scholarly journals P038 Is it all in the eyes?

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Anil Gharatya ◽  
Julekha Wajed ◽  
Natalia Cernovschi ◽  
Dimitrios Christidis
Keyword(s):  
Differential Diagnosis ◽  
Myasthenia Gravis ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Inflammatory Markers ◽  
British Society ◽  
Ischemic Optic Neuropathy ◽  
Temporal Artery Biopsy ◽  
Blurred Vision ◽  
Ocular Symptoms

Abstract Background/Aims  The ocular manifestations of giant cell arteritis (GCA) and ocular myasthenia gravis (OMG) have significant overlap, which can lead to clinical uncertainty in the absence of awareness and routine diagnostic testing. Methods  We present the case of a 63-year-old male patient with a strong family history of autoimmune disease. He presented to the Rheumatology department in 2009 with symptoms of polymyalgia rheumatica (PMR) and was treated successfully with a reducing regime of Prednisolone. In 2013, he presented to the Ophthalmology department with blurred vision and diplopia. Giant cell arteritis was suspected and a high dose of Prednisolone was started. His temporal artery biopsy was normal and inflammatory markers were not raised, but there was a complete resolution of his symptoms since starting steroids. Under the care of the Rheumatology department, reducing regimes of Prednisolone were attempted and a steroid sparing agent, Methotrexate was introduced. Unfortunately, he continued to have recurring symptoms of blurred vision and diplopia, particularly at Prednisolone doses less than 20mg. Over the course of a few years, there was a progression of his ocular symptoms. He developed a horizontal diplopia, monocular ptosis and a 4th nerve palsy. He was referred back to the Ophthalmology department, where alternate diagnoses were investigated. Anti-acetylcholine receptor antibodies (AChR) were strongly positive at 35 mU/L (normal <0.44 mU/L). MRI head was normal, and CT chest showed no thymoma. Single fibre EMG confirmed severe OMG. Our patient was subsequently commenced on Pyridostigmine, with which symptoms improved. Results  The literature on OMG mimicking GCA is sparse. The prevalence of an exclusively ophthalmic presentation in patients with GCA is 1 in 5. Ocular signs and symptoms in GCA can vary drastically due to the propensity of the disease to affect different structures within the eye. Transient and permanent visual loss, affects 10-30% of GCA patients due to anterior ischemic optic neuropathy. Diplopia affects 5% of GCA patients. In comparison, OMG can be the only presenting feature in 15% of patients with Myasthenia Gravis, and is characterised by unilateral ptosis, oculomotor paresis and binocular diplopia. Conclusion  This case highlights the clinical similarities of OMG and GCA, and that OMG should be considered as a differential diagnosis when the clinical picture of GCA does not truly fit. In patients presenting with GCA with ocular symptoms, normal inflammatory markers and no systemic features, OMG should be considered and AChR antibody testing be performed. More awareness of OMG is needed. We also propose that OMG should be included as a differential diagnosis, in the British Society of Rheumatology guidelines for GCA. Disclosure  A. Gharatya: None. J. Wajed: None. N. Cernovschi: None. D. Christidis: None.

Neuro-Ophthalmic Complications of Biopsy-Proven Giant Cell Arteritis

1997 ◽  
Vol 7 (4) ◽  
pp. 375-382 ◽  
Author(s):  
S. Glutz Von Blotzheim ◽  
F.-X. Borruat
Keyword(s):  
Fluorescein Angiography ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Artery ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Ischemic Optic Neuropathy ◽  
Temporal Artery Biopsy ◽  
Choroidal Ischemia ◽  
Retinal Artery

Purpose To define the spectrum of neuro-ophthalmic complications and clinical presentations of patients with giant cell arteritis (GCA). Methods Retrospective study (1977-1994) of clinical charts, fundus photographies and fluorescein angiographies of 66 patients with temporal artery biopsy positive for GCA. Results Clinical data were adquate for 47 patients. Headaches were reported by 83%, weight loss in 73%, jaw claudication in 68%, scapular pain in 64% and asthenia in 57%. Erythrocyte sedimentation rate was normal in 15%. Neuro-ophthalmic complications were present in 33 cases (70%), including anterior ischemic optic neuropathy (22 cases), choroidal ischemia (17 cases), central or branch retinal artery occlusion (seven cases), and oculomotility disturbances (four cases). Fluorescein angiography was very helpful for detecting choroidal ischemia (80.9% of our cases). Twenty-one patients presented with involvement of several distinct orbital arterial territories and one very unusual patient suffered from an orbital infarction (i.e. ischemia of all orbital structures). Conclusions In our series, two-thirds of biopsy-proven GCA patients presented with neuro-ophthalmic complications, ranging from transient visual loss to orbital infarction. Involvement of more than one orbital vascular territory is highly suggestive of an arteritic process. Clinicians should keep in mind the possibility of GCA even when ESR is normal, and fluorescein angiography should be performed. The finding of choroidal ischemia should prompt temporal artery biopsy and steroid therapy.

