scholarly journals Giant Cell Arteritis: From Neurologist’s Perspective

2021 ◽  
Author(s):  
Ravish Rajiv Keni ◽  
M. Sowmya ◽  
Sreekanta Swamy

Giant cell arteritis (GCA) is a granulomatous vasculitis affecting large- and medium-sized arteries in the elderly and potentially causes visual loss. In an elderly patient presenting with acute pain in the distribution of the external carotid artery (e.g., headache, scalp tenderness); polymyalgia rhematica; or acute/transient visual loss or diplopia; a possibility of GCA should be considered in one of the differential diagnosis. Urgent laboratory evaluation (e.g., ESR, CRP, platelet count), followed immediately by empiric high-dose corticosteroid therapy is warranted in patients suspected of having GCA. Although ultrasound techniques are sensitive for the diagnosis of GCA, TAB remains the best confirmatory test. Patients with GCA often require long durations of steroid therapy and steroid-related complications are common. Multidisciplinary care and the use of steroid-sparing regimens are warranted in case of relapse.

2020 ◽  
pp. 1-5
Author(s):  
Purnima Mehta ◽  
Faaiq Hassan ◽  
Muhammed Omar Qadir ◽  
Shirish Dubey ◽  
Sergio Pagliarini ◽  
...  

Background: Giant cell arteritis (GCA) is the most common type of systemic vasculitis affecting the elderly. Ophthalmic presentations of GCA in particular can be difficult to identify prior to permanent visual loss occurring. Methods: Here, we present 3 challenging cases as a retrospective series to highlight the variable presentations of GCA with ophthalmic involvement, but GCA was not suspected due to atypical presentation. Results: Unfortunately, all 3 cases went on to develop visual loss in the affected eye due to a delay in diagnosis or treatment. The authors wish to highlight the challenges posed to the referring clinicians, when patients had systemic/ocular co-morbidities, which delayed the suspicion of GCA Conclusion with a Practical Point: Our cases highlight the variable presentations of this condition as well as the devastating ophthalmic implications that GCA can have. A high index of suspicion must be maintained; particularly in elderly patients with atypical presentations.


2018 ◽  
pp. bcr-2018-225304
Author(s):  
Gesine Boisch ◽  
Sven Duda ◽  
Christian Hartmann ◽  
Heinrich Weßling

We report the rare case of an 80-year-old male patient with hypertrophic pachymeningoencephalitis that may be associated with temporal arteritis. The patient presented to our neurological department with a 2-week history of latent paresis and ataxia affecting his right hand. He had been diagnosed with temporal arteritis 12 years earlier. Brain MRI showed an enhancement of the left-sided frontoparietal meninges with oedema of the adjacent tissue of the precentral and postcentral cortex. A leptomeningeal biopsy was performed. An autoimmune-mediated immunoglobulin G4-associated hypertrophic pachymeningoencephalitis was diagnosed. The patient received a high-dose corticosteroid therapy and his symptoms gradually improved. Our results suggest that hypertrophic pachymeningoencephalitis may occur as a complication of giant cell arteritis and may cause central neurological deficits by cerebral perifocal oedema.


2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Andre Grixti ◽  
Vineeth Kumar

Giant cell arteritis (GCA) is an ophthalmic emergency which requires early diagnosis and treatment with high dose systemic corticosteroids in order to prevent permanent visual loss. However, systemic corticosteroids have significant ocular side effects including cataract formation, raised intraocular pressure, and less commonly, central serous chorioretinopathy (CSCR). We report a case of visual loss secondary to CSCR complicating corticosteroid therapy in GCA. When assessing patients with systemic conditions such as GCA or other vasculitic process, who complain of visual loss which is getting worse on corticosteroid treatment, clinicians should consider other causes such as CSCR as part of the differential diagnosis. Extra caution should be exercised in such cases as increasing the dose of corticosteroids might aggravate CSCR resulting in further visual loss.


2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Jose R. Zaragoza ◽  
Natalia Vernon ◽  
Gisoo Ghaffari

Giant cell arteritis (GCA) is a systemic vasculitis of medium and large arteries that mainly affects the external carotid artery. It is a diagnosis of the elderly that typically presents as low-grade fever, temporal tenderness, claudication of the jaw, and in some patients vision loss. In cases where GCA presents with atypical manifestations, the diagnosis may be more difficult, causing a delay in both diagnosis and treatment and ultimately leading to irreversible complications. In this paper, we present an atypical presentation of GCA with symptoms of neck swelling and lingual pain in an elderly female. These symptoms progressed to bilateral necrosis and eventual dislodgement of the tongue. Lingual necrosis is a severe potential complication in GCA. In patients presenting with lingual swelling, pain, and discoloration, GCA should be suspected and prompt therapy should be initiated to avoid irreversible complications.


2019 ◽  
pp. 9-14
Author(s):  
Matthew J. Thurtell ◽  
Robert L. Tomsak

Arteritic ischemic optic neuropathy occurs in the setting of giant cell arteritis and often produces devastating irreversible vision loss. It is a medical emergency because there is a high risk of fellow eye involvement if high-dose corticosteroid treatment is not initiated in a timely fashion. In this chapter, we begin by reviewing the clinical features of anterior and posterior ischemic optic neuropathy. We next review the clinical features of arteritic ischemic optic neuropathy. We then discuss the clinical features and workup for giant cell arteritis. Lastly, we review the initial and long-term management approach for ischemic optic neuropathy occuring in the setting of giant cell arteritis.


