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|Year : 2015 | Volume
| Issue : 5 | Page : 681-686
Giant cell arteritis in India: Report from a tertiary care center along with total published experience from India
Aman Sharma1, Vinay Sagar1, Mahesh Prakash2, Vikas Gupta3, Niranajan Khaire1, Benzeeta Pinto1, Varun Dhir1, Amanjit Bal4, Ashish Aggarwal1, Susheel Kumar1, Kusum Sharma5, Manish Rathi6, Ashim Das4, Ramandeep Singh7, Surjit Singh1, Amod Gupta7
1 Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 Department of General Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
4 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
5 Department of Medical Microbiology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
6 Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
7 Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
|Date of Web Publication||6-Oct-2015|
Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh
Source of Support: None, Conflict of Interest: None
Objectives: Giant cell arteritis (GCA) is a granulomatous large vessel vasculitis with very scarce data from India. The purpose of this study was to present a comprehensive data of all published Indian cases along with our experience from North India.
Materials and Methods: This was a retrospective study of all patients diagnosed to be having GCA according to the American College of Rheumatology criteria at a large tertiary care hospital. The demographic data, clinical, investigations, treatment details, and outcomes were noted. Details of all case series and case reports published from India were pooled along with our experience in order to generate a cumulative data of all cases from India. This was then compared with several large published case series from South America, Europe, and Asia.
Results: A total of 72 patients (17 patients in the present series and another 55 patients from other Indian case series and case reports) were identified. The findings of our study are similar to the studies published from other parts of the world, except for the onset of the disease a decade earlier, a male predilection, a lower temporal artery biopsy positivity, and a higher incidence of ophthalmic complications.
Conclusions: Indian patients with GCA have an earlier age of onset, male preponderance, and higher ophthalmic complications.
Keywords: Giant cell arteritis; halo sign; India; large vessel vasculitis; temporal arteritis; temporal artery biopsy; vasculitis
|How to cite this article:|
Sharma A, Sagar V, Prakash M, Gupta V, Khaire N, Pinto B, Dhir V, Bal A, Aggarwal A, Kumar S, Sharma K, Rathi M, Das A, Singh R, Singh S, Gupta A. Giant cell arteritis in India: Report from a tertiary care center along with total published experience from India. Neurol India 2015;63:681-6
|How to cite this URL:|
Sharma A, Sagar V, Prakash M, Gupta V, Khaire N, Pinto B, Dhir V, Bal A, Aggarwal A, Kumar S, Sharma K, Rathi M, Das A, Singh R, Singh S, Gupta A. Giant cell arteritis in India: Report from a tertiary care center along with total published experience from India. Neurol India [serial online] 2015 [cited 2019 Nov 15];63:681-6. Available from: http://www.neurologyindia.com/text.asp?2015/63/5/681/166543
| » Introduction|| |
Giant cell arteritis (GCA) is chronic granulomatous large vessel vasculitis involving aorta and its branches. It is seen almost exclusively in patients over 50 years of age and has a special predilection for extra-cranial branches of carotid arteries. Clinical features occur due to an ischemic insult to the involved vessels, with permanent vision loss being the most dreaded outcome.
Giant cell arteritis is common in the Northern European population and is rare in African Americans, native Americans, and Asians. The reports from India are either in the form of small case series or single case reports. We are reporting the first case series from North India and have also collated data of all the published case series and case reports from India. This paper gives a comprehensive overview of all the cases from different regions of India.
| » Materials and Methods|| |
The study included all those patients who attended the rheumatology outpatient clinic at a tertiary care teaching hospital and satisfied the American College of Rheumatology 1990 classification criteria for GCA [Table 1]. This is a retrospective study from 2008 to 2014 in which records of the patients who were diagnosed as having GCA were reviewed. The demographic profiles, clinical features, laboratory investigations such as hemoglobin, total leukocyte count, liver function tests, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and temporal artery duplex ultrasound (USG) were recorded. Temporal artery biopsy (TAB) was performed from the most affected side (irrespective of the duration of immunosuppressive treatment administered) and the histopathological findings were recorded. The details of all case series and case reports published from India identified in PubMed search were noted (excepting a case series of four patient with GCA where clinical details were not described). The keywords included 'giant cell arteritis', 'temporal arteritis' and 'India'. A total of 47 cases have been published from South and Western India, and only 4 cases have been published from Eastern India.,,,, Details of the four single case reports were also included in the pooled Indian data.,,, These were then compared with case series from Europe (Spain), South America (Brazil), and Asia (Japan).,, The study was approved by the Institute ethics committee.