scholarly journals Myasthenia Gravis and Stroke in the Setting of Giant Cell Arteritis

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Elli-Sophia Tripodaki ◽  
Sotirios Kakavas ◽  
Ioanna Skrapari ◽  
Dimitrios Michas ◽  
Giorgios Katsikas ◽  
...  
Keyword(s):  
Case Report ◽  
Myasthenia Gravis ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Inflammatory Markers ◽  
Low Grade ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Laboratory Findings ◽  
Receptor Antibodies

This case report concerns the diagnosis of two independent chronic diseases in a patient hospitalized for stroke, myasthenia gravis (MG) and giant cell arteritis (GCA). MG has been found to be associated with several diseases, but there are very few cases documenting its coexistence with GCA. We report the case of a 79-year-old woman initially hospitalized for stroke. Patient’s concurrent symptoms of blepharoptosis, dysphagia, and proximal muscle weakness were strongly suggestive of myasthenia gravis. The persistent low-grade fever and elevated inflammatory markers in combination with the visual deterioration that developed also raised the suspicion of GCA. Histological examination confirmed GCA, while muscle acetylcholine receptor antibodies were also present. Even though in medicine one strives to interpret a patient’s symptoms with one diagnosis, when one entity cannot fully interpret the clinical and laboratory findings, clinicians must consider the possibility of a second coexisting illness.

scholarly journals Peripheral retinal arteriolar leakage in giant cell arteritis: a case report

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Meleha Ahmad ◽  
Andrew R. Carey ◽  
Charles G. Eberhart ◽  
Sepideh Siadati ◽  
Amanda D. Henderson
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Vision Loss ◽  
Small Vessel ◽  
Oral Steroid ◽  
Small Vessel Vasculitis ◽  
High Dose ◽  
Ischemic Optic Neuropathy ◽  
Temporal Artery Biopsy ◽  
Retinal Arteriolar

AbstractA 76-year old African American female with a history of arteritic ischemic optic neuropathy (AION) secondary to biopsy-proven giant cell arteritis (GCA) presented with unilateral vision loss in her contralateral eye despite high-dose oral steroid treatment. Dilated fundus examination revealed three cotton wool spots. Fluorescein angiography showed slowed arteriolar filling with late staining of small peripheral arteries, consistent with small vessel arteritis. Laboratory tests for alternative vasculitides were negative. Review of her temporal artery biopsy specimen confirmed lymphoplasmacytic inflammation around small adventitial vessels with no destructive granulomatous or leukocytoclastic small vessel vasculitis, consistent with GCA. Our unique case demonstrates peripheral small vessel retinal arteriolar leakage in GCA, which is a rare finding. This association is of interest because GCA is commonly associated with medium to large vessel pathology without small vessel involvement.

scholarly journals P14. Investigations prior to temporal artery biopsy: a retrospective audit of compliance with the British Society for Rheumatology Guidelines for giant cell arteritis

Rheumatology ◽  
2014 ◽  
Vol 53 (suppl 2) ◽  
pp. i17-i17
Author(s):  
C. S. Ramakumaran ◽  
S. Donaldson ◽  
A. W. Morgan ◽  
A. Chakrabarty ◽  
C. T. Pease ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Artery ◽  
British Society ◽  
Temporal Artery Biopsy ◽  
Retrospective Audit

THU0291 IS GIANT CELL ARTERITIS REALLY GIANT CELL ARTERITIS? A HISTOLOGICAL REVIEW OF TEMPORAL ARTERY BIOPSIES FROM A UK DISTRICT GENERAL HOSPITAL

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 373.1-373
Author(s):  
S. K. Amar ◽  
D. Christidis ◽  
G. Kousparos ◽  
M. Lloyd
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Inflammatory Infiltrate ◽  
Temporal Artery ◽  
Giant Cells ◽  
The Body ◽  
British Society ◽  
Temporal Artery Biopsy ◽  
Intimal Thickening ◽  
Inflammatory Lesions

