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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 60  |  Issue : 1  |  Page : 78-82

Various etiologies and presentations of giant cell arteritis: A series of case reports


1 Department of Ophthalmology, Adesh Medical College and Hospital, Kurukshetra, Haryana, India
2 Department of Medicine, Adesh Medical College and Hospital, Kurukshetra, Haryana, India
3 Department of Neurology, Adesh Medical College and Hospital, Kurukshetra, Haryana, India

Date of Submission28-Jul-2021
Date of Decision10-Nov-2021
Date of Acceptance06-Dec-2021
Date of Web Publication22-Mar-2022

Correspondence Address:
Dr. Sujit Das
Department of Ophthalmology, Adesh Medical College and Hospital, NH-1, Mohri, Kurukshetra - 136 135, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_116_21

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  Abstract 


GCA is a granulomatous vasculitis of unknown origin and targets large vessels with predominance for the aortic arch and the cranial branches. The underlying etiology of GCA is complex and has been widely researched, yet is still not well understood. The average age of presentation is 72.5 years for women and 70.3 years old for men. Furthermore, women are affected between 2 and 6 times more often than men. With vision loss, ophthalmologists are on the front lines of diagnosing the disorder. In addition to vision loss, patients commonly note associated symptoms such as headache, jaw claudication, diplopia, myalgias, and constitutional symptoms. The most commonly feared sequel of GCA is permanent visual loss secondary to arteritic anterior ischemic optic neuropathy (AAION). Here we present a series of case reports of GCA with their various presentations.

Keywords: Anterior ischemic optic neuropathy (AION), Giant cell arteritis (GCA), Jaw claudication, intermittent claudication


How to cite this article:
Das S, Khurana T, Tiwary R. Various etiologies and presentations of giant cell arteritis: A series of case reports. TNOA J Ophthalmic Sci Res 2022;60:78-82

How to cite this URL:
Das S, Khurana T, Tiwary R. Various etiologies and presentations of giant cell arteritis: A series of case reports. TNOA J Ophthalmic Sci Res [serial online] 2022 [cited 2022 Jun 29];60:78-82. Available from: https://www.tnoajosr.com/text.asp?2022/60/1/78/340340




  Introduction Top


Giant cell arteritis (GCA), also called temporal arteritis, is an inflammatory disease of medium-to-large blood vessels. GCA is the most common primary vasculitis in adults. Histopathologically, GCA is marked by generalized granulomatous inflammation of medium-to-large-sized vessels cause arterial ischemia.[1] In addition, high-vascular risk individuals who are diagnosed with GCA are usually elderly with age > 55 years with high-vascular risk and thus can have underlying atherosclerosis.[2] All these factors can pose a diagnostic dilemma for the physicians as GCA is a medical emergency which if left untreated can result in significant morbidity and mortality. Specifically, the average estimates of GCA range between 2.3/100,000 cases per year in the sixth decade of life to 44.7/100,000 cases per year in patients in their ninth decade. Furthermore, women are affected between two and six times more often than men. Based on the United States census data from 2000, the prevalence of GCA is approximately 160,000.[3] Since patients with GCA often present with vision loss, ophthalmologists are on the front lines of diagnosing the disorder.[4] In addition to vision loss, patients commonly note associated symptoms such as headache, jaw claudication, diplopia, myalgias, and constitutional symptoms.[5] The most commonly feared sequel of GCA is permanent visual loss secondary to arteritic anterior ischemic optic neuropathy (AION).[6] Here, we present a series of case reports of GCA with their various presentations.


  Case Reports Top


Case 1 (hypertension + chronic obstructive pulmonary disease + giant cell arteritis)

A 74-year-old male patient presented to our eye outpatient department (OPD) with complaints of rapid loss of vision in his right eye for 2 days. There was associated history of on off right-sided hemicranial severe pain for the past 3 months. He is a known case of hypertension and chronic obstructive pulmonary disease (COPD) for the past 3 years and was on regular medication. There was no history of an associated small joint pain, tenderness, and stiffness. There was no history of any jaw pain, shoulder pain, and chronic muscle ache. On examination, the patient was conscious and oriented. The vision was perception of light (PL) positive and perception of ray (PR) inaccurate in the right eye, whereas it was 6/9 in the left eye. Intraocular pressure was 14 mg in both the eyes. Relative afferent pupillary defect (RAPD) was present in the right eye, whereas in the left eye, pupillary response was normal. The patient was bilaterally pseudophakic. There was bilateral xanthelasma in the eyes with moderate scalp tenderness. Slit-lamp findings were normal. On fundus examination, there was pale optic disc in the right eye, whereas in the left eye, optic disc was normal. Retinal vessels were attenuated in both the eyes. There were no other retinal findings noted [Figure 1]. Considering the clinical diagnosis of GCA, high dose of oral prednisolone (1 mg/kg/bwt) per day was initiated. On investigation, erythrocyte sedimentation rate (ESR) was found 108. Blood pressure was 120/80 mmHg and pulse was 90/min. Rheumatoid factor (RA) was found negative. Computed tomography (CT) scan of the brain revealed diffuse cerebral atrophy. Ultrasonography color Doppler study of the temporal artery showed few areas of hypoechoic halos suggestive of arteritis. Medicine consultation was taken and was on follow-ups. After 1 month, the vision was improved to 6/60 in the right eye and there was no scalp pain. RAPD was persisting in the right eye. The patient was put on tapering dose of oral steroid – a 12-week regimen by medical specialist.
Figure 1: Hypertension + chronic obstructive pulmonary disease + giant cell arteritis

