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Baylor College of Medicine, Houston, TXHouston Methodist Hospital, Houston, TXUniversity of Texas Medical Branch (UTMB), Galveston, TXWeill Cornell Medicine, New York, NYThe UT MD Anderson Cancer Center, Houston, TX
Giant cell arteritis (GCA) is a systemic vasculitis that can cause permanent vision loss. Even after treatment, relapses have been reported in 40%–64% of patients, depending on how relapses are defined.
Here, we present a rare case of a GCA relapse mimicking idiopathic orbital inflammation (IOI) on the side contralateral to the initial episode of the disease. Simultaneous, positive biopsies of the orbit and temporal arteries were obtained, offering histological evidence that the orbital inflammation and GCA flare were linked.
IOI commonly presents with pain, periorbital edema, injection, diplopia, chemosis, proptosis, and decreased vision.
As an idiopathic condition, secondary etiologies should be considered before a diagnosis of IOI can be made.
GCA is a granulomatous vasculitis of medium and large vessels that can have vision and life-threatening consequences if not treated promptly. Although prior cases of orbital inflammation due to GCA have been reported, to our knowledge, this is the first case in the English-language ophthalmic literature of a GCA relapse presenting as orbital inflammation in the contralateral, previously unaffected eye.
A 74-year-old Caucasian female with a history of biopsy-proven GCA treated with prednisone presented with a 3-week history of painful and progressive vision loss, orbital erythema, epiphora, and periorbital edema in her right eye (OD), as well as pain over her right maxillary sinus.
She was originally diagnosed with GCA 2.5 years before the current presentation via a left temporal artery biopsy after presenting with left-sided scalp tenderness, jaw claudication, headache, weight loss, and left-sided vision loss from 20/20 to counting fingers at the face. At that time, she had a left-sided relative afferent pupillary defect (RAPD) and an edematous left optic disc (arteritic anterior ischemic optic neuropathy). She had been started on high-dose steroids and was tapered down to 9 mg of oral prednisone, with improvement in vision to 20/30.
In the current presentation, she complained of associated headaches and facial pain but denied scalp tenderness, jaw claudication, or fever. Her symptoms did not improve with topical antibiotics or steroids, and a short trial of increased systemic steroids (20 mg daily) was also ineffective.
On physical examination, her corrected visual acuity was 20/70 OD and 20/40 OS, down from 20/25 OU 3 months prior. Ishihara color plates were 4/14 OD and 9/14 OS, down from 14/14 and 12/14, respectively. There was no anisocoria or a relative afferent pupillary defect. Her intra-ocular pressure was normal OU. Slit-lamp examination showed chemosis and injection OD (Fig. 1) and new optic disc edema OD. No iritis or corneal edema was present. Humphrey (24-2 automated) visual field testing showed a new central scotoma OD.
C-reactive protein was elevated at 3.23 mg/dL, but her erythrocyte sedimentation rate was normal at 37 mm/hr. The patient was afebrile, and her hemoglobin, white count, platelets, and thyroid function levels were all normal. Magnetic resonance imaging of the brain and orbits revealed enhancement in the right orbit due to inflammation (Fig. 2) as well as mild mucosal thickening in the maxillary and sphenoid sinuses. Findings of cerebrospinal fluid analysis were within normal limits, with no signs of infectious etiologies.
Biopsies of the right orbit and right temporal artery were taken simultaneously. Chronic inflammation with fibrosis was found in the orbit (Fig. 3A, B). In the right temporal artery, there was multifocal segmental absence of the internal elastic lamina, with transmural CD68+ histiocytic infiltrate (Fig. 3C, D). Medial fibrosis and marked intimal thickening were also observed. Chronic inflammation in the artery consisted predominantly of T-cells. These findings were strongly consistent with active GCA.
The patient was diagnosed as having a GCA relapse mimicking IOI. She received 3 days of 1 g intravenous solumedrol, with dramatic improvement in her symptoms. She was discharged on 80 mg of prednisone. On follow-up 3 weeks later, her visual acuity was 20/50 OD and 20/30 OS without an RAPD. Visual fields and optic disc thickness returned to baseline with stable optic atrophy OU.
GCA has a lifetime risk of 1% for females and 0.5% for males.
Our patient had previously been diagnosed with GCA based on a left-sided clinical presentation, a positive temporal artery biopsy, and response to steroid therapy. Although uncommon, the level of visual recovery seen after her initial GCA episode has been previously reported.
Two and a half years later, she presented with clinical and radiographic findings suggestive of right-sided orbital inflammation. Biopsies of both the right temporal artery and right orbit revealed arterial inflammation diagnostic for GCA with concurrent orbital inflammation. Although it is possible that the orbital inflammation was an unrelated and concurrent idiopathic pathology, the more likely possibility is that it was caused by the patient's underlying GCA.
Although our patient was on 9 mg prednisone at the onset of her symptoms, one study showed that 22% of patients with their first GCA relapse were taking more than 7.5 mg prednisone.
The most common presentation of GCA relapse is polymyalgia rheumatica. Other common findings include cranial symptoms, such as headache and scalp tenderness, and systemic symptoms such as fever and weight loss.
Our case has 2 unique features. First, unlike the 2 prior cases, the eye with orbital inflammation was contralateral to the initial GCA episode. Second, the simultaneous, ipsilateral orbital and temporal artery biopsies provide strong evidence that relapsing GCA may cause inflammation of the orbit, leading to signs and symptoms of IOI. It is also important to highlight that, on relapse, this patient did not have the classic symptoms of GCA. She had no scalp tenderness, jaw claudication, polymyalgia rheumatica, or fever. Rather, IOI was the primary manifestation of the disease.
Early treatment of GCA is crucial for preventing irreversible vision loss.
By becoming familiar with the various presentations of the disease's relapses, clinicians can hasten its diagnosis and treatment, reducing morbidity. To our knowledge, this is the first case of a contralateral orbital relapse of GCA to be reported in the English-language ophthalmic literature.
Footnotes and Disclosure:
The patient consented to the publishing of this information and signed a form verifying her consent.
Relapses in patients with giant cell arteritis: prevalence, characteristics, and associated clinical findings in a longitudinally followed cohort of 106 patients.