Background:Despite the advent of newer imaging techniques, temporal artery biopsy (TAB) retains a key role in the diagnosis of giant cell arteritis (GCA). The classical histological description of GCA is that of granulomatous lesions characterized by a transmural inflammatory infiltrate1. Giant cells are typically noted in the internal elastic lamina. Vascular remodeling and structural changes are also frequently described, with intimal hyperplasia or fragmentation, fibrosis and calcifications1.Objectives:To identify the type and location of the inflammatory lesions in TAB-positive cases of GCA.Methods:We conducted a retrospective analysis of all TABs undertaken at our unit between 2011- 2018 with clinical record review. TABs were performed by vascular, ophthalmology and ENT teams.Results:379 TABs were reviewed of which 68 (17.9%) were reported as positive and 10 (2.6%) were equivocal (presence of fragmentation and intimal thickening). Of the TAB-positive cases, 43 (63.2%) were greater than 1cm in keeping with the British Society for Rheumatology guidance and 65 (95.6%) were biopsies in patients on corticosteroids at the time of procedure. The following tables demonstrate the frequency of the type and location of the inflammatory lesions detected in TAB-positive cases of GCA.Type of inflammatory lesionFrequencyChronic inflammatory infiltrate (lymphocytes, macrophages, plasma cells)66Giant cells41Intimal thickening22Intimal fragmentation33Location of inflammatory infiltrateFrequencyFull thickness32Intima only7Intima and Media only3Media only7Media and Adventitia only8Adventitia only4Intima and Adventitia only3Not recorded4Conclusion:Only 60% of our TAB-positive biopsies had giant cells present. Although perhaps surprisingly low, this finding is similar to other studies1,2. It emphasises the need to review the body of a report as well the conclusion. Other non-giant cell features present in positive reported biopsies may suggest a less certain diagnosis and prompt clinical review. There was considerable variablity in the style of reporting. With no standardised scoring system in place, the variable spectrum of inflammation and differences in reporting, there is the potential for inconsistencies amongst pathologists in interpreting and recording TAB results. Consistent reporting templates and close collaboration between rheumatologists and pathologists is needed to help correlate clinical, laboratory and imaging findings.References:[1]Cavazza A, Muratore F, Boiardi L, Restuccia G, Pipitone N, Pazzola G, et al. Inflamed temporal artery: histologic findings in 354 biopsies, with clinical correlations. Am J Surg Pathol. 2014;38(10):1360-70.[2]Hernandez-Rodriguez J, Murgia G, Villar I, Campo E, Mackie SL, Chakrabarty A, et al. Description and Validation of Histological Patterns and Proposal of a Dynamic Model of Inflammatory Infiltration in Giant-cell Arteritis. Medicine (Baltimore). 2016;95(8):e2368.Disclosure of Interests:Soha Khaled Amar: None declared, Dimitrios Christidis: None declared, George Kousparos: None declared, MARK LLOYD Speakers bureau: £700 into department fund

scholarly journals P36 Never say never: two cases of PET-CT negative but biopsy proven giant cell arteritis

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Sameena Khalid ◽  
James Maxwell
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Predictive Value ◽  
Inflammatory Markers ◽  
Systemic Vasculitis ◽  
Temporal Artery Biopsy ◽  
First Line ◽  
Post Discharge ◽  
Visual Aura ◽  
Pet Ct