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Case 2 (cardiac and renal disease + branch retinal vein occlusion + giant cell arteritis + pituitary adenoma operated)

A 64-year-old male patient presented to the eye emergency with complaints of sudden loss of vision in the right eye. There was a preceding history of severe right-sided temporal headache 3 days back. He had a history of on-off diplopia and headache. There was no history of any associated fever, vomiting, and altered sensorium. There was no history of any joint pain and chronic muscle ache. There was no history of diabetes and hypertension. The patient was a known case of cardiac disease with left ventricular ejection fraction of 35% with renal disease. On examination, the patient had a visual acuity of PL negative in the right eye and hand movement close to the face in the left eye. Intraocular pressure was 12 mmHg in both the eyes. There was RAPD in the right eye. Temporal artery tenderness was present in the right side, whereas it was prominent and tortuous in the left side. Extraocular movements were full and free. The slit-lamp examination was normal. On fundus examination, there was a pale optic disc with splinter hemorrhages in the right eye with inferior branch retinal vein occlusion (BRVO), whereas it was normal in the left eye [Figure 2]. Urgent ESR was found 95 mm in h. A clinical diagnosis of GCA was made and put on high dose of oral prednisolone (1 mg/kg/bwt) per day in tapering manner. C-reactive protein was 157. Neurology consultation was taken. On noncontrast CT (NCCT) of the brain, there were the features of chronic infarct and a suprasellar mass lesion (pituitary adenoma). The patient was operated for tumor. On subsequent visit, there was no improvement of vision in the right eye, whereas in the left eye, it was 6/9 with spectacle.
Figure 2: Cardiac and renal disease + branch retinal vein occlusion + giant cell arteritis + pituitary adenoma

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Case 3: (rheumatoid arthritis and giant cell arteritis)

A 60-year-old female presented to our OPD with complaints of sudden loss of vision in her left eye. There was no history of ocular pain but had a history of on-off temporal headache with visual obscuration of vision. She is a known case of rheumatoid arthritis for the past 10 years and was on antirheumatic drugs. The small joints of the hands had typical swan-neck deformity [Figure 3]. Her blood pressure was 140/80 mmHg and sugar was 110 mg%. On ocular examination, her visual acuity was PL negative in the left eye and 6/18 in the right eye. Intraocular pressure was 12 mmHg in both the eyes. There was RAPD in the left eye, and in the right eye, papillary response was normal. Slit-lamp examination was unremarkable. On dilated fundus examination, the right eye was found normal, whereas there was a pale optic disc in the left eye. The temporal artery was found thick, tortuous, and tender. Urgent ESR was found 90 mm in h. A clinical diagnosis of GCA was made and put on high dose of oral prednisolone (1 mg/kg/bwt) per day in the tapering manner. C-reactive protein was 100. Neurology consultation was taken. On NCCT brain was normal. USG Doppler of temporal artery showed segmental lesions of hyperechoic halos, suggestive of GCA.
Figure 3: Rheumatoid arthritis and giant cell arteritis

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Case 4 (giant cell arteritis + intermittent claudication)