|Table 1: American College of Rheumatology criteria for classification of giant cell arteritis|
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| » Results|| |
A total of 17 patients, fulfilling the classification criteria for GCA, were included. The details of these patients are shown in [Table 2]. The mean age at presentation was 67 years (range 54-82 years). The most common symptom was headache, which was present in all patients, was bilateral in 5 (29.4%), and was localized to temporal, occipital, and parietal regions in rest of the patients. There was a delay of 53 days (range 4-180 days) in making the diagnosis. Diminution of vision was the second most common symptom seen in more than half of the patients. Nine of these patients had unilateral vision loss whereas one patient had bilateral vision loss. Jaw claudication was present in nine patients, while almost half of the patients had features of polymyalgia rheumatica (PMR). Scalp tenderness and thickened or tender temporal artery were identified in 13 and 12 patients, respectively. Fever was documented in only 3 patients. Constitutional symptoms such as malaise/fatigue, anorexia, and weight loss were reported only in 5, 3 and 3 cases, respectively. The mean ESR was 69 mm in the 1st hour. CRP was obtained in 11 patients and was elevated in 8 of them. Superficial temporal artery biopsy (TAB) was planned in all patients except 4 who did not give their consent for it. Among the 13 patients who underwent TAB, only 5 biopsies were consistent with GCA.
Prednisolone (1 mg/kg/day) was given to all the patients suffering from GCA except 2 patients, who were given intravenous methyl prednisolone. High dose steroids were continued for 3-4 weeks and were gradually tapered off with a close watch on the clinical and laboratory parameters. After institution of corticosteroids, headache subsided in all patients, there was resolution of associated symptoms of PMR but the visual impairment was persistent (in all those who presented with vision loss). Of the 2 patients who had a relapse, one received azathioprine, and the other who relapsed twice on tapering the doses of steroids received tocilizumab. He received nine monthly pulses of tocilizumab and is presently doing well. The comparison of various case series and case reports from India with the present case series, cumulative Indian data, and case series from Spain, Brazil, and Japan, are shown in [Table 3].
|Table 3: Comparison of various case series and case reports from India with the present case series, cumulative Indian data and case series from Spain, Brazil, and Japan|
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| » Discussion|| |
GCA is a disease of the elderly and is almost exclusively seen in individuals over 50 years of age. While the reported age in most of the published series from different parts of the world is above 70 years, the mean age in our present study was 67 years and this was similar to the mean age of most of the case series and case reports from India.,,,,,, The other difference in our case series was the male predominance while most case series have reported either no gender variation or a female predominance.,,,,, Our experience was similar to some of the case series from India and cumulative Indian data where there was a male predominance., The cause of this gender variation is not known but one explanation may be that it might be due to the difference in health seeking behavior among the two genders.
The mean duration of symptoms in our series was 53 days (range 4-180 days), which was similar to that of the studies from South India and Japan.,, However, other studies from Western and Eastern India had a longer delay in establishing the diagnosis.,, The reason for this may be the lack of awareness of this disease entity owing to its rarity in India.
The typical manifestations of GCA include headache, constitutional symptoms, symptoms of PMR, scalp pain or tenderness, jaw claudication, and visual disturbances. In the present series, headache was the most common symptom, followed by visual impairment. This was similar to the series from Brazil and other Indian series but higher than in case series from Japan (89.5%) and Spain (86.4%).,,,,,,, Fever was present in only 3 of our patients while it was reported more frequently in other Indian series (28-56%)., However, its frequency in cumulative Indian data was similar to that reported from Japan and Brazil., PMR is closely linked to GCA. It has been seen that 16-21% of patients with PMR develop GCA later in the course of the disease, whereas conversely, 58% of patients with GCA have PMR., Seven (41%) of our patients had PMR, whereas percentages in other series from India vary from 11% to 75%.,,,, PMR was as common in our series, as in the cumulative Indian data, and the series from Spain and Brazil., Visual manifestations are usually preceded by the development of amaurosis fugax later on leading to partial or complete vision loss. In our series, visual impairment was the second most common manifestation (58.8%), and this unexpected finding could be explained by selection and referral bias of a tertiary care hospital. The patients presented at a late stage of vision loss due to lack of awareness of the existence of this entity among the treating physicians due to its rarity. All other Indian series have reported visual manifestations at a much lower frequency (18-28%) but an almost similar incidence was described from the Brazilian series with acute ischemic optic neuropathy (AION) being the most common fundus finding.,,,,, One large cohort from Spain had observed visual manifestations in 26.1% of biopsy proven GCA patients with AION being the most common cause of permanent visual loss (91.7%). Jaw claudication is one of the ischemic manifestations seen in about 50% of the patients. It was present in 53.3% of patients in our series. This figure was almost identical to that seen in most of the other previous Indian series., The incidence in the cumulative Indian data (40.3%) as well as the Spanish (40.8%), and Brazilian (48.9%) series was also almost identical., On examination, tender or thickened temporal artery was present in 70.6% of patients, while other Indian series have reported this finding in 22-100% of patients. This figure was approximately similar to the cumulative Indian data and series from Japan. Scalp tenderness, a very characteristic examination finding, was present in 13 patients. However, the cumulative Indian data and series from Spain have demonstrated this finding with much lower frequency.