Abstract Background Giant cell arteritis (GCA) usually affects the temporal arteries but can result in systemic vasculitis that can be seen on Positron Emission Tomography-Computed Tomography (PET-CT). Currently PET-CT is not used first line to diagnose cranial-GCA and temporal artery biopsy (TAB) remains the most common investigation to assess for this. There have been few studies performed to assess the use of PET-CT as an initial diagnostic tool in cranial-GCA. One study performed PET-CT for suspected GCA in their cohort of 64 patients. They found, when compared with TAB, global GCA assessment by PET-CT had a sensitivity of 92%, specificity of 85%, positive predictive value of 61% and negative predictive value of 98%. They concluded PET-CT had good diagnostic accuracy when compared with TAB and can be used as a first-line test to assess for GCA and rule out lower-risk GCA. Another study used PET-CT in steroid-naïve patients and also showed a high accuracy (sensitivity 64% and specificity of 100%) in diagnosing cranial artery inflammation. Methods We present two cases where the patient had symptoms of headache, constitutional symptoms and raised inflammatory markers, however PET-CT did not suggest GCA. The patients were subsequently re-referred later with ongoing symptoms and TAB confirmed GCA. Results Case 1: A 71-year-old woman presented with a one-month history of intermittent right sided headache and rigors. Her inflammatory markers were elevated (CRP of 115mg/L and ESR of 57mm/hr). Suspecting GCA, prednisolone 30mg od was commenced. Upon rheumatology review, it was felt her symptoms were atypical for GCA. Prednisolone was discontinued, and a PET-CT scan was arranged. This was performed ten days later and did not show any features of vasculitis. Six weeks later, the patient presented to ophthalmology reporting visual aura, ongoing headache with elevated inflammatory markers. Prednisolone 60mg od was commenced and a TAB was arranged. This was reported as partially treated GCA. Case 2: A 73-year-old gentleman had a prolonged admission for pyrexia of unknown origin. Headache was present throughout admission and rheumatology opinion was sought. A PET-CT did not show vasculitis. The patient was discharged when pyrexia improved however his inflammatory markers remained consistently high (CRP of 82mg/L and ESR 106mm/hr). One-month post-discharge, the patients GP contacted rheumatology concerned about ongoing headache and scalp tenderness. Prednisolone 40mg od was commenced and a TAB was arranged which reported features consistent with GCA. Both patients responded well to steroids. Conclusion As demonstrated, patients with biopsy proven GCA do not always exhibit vasculitis on imaging. A negative PET-CT did not reliably exclude GCA in our patients suggesting its use as a diagnostic investigation for GCA needs to be approached with caution. A TAB should also be performed when there is ongoing suspicion of GCA. Disclosures S. Khalid None. J. Maxwell Honoraria; BMS, Pfizer, Lilly, Abbvie. Other; Talks, BMS, Pfizer, Lilly, Abbvie.

scholarly journals FRI0201 OPHTHALMOLOGICAL FEATURES AT DIAGNOSIS AND IN RECURRENCES IN PATIENTS WITH GIANT CELL ARTERITIS CONFIRMED BY BIOPSY IN A TERTIARY HOSPITAL

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 684.2-684
Author(s):  
A. De Diego Sola ◽  
N. Alcorta Lorenzo ◽  
J. A. Valero Jaimes ◽  
C. A. Egüés Dubuc ◽  
O. Maiz-Alonso ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Optic Neuropathy ◽  
Central Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Lower Percentage ◽  
Ischemic Optic Neuropathy ◽  
Temporal Artery Biopsy ◽  
Visual Symptoms ◽  
Cilioretinal Artery Occlusion

Background:Giant cell arteritis (GCA) presents with visual symptoms in approximately 37%1of patients at diagnosis. Patients with visual symptoms present, according to some series, lower levels of C-reactive protein (CRP) at diagnosis and associate less headache, fever, cranial nodules or polymyalgia (PM).Relapses despite glucocorticoid treatment (GC) are frequent (40-60%). Visual symptoms are very rare in relapses (0% in several series). Since most series focus on northern European populations, it is interesting to study the characteristics in our population.Objectives:To describe visual manifestations in both diagnosis and recurrence of stroke, only in patients diagnosed with temporal artery biopsy. To analyze demographic, clinical and analytical features in patients with and without visual symptoms.Methods:We retrospectively reviewed the medical records of patients with positive biopsy for GCA at our center from January 2000 through December 2018. Relapse was defined as the appearance of clinical symptoms in a previously asymptomatic patient requiring a dose increase or restart of GCS. Patients with no response to GCS were not included. Qualitative variables are shown with absolute value and percentage and quantitative variables with mean and standard deviation (SD). Kruskal Wallis, Fisher test and Mann-Whitney U test were used for bivariate analysis.Results:52 patients were found: 39 women (73.6%), with an average age at diagnosis of 77.6 years (SD 6.3). At diagnosis, 28 presented visual symptoms (53.84%): oculomotor paralysis 5 (9.61%), amaurosis fugax 8 (15.38%), blindness 6 (11.54%), decreased visual acuity 8 (28.57%) and other visual symptoms 1 (1.92%). Eleven had monocular symptoms (38%) and 9 binocular (32%), in 10 patients this data was not collected. The symptoms were permanent in 11 (39.2%) despite GC. Type of visual impairment was: Anterior Ischemic Optic Neuropathy (AION) (12, 42.86%), impairment of cranial pairs (5, 17.86%), central retinal artery occlusion (1, 3.57%), cilioretinal artery occlusion (1, 3.57%) and Posterior Ischemic Optic Neuropathy (1, 3.57%).Of the 52 patients, 17 (32.69%) presented a minimum of one recurrence and 3 (5.77%) presented 2 or more. No patient relapsed with visual clinic.There were no differences among patients with and without visual symptoms in the variables studied (table 1). However, patients with visual symptoms at diagnosis had numerically less PM and cranial nodules.Table 1.Demographic, clinical and analytical differences between patients with/without visual symptomsVariablesWith visual symptoms (28)Without visual symptoms (24)ESR >5017 (60.7%)15 (62.5%)ESR <504 (14.3%)2 (8.33%)CRP>1017 (60.7%)13 (54.16%)CRP <103 (10.7%)2 (8.33%)Cephalea24 (85,7%)23 (95,8%)Polymyalgia9 (32,1%)12 (50%)Constitutional syndrome9 (32,1%)7 (29,1%)Jaw claudication17 (60.7%)12 (50%)Cranial nodules4 (14,3%)8 (33,3%)Sex: Man/Woman9/195/19Previous PM diagnosis4 (14,3%)3 (12,5%)Conclusion:In our series, 53% of the patients presented visual symptoms at diagnosis, a number higher than that described in the literature. It is important to remember that only patients with biopsy-confirmed ACG were included. The most frequent manifestations were AION followed by oculomotor paralysis. A numerically lower percentage of PM and cranial nodules was observed at diagnosis in patients with visual symptoms compared to patients without them, as seen in some series. The absence of visual clinic in recurrences coincides with that reported in the available literature.References:[1]Alba M et al. Relapses in Patients With Giant Cell Arteritis Prevalence, Characteristics, and Associated Clinical Findings in a Longitudinally Followed Cohort of 106 Patients. Medicine. 2014;93: 194–201.Disclosure of Interests:None declared

