Giant cell arteritis with stroke and systemic diffuse vasculitisC Lu1, S Zhou2, C Yang1, J Ye1
1 Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Songjiang District, Shanghai, China
2 Department of Hematology and Rheumatology, Shanghai Songjiang District Central Hospital, Songjiang District, Shanghai, China
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/jpgm.jpgm_800_21
Source of Support: None, Conflict of Interest: None
Keywords: Abdominal artery, femoral artery, giant cell arteritis, positron emission tomography, stroke
Giant cell arteritis (GCA) is a chronic, granulomatous, and large-vessel vasculitis with the incidence of 15--30/100.000 over the age of 50, and the classical manifestations include headache, fever, jaw claudication, and visual impairment. GCA affects mainly the aorta and its branches, predominantly the carotid artery and the subclavian and axillary branches, while the abdominal aorta, the iliac artery, and the limbs involvement are rare.,, Here, we present the first report of GCA with stroke and systemic diffuse vasculitis with the abdominal aorta, iliac artery, and femoral artery involved simultaneously.
A 71-year-old man presented to Department of Rheumatology with headache and fever for 2 weeks as well as weakness of the right limb for one day. Physical examination showed both sides of the superficial temporal arteries were swelling, tortuous and tender and the pulses were decreased [Figure 1]b. Besides, decreased pulses were also found on bilateral femoral arteries, and more pronounced on the right side. Neurologic examination revealed right-sided hemiplegia. Laboratory testing indicated an erythrocyte sedimentation rate of 126 mm per 1sth (normal value, <15), C-reactive protein level of 131.25 mg per liter (normal value, <8), a normal complete blood count and negative ANA.
Cerebral magnetic resonance imaging (MRI) showed infarction of the left frontal and parietal lobe [Figure 1]a. Doppler ultrasound disclosed significant thickening of the intima and the blood flow was decreased in bilateral superficial temporal arteries [Figure 1]c. Moreover, 18F-fluorodeoxyglucose-positron emission tomography (PET) showed increased tracer uptake in the ascending aorta, thoracic aorta, abdominal aorta, bilateral vertebral arteries, subclavian arteries, axillary arteries, common iliac artery, right internal iliac artery and bilateral femoral arteries, suggesting inflammation in the vessel walls [Figure 1]f. Furthermore, computed tomographic angiography (CTA) indicated thickening of the vessel walls of the thoracic and abdominal aorta, thrombosis at the bifurcation of the common iliac artery, occlusion of the right common iliac artery and severe stenosis of the left common iliac artery [Figure 1]d, [Figure 1]e. In addition, three-dimensional reconstruction of CTA of the thoracic and abdominal aorta demonstrated occlusion of the right common iliac artery and severe stenosis at the beginning of the left common iliac artery [Figure 1]g.
Therefore, he was diagnosed with GCA according to the American College of Rheumatology (ACR) criteria for the classification of GCA, age ≥50 years at disease onset, new onset of localized headache, ESR ≥50 mm/hour, tenderness or decreased pulse of the temporal artery. Oral prednisone was initiated at 50 mg daily. After treatment, he had no fever on the next day, and a week later the headache disappeared. Swelling of the bilateral temporal arteries and decreased pulses of the bilateral femoral arteries were also improved. He received low molecular weight heparin, cyclophosphamide at a dose of 800 mg/m2 and at the same time corticosteroid was tapered. The patient has been followed up for 6 months and his symptoms have improved significantly.
GCA is a systemic vasculitis with unclear etiology, which mainly affects older people. The peak incidence is between 70 and 80 years of age, with a predilection for women. Cerebrovascular accidents are relatively rare in GCA, affecting about 1.5‒7% of patients. Due to its diverse clinical manifestations, patients with GCA can present to various departments such as ophthalmology, neurology, and rheumatology. The present case had headache, fever, and hemiplegia, so he visited Department of Neurology. Temporal artery biopsy (TAB) is the gold standard for the diagnosis of GCA, but as the granulomatous lesions may be segmentally distributed, TAB may also give a false-negative result. Ultrasonography of the temporal artery and PET have been found to have high sensitivity and specificity for diagnosis of GCA., Although the present case did not undergo TAB, the clinical characteristics, laboratory and imaging features supported the diagnosis of GCA. GCA may also have large-vessel complications such as arterial stenosis, occlusion, aneurysm, or dissection., Literature, indicates that, GCA affects mainly the subclavian artery, carotid and axillary arteries, while abdominal and limb arteries involvement is rare. The reason for this may be that earlier most of GCA were diagnosed by TAB and vascular ultrasound (wherein thoracic and abdominal arteries could not be thoroughly evaluated). However, CTA can detect these lesions. Besides, PET can reflect the inflammation of vessels throughout the body and has a great advantage in evaluating systemic vascular inflammation.
In summary, GCA is a granulomatous vasculitis, which can cause systemic vascular involvement including abdominal artery and limb arteries, and PET has an advantage in evaluating systemic vascular inflammation.
Declaration of patient consent
The authors certify that appropriate patient consent was obtained.
The authors thank Ruifeng Zhu and Sufang Xiao for their assistance in data collation, and Libin Ma and Shiliu Ge for their assistance with reading the radiographic and Doppler ultrasound images.
Financial support and sponsorship
This work was supported by National Natural Science Foundation of China (81801592), Clinical Research Plan of SHDC (SHDC2020CR4011) and National Innovative research team of high-level local universities in Shanghai.
Conflicts of interest
There are no conflicts of interest.