A 66-year-old male patient presented to our OPD with complaints of temporal headache and reduced vision in his right eye of 1 day duration. There was on and off history of temporal headache and tenderness for the past 3 months. There was no history of joint pain and shoulder pain. He had a history of on-off calf muscle pain that subsided on rest (claudication) along with chronic body aches. He is chronic smoker for the past 40 years. His blood pressure was 140/80 mmHg and sugar was 100 mg%. On ocular examination, his visual acuity was PL negative in the right eye and 6/18 in the left eye. Intraocular pressure was 12 mmHg in both the eyes. There was RAPD in the right eye, and in the left eye, papillary response was normal. Slit-lamp examination was unremarkable. On dilated fundus examination, there was a pale optic disc in the right eye, whereas the left eye was found normal [Figure 4]. Temporal artery was found thick, tortuous, and tender. Urgent ESR was found 99 mm in h. A clinical diagnosis of GCA was made and put on high dose of oral prednisolone (1 mg/kg/bwt) per day in the tapering manner. C-reactive protein was 95. Neurology consultation was taken. There was multiple lacunar infracts on NCCT brain. USG Doppler of the temporal artery showed segmental lesions of hyperechoic holos, suggestive of GCA.
Figure 4: Giant cell arteritis + intermittent claudication

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Case 5 (young patients + intermittent calf and jaw claudication + carotid thrombus + anterior ischemic optic neuropathy)

A 37-year-old male patient presented to our eye emergency with the complaint of sudden loss of vision in his left eye with preceding history of severe pain in his left temporal area and mandibular area 2 days back. There was no history of fever and vomiting. He is a known case of diabetes mellitus for the past 10 years and was on diabetic medication. He also had a history of chronic smoking and depression for which he was on antidepressive medication. Since his early first and second decades, he was suffering from intermittent claudication. His blood pressure was 120/80 mmHg and sugar was 100 mg%. On ocular examination, his visual acuity was PL negative in the left eye and 6/6 in the right eye. Intraocular pressure was 12 mmHg in both the eyes. There was RAPD in the left eye, and in the right eye, papillary response was normal. There was the acute onset of exotropia in his left eye. Color vision was defective in the left eye. The slit-lamp examination was unremarkable. On dilated fundus examination, there was pale optic disc in the left eye, whereas the right eye was found normal. The temporal artery was found normal and nontender. There was no tenderness elicited in calf muscle. There was weak pulsation of arteria dorsalis pedis. Mild jaw claudication was noticed while on examination. Urgent ESR was found 105 mm in h. A clinical diagnosis of query GCA and optic neuritis was made and put on high dose of oral prednisolone (1 mg/kg/bwt) per day in the tapering manner. C-reactive protein was 95. On perimetry, classical altitudinal field defect [Figure 5] was noticed and confirmed the diagnosis of AION. Neurology consultation was taken. Magnetic resonance imaging of the brain and orbit was unremarkable. USG Doppler of the temporal artery and artery dorsalis pedis was unremarkable. Carotid artery Doppler showed eccentric atheromatous plaque/thrombus in bilateral common carotid artery; however, no significant stenosis was there. Both vertebral arteries were found normal.
Figure 5: Young patients + intermittent calf and jaw claudication + carotid thrombus + anterior ischemic optic neuropathy

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  Discussion Top


GCA is a granulomatous vasculitis of unknown origin and targets large vessels with predominance for the aortic arch and the cranial branches. The underlying etiology of GCA is complex and has been widely researched yet is still not well understood. This includes genetic and possibly infectious factors, which go on to trigger an immune response. The average age of presentation is 72.5 years for women and 70.3 years old for men.[1],[2],[3] Furthermore, women are affected between two and six times more often than men. This increased incidence in women might be explained by the higher proportion of women in the elderly population.[4] When examining the role of race and epidemiology in GCA, white patients of Northern European descent have the highest rates (about 30 / 100,000 in Norway), whereas African, Asian, and Arab populations had the lowest rates (1.47 / 100,000 in Japan). Other risk factors may include smoking, low body mass index, early menopause, and relative adrenal hypofunction.[5] Some authors have recently reported a few subgroups of GCA. Hayreh et al. investigated 85 GCA patients, of which occult GCA, defined as ocular involvement without any systemic symptoms, occupied 21%.[7] De Boysson et al. presented 143 GCA patients, and GCA without cranial manifestations occupied 22%.[3] According to the studies in GCA without cranial symptoms, the characteristics were described as follows: lower inflammatory laboratory parameters, more frequent large arterial involvement, and less disease relapse.[3],[7],[8]

GCA should be presumed as a possibility in all elderly patients more than 50 years of age who present with a new-onset headache and vision changes.[2],[3] A high clinical suspicion should be maintained in such individuals while understanding the subtle clinical differences in GCA and carotid atherosclerosis such as vascular distribution and ophthalmological findings.[4],[5] The criteria established by the American College of Rheumatology (ACR)[3] can further aid in the diagnosis according to which a patient is deemed as having GCA if three of the following are present: (1) age over 50 years; (2) new-onset localized headache; (3) temporal artery tenderness or reduced pulse; (4) ESR of 50 mm/h or higher; and (5) temporal artery biopsy demonstrating mononuclear infiltration or granulomatous inflammation. GCA is considered a medical emergency due to the potential of irreversible vision loss.[9] Corticosteroids, typically high-dose prednisone (1 mg/kg/day), should be started as soon as the diagnosis is suspected (even before the diagnosis is confirmed by biopsy) to prevent irreversible blindness secondary to ophthalmic artery occlusion.[5],[6],[7] Injections of tocilizumab may also be used.[10]

