Background: Giant Cell Arteritis (GCA), is the most common primary vasculitis. It
affects large vessels such as the aorta and its branches. According to Chapel Hill Consensus, GCA
is one of the larger vessel vasculitis. The underlying mechanism involves inflammation of the large
arteries. The most frequent presentation consists of headache, polymyalgia, and jaw claudication.
GCA can put the visual prognosis at risk, and rapid diagnosis is compulsory. Cotton wool spots,
due to focal inner retinal ischemia, are an early diagnostic ophthalmological sign. The most frequent
presentation is a rapid, partial or complete blindness. However, atypical presentations, such
as uveitis, especially in the anterior chamber, can delay diagnosis.
Case Report: We report a 75-year-old woman with GCA who initially presented with anterior uveitis
and without any other clinical sign. At the beginning, there was the only ophthalmic sign and
systemic inflammation, the all exhaustive work-up including positron emission tomography (PET)
scan was negative. The biology was fully normal without auto-immune profile (Angiotensin converting
enzyme level, Interferon Gamma Release Assay, Syphilis serology, antinuclear antibody
titer, Rheumatoid factor, CCP antibodies, and chest x-ray were normal. HLA B27 was negative). In
the following weeks, she subsequently developed large vessel vasculitis with headache and more
typical sign. She developed cotton wool spots linked to retinal arteriolar hypoperfusion. Anterior
uveitis has been reported rarely in GCA and moreover, it is very uncommon at the early stages of
GCA. Our case stresses that uveitis onset can precede large vessels vasculitis and typical symptoms
of GCA. PET-scan is a useful tool for atypical GCA, but its sensitivity is not perfect, and its repetition
can be helpful in selected cases such as that of this patient.