The characteristic inflammatory markers that are often found elevated in GCA are ESR and CRP. ESR and CRP may be normal in 4% of TAB proven GCA patients. One of our patient had a normal ESR. The mean ESR in our series was 69 mm in the first hour (range 10-124), which is much lower than that observed in the previous Indian studies (mean 90.8-106.5) and the series from Spain (92) and Brazil (78.4).,,,,,, Other inflammatory mediators such as leukocytosis and thrombocytosis were evident in one and two patients, respectively. Duplex ultrasonographic abnormalities observed in patients with GCA include stenosis or occlusion of the temporal artery or the presence of a hypoechoic halo (halo sign) around the involved temporal artery. Although duplex USG detects the halo sign in 73% of biopsy proven GCA, biopsy consistent with GCA was found in only 40% of the suspected GCA patients in whom the halo sign was demonstrated., Recently, a meta-analysis observed the sensitivity and specificity of halo sign to be 69% and 82%, respectively. Duplex USG was performed in 6 patients in the current series, and out of them, 4 had a halo sign or a thickened temporal artery. In one previous series from India, a color Doppler USG was performed in 9 patients and a “halo sign” observed in 6 (66.7%) patients. The cumulative Indian data and the series from Japan demonstrated findings suggestive of halo sign in 79 and 83% patients, respectively. Pure PMR patients generally do not require a TAB. These patients, however, should be followed closely as a small proportion of them may later show evolution of the disease to a fully manifest GCA despite being on low dose steroids. As has already been emphasized, the patients with isolated clinical PMR when subjected to TAB, demonstrated histopathologic findings consistent with GCA in 16-21% patients in the Olmsted County and 23% in the Spanish cohort.,, In the present series, TAB was performed in 13 patients and features consistent with GCA were evident in 5 (38.5%) patients. In the previous series from India, TAB consistent with GCA were seen in 71-91% of the total TAB performed.,,,, However, the series from Brazil (89%) and Japan (73.3%), and the cumulative Indian data (75.4%) had a much higher number of patients with the biopsy consistent with GCA., A much lower sensitivity of 15.9% was reported in a series of 44 patients from UK.
Steroids (prednisolone) are recommended as the established treatment of GCA. They should be initiated at the dose of 1mg/kg/day and should be instituted promptly when the diagnosis is strongly suspected, even before the latter is being confirmed with a TAB. In case vascular complications such as visual loss coexist, the therapy should be started immediately with an intravenous high dose (1 g daily for 3 days) methylprednisolone pulse. If the TAB is not conclusive of GCA and yet, the clinical suspicion of GCA remains strong, steroids should be continued. It has been observed that once visual loss is established, it is rarely possible to recover useful vision. Thus, prevention of visual loss is essential, particularly at the stage of initial symptoms (including amaurosis fugax or diplopia), with the usage of parenteral steroid therapy. Our patients received oral prednisolone 1mg/kg/day as soon as the diagnosis was suspected; 2 patients, however, received intravenous methylprednisolone and both of them had an urgent indication (visual loss) for instituting high dose immunosuppression. Twenty three Brazilian and one Japanese patients were also treated with high dose intravenous methylprednisolone for similar neuro-ophthalmologic indications., Our 2 patients had a recurrence of symptoms on tapering the steroids. They both later received azathioprine and tocilizumab and are now doing well. The recurrence rate reported in the cumulative Indian data (8.3%) was very low as compared to the series from Japan (26.3%) and Brazil (29%)., Most of the episodes of relapse were treated with introduction of more intense immunosuppression and the patients responded well.
| » Conclusion|| |
Giant cell arteritis is a chronic granulomatous medium- to- large vessel vasculitis. It is a rare entity in the Indian subcontinent, and early recognition and prompt institution of immunosuppressive treatment is required to prevent disabling consequences. Most of the findings in our study were similar to the previous series from other parts of the world except for its occurrence almost a decade earlier, the presence of a male predominance, a low rate of TAB positivity, and the higher incidence of ophthalmological complications.
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Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2], [Table 3]