Here, we present a series of case report whose symptoms of vision loss and headache were attributed to carotid stenosis but later was found to have GCA. Superficial temporal and maxillary arteries arise from carotid artery and stenosis of these vessels due to any cause such as GCA, atherosclerosis, thromboembolism result in ischemia of facial and masticatory muscles leading to headache, jaw pain, or claudication.[10] Our patient was thought to have these symptoms due to carotid atherosclerosis. However, as mentioned above, color duplex ultrasound showed segmental hypoechoic halos in the temporal arteries and carotid thrombus. This fact indicated further investigation for an alternate diagnosis such as GCA which can cause “skip” lesions of arteries[9] and can be missed on a limited ultrasonography study. Recent data have shown promising results regarding the use of color duplex ultrasonography in the diagnosis of GCA but only if done in detail on all the large arteries including temporal artery. This imaging modality can pick up signs such as “halo” sign due to inflammatory edema of the arterial wall with sensitivity and specificity as high as 85% and 92%, respectively.[9] Furthermore, the complaints of shoulder achiness and stiffness were highly suggestive of polymyalgia rheumatica (PMR) which is another rheumatic condition associated with GCA. 40%–60% of patients with GCA can have PMR on initial presentation while 16%–21% of patients with PMR develop GCA during their lifetime.[8],[9],[10]

GCA produces “skip” lesions due to segmental arterial wall inflammation.[9],[10] Considering these limitations, biopsy can be avoided, especially if the patient already fulfills ACR criteria. However, if unavoidable, a bilateral temporal artery biopsy should be performed while acquiring a longer biopsy specimen.[9]


  Conclusion Top


Carotid stenosis secondary to atherosclerosis is a common medical condition. However, there can be unique cases of carotid stenosis due to an uncommon cause such as GCA, thus necessitating a high clinical suspicion. We believe that the high degree of clinical suspicion and the acute onset of symptoms was sufficient enough to start high dose of steroid to save the vision.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Watelet B, Samson M, de Boysson H, Bienvenu B. Treatment of giant-cell arteritis, a literature review. Mod Rheumatol 2017;27:747-54.  Back to cited text no. 1
    
2.
Winkler A, True D. Giant cell arteritis: 2018 review. Mo Med 2018;115:468-70.  Back to cited text no. 2
    
3.
de Boysson H, Lambert M, Liozon E, Boutemy J, Maigné G, Ollivier Y, et al. Giant-cell arteritis without cranial manifestations: Working diagnosis of a distinct disease pattern. Medicine (Baltimore) 2016;95:e3818.  Back to cited text no. 3
    
4.
Klein RG, Hunder GG, Stanson AW, Sheps SG. Large artery involvement in giant cell (temporal) arteritis. Ann Intern Med 1975;83:806-12.  Back to cited text no. 4
    
5.
Lensen KD, Voskuyl AE, Comans EF, van der Laken CJ, Smulders YM. Extracranial giant cell arteritis: A narrative review. Neth J Med 2016;74:182-92.  Back to cited text no. 5
    
6.
Chaigne-Delalande S, de Menthon M, Lazaro E, Mahr A. Giant-cell arteritis and Takayasu arteritis: Epidemiological, diagnostic and treatment aspects. Presse Med 2012;41:955-65.  Back to cited text no. 6
    
7.
Hayreh SS, Podhajsky PA, Zimmerman B. Occult giant cell arteritis: Ocular manifestations. Am J Ophthalmol 1998;125:521-6.  Back to cited text no. 7
    
8.
Chacko JG, Chacko JA, Salter MW. Review of giant cell arteritis. Saudi J Ophthalmol 2015;29:48-52.  Back to cited text no. 8
    
9.
Czihal M, Zanker S, Rademacher A, Tatò F, Kuhlencordt PJ, Schulze-Koops H, et al. Sonographic and clinical pattern of extracranial and cranial giant cell arteritis. Scand J Rheumatol 2012;41:231-6.  Back to cited text no. 9
    
10.
Pfeil A, Oelzner P, Hellmann P. The treatment of giant cell arteritis in different clinical settings. Front Immunol 2018;9:3129.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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