mild left sided temporal artery tenderness and decreased temporal artery pulses bilaterally, no vision in the right eye along with the blurring in the left eye. There were noaudible bruits in carotid, axillary, femoral arteries. Her acute phase maker (CRP) was mild but persistently elevated with a maximum level of 12 g/L for one week prior the admission.USSshowedfeatures consistent withGCAwith extensive non-compressible halo signs in temporal arteries and its branches bilaterally. Anurgent ophthalmology review revealedAIONchanges in the left eye, her vision deteriorated over the next forty-eight hours despite the initiation of high dose intravenous methylprednisolone. The patient was alsostartedontocilizumabinfusioninanattempttoreversethesightloss. Discussion: The ophthalmic manifestations of GCA range from AION (anterior ischaemic optic neuropathy), CRAO (anterior central retinal artery occlusion), cilioretinal artery occlusion to occipital lobe infarcts, transientmonocular vision loss,photopsiasor diplopia.OphthalmicGCA is an emergency and requires urgent ophthalmological evaluations and initiation of treatment with high dose of steroids to avoid permanent loss of vision. The reported incidence of visual symptoms in GCA ranges widely from12%to70%of cases.GCAis often associated with constitutional symptoms such as weight loss, night sweats, fevers, systemic inflammatory response with elevated inflammatory markers. However, it hadbeenreportedthatthiscanbeverymildinischaemicGCA. The diagnosis of GCA in this particular case was delayed for approximately six weeks due to the atypical initial presentation with mainly constitutional symptoms and unilateral vision blurring as well as mildly elevated inflammatory markers. A collaboration of three teams (namely ophthalmology, stroke and rheumatology) proved necessary to initiate high dose steroid treatment and tocilizumab. However, patient still suffered from permanent binocular vision loss.Shewasregistered blindand will infuturerequireextensivephysicalandpsychologicalsupport. Keylearningpoints: Despite the recentadvancesandincreasedawareness with good availability of fast track GCA services, cases of bilateral blindness are still observed. This is due to a lack of awareness of atypical non-cranialsymptomsthat frequentlyaccompanyaGCAischaemic presentation. Inflammatory markers are often only mildly elevated in such cases.Wesuggestaheightenedpublicandprofessional awareness programtomitigatethis irreversibledisastrouscomplication. Conflicts of interest: The authors have declared no conflicts of interest.