2018 ◽  
Vol 69 (1) ◽  
pp. 152-154
Author(s):  
Vasilica Cristescu ◽  
Aurelia Romila ◽  
Luana Andreea Macovei

Polymyalgia rheumatica is a disease that occurs mostly in the elderly and is rarely seen in patients less than 50 years of age. Polymyalgia rheumatica is a vasculitis, which manifests itself as an inflammatory disease of the vascular wall that can affect any type of blood vessel, regardless of its size. It has been considered a form of giant cell arteritis, involving primarily large and medium arteries and to a lesser extent the arterioles. Clinical manifestations are caused by the generic pathogenic process and depend on the characteristics of the damaged organ. PMR is a senescence-related immune disorder. It has been defined as a stand-alone condition and a syndrome referred to as rheumatic polyarteritis with manifestations of giant cell arteritis (especially in cases of Horton�s disease and temporal arteritis) which are commonly associated with polymyalgia. The clinical presentation is clearly dominated by the painful girdle syndrome, with a feeling of general discomfort. Polymyalgia and temporal arteritis may coexist or be consecutive to each other in the same patient, as in most of our patients. The present study describes 3 cases of polymyalgia rheumatica, admitted to the Clinic of Rheumatology of Sf. Apostol Andrei Hospital, Galati. The cases were compared with the literature. Two clinical aspects (polymyalgia rheumatica and/or Horton�s disease) and the relationship between them were also considered. Polymyalgia rheumatica is currently thought to have a multifactorial etiology, in which the following factors play a role: genetic factors or hereditary predisposition (some individuals are more prone to this disease), immune factors and viral infections (triggers of the disease). Other risk factors of polymyalgia rheumatica include age over 50 years and the association with giant cell arteritis. The characteristic feature of the disease is girdle pain, with intense stiffness of at least one hour�s duration. Markers of inflammation, erythrocyte sedimentation rate and C-reactive protein are almost always increased at the onset of the disease. Diseases that can mimic the clinical picture of polymyalgia rheumatica are neoplasia, infections, metabolic disorders of the bone and endocrine diseases.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 383-384
Author(s):  
T. Kise ◽  
E. Takamasu ◽  
Y. Miyoshi ◽  
N. Yokogawa ◽  
K. Shimada

Background:Temporal artery biopsy (TAB) is the gold standard for diagnosing giant cell arteritis (GCA). However, previous studies have reported that the discordance rate of TAB is 3-45%,i.e., in unliteral TAB, GCA may be overlooked in one in five patients, approximately. Evidence as to whether bilateral TAB should be performed initially or one-sided TAB is sufficient for diagnosing GCA is lacking.Objectives:To investigate the predictors of patients with GCA in whom one-sided TAB is sufficient.Methods:The present study was a cross-sectional, single center study conducted from April 1, 2011 to July 31, 2019 at Tokyo Metropolitan Tama Medical Center. Of all consecutive GCA cases for which bilateral TAB was performed, bilaterally positive cases and unilaterally positive cases were extracted as bilateral positive group (BPG) and unilateral positive group (UPG), respectively. GCA was defined in accordance with the classification criteria of the 1990 American College of Rheumatology, and GCA was diagnosed if no other etiology was found within six months after beginning of high-dose glucocorticoid treatment. Demographic, clinical and laboratory data were obtained from the medical records, and the BPG and the UPG were compared statistically in each variable. Statistical significance was defined asp< 0.05.Results:During study, 264 biopsies were performed for 145 cases, who suspected GCA and underwent TAB. The pathological positivity rate was 26.1% (68 / 264 biopsies). Of these, 53 cases had final diagnosis of GCA, in which 43 cases were biopsy proven GCA. Thirty-seven biopsy proven GCA with bilateral TAB were enrolled; 64.9% women; mean (SD) age 75 (8.9) years; median [IQR] TAB length 17.5 [13.0,20.0] mm; headache 54.1%; jaw claudication 45.9%; scalp tenderness 16.2%; temporal artery (TA) tenderness 32.4%; TA engorgement 32.4%; TA pulse abnormality 5.4%; visual symptoms 2.7%; a fever of 38.5°C or higher 40.5%; shoulder girdle pain 48.6%; imaging of aortitis or arteritis 40.5%; median [IQR] white blood cell 9,100 [7200, 12050] /μl; median [IQR] platelet cell 37.5 [27.0, 46.3] ×104/μl; median [IQR] C-reactive protein (CRP) 10.1 [3.9, 16.5] mg/dL; erythrocyte sedimentation rate [IQR] 105 [66, 129] mm/h. Thirty-one in 37 cases were positive bilaterally while 6 in 37 cases were positive unilaterally; and the discordance rate was 16.2%. The median sample length after formalin fixation was 19.0 mm for the BPG and 14.5 mm for the UPG (p= 0.171). The parameters above were compared between UPG and BPG. Of these, only the serum CRP value (mg/dL) differed statistically between groups, and the median value of the two groups was 10.6 and 6.5, respectively (median test:p= 0.031). To predict BPG, in whom unilateral TAB is sufficient for diagnosing GCA, the cut-off value of serum CRP with a specificity of 100% and a sensitivity of 61.3% was set at 9.3 mg/dL (ROC analysis: AUC 0.726).Conclusion:When the serum CRP level is 10 mg/dL or higher in GCA suspected patients, an unilateral TAB alone was sufficient for an accurate diagnosis.References:[1]Hellmich, B, et al.Ann Rheum Dis2020;79(1):19-30.[2]Breuer, GS, et al.J Rheumatol. 2009;36(4):794-796.[3]Czyz CN, et al.Vascular2019;27(4):347-351.[4]Durling B, et al.Can J Ophthalmol2014;49(2):157-161.Figure.Comparison of median CRP levels between unilaterally positive group and bilaterally positive group.Disclosure of Interests:None declared


BMJ ◽  
2009 ◽  
Vol 338 (may06 1) ◽  
pp. b1809-b1809 ◽  
Author(s):  
C. M Guly ◽  
J. A Olson

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