Horner’s syndrome and fever: An unusual presentation of the giant cell arteritis

Giant Cell Arteritis (GCA), or temporal arteritis, is a systemic infl ammatory vasculitis of unknown aetiology that occurs in older persons and can result in a wide variety of systemic, neurologic, and ophthalmologic complications [1]. A symmetrical polyneuropathy and mononeuritis both occur as manifestations of GCA [2-3], while the Horner’s syndrome resulting from GCA is very rare. The case emphasises the need for physicians to promptly recognise and treat GCA to avoid long-term complications. In addition, we urge that GCA is considered as a diagnosis even when faced with uncommon presenting features


Background
Giant Cell Arteritis (GCA), or temporal arteritis, is a systemic infl ammatory vasculitis of unknown aetiology that occurs in older persons and can result in a wide variety of systemic, neurologic, and ophthalmologic complications [1]. A symmetrical polyneuropathy and mononeuritis both occur as manifestations of GCA [2][3], while the Horner's syndrome resulting from GCA is very rare. The case emphasises the need for physicians to promptly recognise and treat GCA to avoid long-term complications. In addition, we urge that GCA is considered as a diagnosis even when faced with uncommon presenting features

Case presentation
An 81-year-old, independent, Caucasian female, with no signifi cant medical history, was admitted with a 4-week history of daily fevers of up to 38°C which had been unsuccessfully treated with antibiotics (amoxicillin/clavulanate and clarithromycin) and in last days presented a drooping of the right upper eyelid with a referred eye discomfort, fl eeting diplopia and lost weight. Moreover, she referred to a headache note quickly responding to paracetamol intake, but that in the last week was more localized to the right. At clinical visit she had palpable temporal arteries but no visual or speech disturbance, limb weakness, neck stiffness or rash; nor did she have any scalp tenderness or jaw claudication. Physical examination was unremarkable. Neurological examination was unremarkable with normal motor and sensory systems and intact cranial nerve function with the exception of a miosis, apparent enophthalmos and ptosis of the right eyelid ( Figure  1a). Initial blood tests, blood and urine culture, chest x-ray, CTA of the chest, neck and brain, were normal apart from a raised erythrocyte sedimentation rate (ESR) of 66mm/h and C reactive protein (CRP) of 12g/dl with a normal levels of procalcitonin. The rheumatoid factor was very high (207Ui/mL) in the absence of anti-citrullinated protein antibodies. Ophthalmology and ear, nose and throat reviews did not reveal any signifi cant abnormalities or a cause for her symptoms. Visual acuity was 10/10 bilaterally. Neuro-ophthalmic examination confi rmed a miosis and partial rightsized ptosis, while the optic nerves remained fully intact.

Investigations
Gadolinium-enhanced MRI of the spine and brain were normal. However, ESR and CRP were risen to 91mm/h and 8.8mg/dl, respectively. The diagnosis of Temporal Arteritis (TA) was considered according to American College of Rheumatology criteria [4], we performed a temporal artery biopsy and started on a 3-day course of intravenous methylprednisolone (1g/day).
After 3 days of intravenous steroid treatment, the cenesthesia was markedly improved, the fever had disappeared and the ESR and CRP were dropped to 51mm/h and 1.5mg/dl. However, the miosis and partial right-sided ptosis persisted.
Histology of temporal artery biopsy returned a fl orid positive result for GCA (Figure 1b,c)

Summary
The authors present a case of a histologically confi rmed giant cell arteritis that presented unusually with fever and Horner's syndrome. The aetiology of the presenting features and the treatment choices are discussed.

Treatment
The initial high-dose corticosteroid therapy and steroid tapering regimen was established in accordance with the British Society of Rheumatology guidelines [5].

Outcome and Follow-up
After about 12 days of treatment the miosis had disappeared and ptosis of the right eyelid was improved but not resolved to the Ophthalmology and Neurology reviews. Currently, the patient is maintained on a reducing dose of oral steroid and has follow-up with rheumatology. The absence of brainstem and spinal cord signs and a normal brain MRI argues against a preganglionic location.

Discussion
Ophthalmoplegia in GCA is commonly attributed to neuronal damage. There has been debate among authors whether ocular muscle ischaemia or nerve involvement is the primary pathological cause of the ophthalmoparesis [7][8][9]. The complete recovery of miosis and ptosis, as seen in our patient, would suggest that permanent neuronal damage has not occurred and this uncommon clinical presentation of GCA could be attributed to ischaemia of the postganglionic nerves due to an arteritis of the vasa nervorum [10], but we did not perform imaging to prove such a theory that continues to be an interesting area for further research and discussion in neurologic complication of GCA.
It is likely that in patients with high levels of rheumatoid factor, the involvement of the vasa nervorum is earlier and more intense in the autonomic nervous system, as documented in patients with rheumatoid arthritis [11,12]. However, in our patient it is not possible at the moment to speculate about the involvement only of the preganglionic fi bers of the right side only. This is to our knowledge, the fi rst reported clinical case with a GCA who presented with persistent fever and an acute postganglionic isolated Horner's syndrome.
We feel that the association of GCA and Horner's syndrome, taken together with that other rare reported clinical cases [13][14][15], indicates an unusual manifestation of GCA, which has possibly been overlooked in the past.

Learning points/take home messages
 Giant cell arteritis (GCA) may very rarely present with a persistent fever and Horner's syndrome.
 It is important to consider the diagnosis of GCA even with an unusual presentation.