scholarly journals OP0150 WHAT IS THE ROLE OF TEMPORAL ARTERY BIOPSY IN GIANT CELL ARTERITIS FAST-TRACK PATHWAYS WHEN TEMPORAL ARTERY ULTRASOUND IS NEGATIVE?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 95.3-95
Author(s):  
A. Sachdev ◽  
S. Dubey ◽  
C. Tiivas ◽  
M. George ◽  
P. Mehta
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Sensitivity And Specificity ◽  
Fast Track ◽  
Temporal Artery ◽  
Clinical Findings ◽  
Specimen Size ◽  
Temporal Artery Biopsy ◽  
Duration Of Treatment ◽  
Relevant Role

Background:A number of centres are now running fast track pathways for diagnosis and management of Giant cell arteritis with ultrasound as the first port of call for diagnosis1. Temporal artery biopsies (TABs) have become the second line of investigation, and it is unclear how useful TAB is in this setting.Objectives:This study looked at accuracy of Temporal artery biopsy (TAB) in patients with suspected Giant Cell arteritis (GCA) with negative/inconclusive ultrasound (U/S) and how duration of treatment on steroids prior to these investigations and arterial specimen size affected it.Methods:Prospective study of all patients with suspected GCA referred for TAB when U/S was negative or inconclusive, as part of the local fast-track pathway (Coventry). Database included clinical findings, serological work up, U/S and TAB results and treatment. Sensitivity and specificity of U/S and TAB was calculated and compared based on duration of treatment with steroids.Results:One hundred and nine patients were referred for TAB via Coventry fast-track-pathway. The sensitivity of U/S in this cohort of patients was 9.08% and specificity was 93.33%. After 3 days of steroid this was 0% and 100% respectively. For TAB when done within 10 days of starting steroids, this was 65% and 87.5% respectively. After 20 days of steroids this was 0 % and 100%. The sensitivity and specificity was 20% and 85% when arterial specimen size was 11-15mm and 47% and 100% when specimen size was 16 mm or more. Sensitivity and specificity of U/S of 644 suspected GCA patients was 48% and 98%.Conclusion:Our study demonstrates that TAB plays a relevant role in GCA fast-track-pathways, when U/S is negative/inconclusive. TAB was more sensitive than U/S in this cohort of patients, but overall sensitivity of U/S was higher when calculated for all patients suspected with GCA. Both remain useful tests if performed early. TAB specimen size should ideally be 16mm or more and done within 10 days of starting steroids.References:[1]Jonathan Pinnell, Carl Tiivas, Kaushik Chaudhuri, Purnima Mehta, Shirish Dubey, O38 The diagnostic performance of ultrasound Doppler in a fast-track pathway for giant cell arteritis,Rheumatology, Volume 58, Issue Supplement_3, April 2019, kez105.036,https://doi.org/10.1093/rheumatology/kez105.036Disclosure of Interests:None declared

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