Case description
A 51-year-old man came to our outpatient clinic for visual blurring of
the left eye. Ophtalmologic examination revealed signs of papilledema
with suspicion of focal retinal ischemia. Two months earlier, the
patient reports polymyalgia with functional impairment, asthenia, weight
loss of 3 kg and night sweats. Blood test showed increased C-reactive
protein (CRP) at 62 mg/l. Viral and bacterial infectious disease were
excluded, notably a SARS-CoV-2 infection. A methylprednisolone (mPDN)
trial – without a definite diagnosis - with an initial dose of 32
mg/day during 5 days was tried with clinical improvement. Visual
blurring occured about 15 days after the discontinuation of
corticosteroids therapy and lasted for several days in a steady state.
The rest of the physical examination was unremarkable. His medical
history includes hypothyroidism, arterial hypertension and a chronic
renal insufficiency stage 2 of unknown origin. The patient did not
describe headache, jaw claudication, scalp tenderness or temporal artery
tenderness. Control blood test in hospitalization showed high
erythrocyte sedimentation rate (ESR) and CRP (73 mm/h and 54 mg/l
respectively). Screening for auto-immune disease showed low positivity
of lupus anticoagulant with negative antinuclear factors (FAN) and
anti-neutrophil cytoplasmic antibodies (ANCA). Screening for
tuberculosis, syphilis, HIV, borrelia, bartonella and toxoplasmosis was
negative. Cerebral magnetic resonance imaging (MRI), lumbar puncture
(with meningeal panel excluding cytomegalovirus, herpes simplex virus 1
and 2 and herpes zoster virus) and visual evoked potential were normal.
Due to the suspicion of GCA, treatment by mPDN 64 mg/day was started
with improvement of the inflammatory syndrome and disappearance of
papilledema at ophtalmological control. PET-CT scan and ultrasonography
of the temporal arteries – performed before starting mPDN - were
normal. Microscopic examination of temporal artery biopsy revealed a
discrete inflammatory infiltration of the adventitia with preservation
of media and intima layers. No disruption of the internal or external
elastic lamina, nor giant cell formation were observed in serial
sections. According to the histologic patterns’ classification proposed
by Hernandez-Rodriguez et al (5), the case was diagnosed as giant-cell
arteritis adventitial pattern (Figures 1 & 2 ).