scholarly journals Giant Cell Arteritis Presenting as Cholestatic Hepatitis

2021 ◽  
Vol 2021 ◽  
pp. 1-2
Author(s):  
Gerald Riordan ◽  
John Riordan

Background. Severely deranged liver function tests (LFTs) are an atypical presentation of giant cell arteritis (GCA). Atypical presentations of GCA may result in missed or delayed diagnosis. This increases the risk of visual loss, the most feared outcome of GCA. Our patient presented with significant cholestatic derangement of his LFTs with a peak alkaline phosphatase level (ALP) of 3091 IU/L, which is the highest published level for patients with GCA. Case Presentation. Our patient was investigated for abnormal LFTs associated with sinus pain, fevers, and a dry cough. Bilateral temporal artery biopsies confirmed GCA. His symptoms and LFTs improved with corticosteroids. Conclusion. This is an unusual presentation of GCA and highlights the need to consider GCA in patients with unexplained cholestatic LFT abnormalities.

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.


2019 ◽  
Vol 80 (8) ◽  
pp. 448-455
Author(s):  
Mehdi Raza ◽  
Yasser El Maideny ◽  
Nadia Bokhari

Giant cell arteritis has been widely studied throughout the world. Involvement of cranial vessels can lead to visual loss and strokes. This review primarily focusses on the presentation, diagnosis and treatment. The last 10 years have brought dramatic improvements in the imaging and medical therapies for this condition. After the American College of Rheumatology suggested criteria for the diagnosis of giant cell arteritis, many studies have been performed to find alternatives to a temporal artery biopsy. There is growing evidence that a biopsy may not be needed when one can make a convincing clinical and radiological diagnosis. Although glucocorticoids are the mainstay of treatment and their role has not changed, various biological and non-biological therapies are being used to reduce relapses and prolong remission of symptoms.


2008 ◽  
Vol 18 (2) ◽  
pp. 91-101 ◽  
Author(s):  
H A Bird ◽  
Helen Mac Iver

Polymyalgia rheumatica and giant cell arteritis are closely related conditions, considered by many to represent opposite poles of a single disease spectrum. They can occur together or separately.Polymyalgia rheumatica is characterized by pain and morning stiffness in the shoulder girdle and sometimes the pelvic girdle. The symptoms are felt to be related to synovitis of proximal joints and extra-articular synovial structures. Giant cell arteritis displays a frank vasculitis affecting the regions supplied by the temporal artery to give visual loss and scalp tenderness but is increasingly recognized to also affect the aorta and its extra-cranial branches. For this reason the term ‘giant cell arteritis’, which is descriptive of the pathology, is used instead of the alternative term ‘temporal arteritis’, which gives a misleading impression of localization but which was the term used in previous reviews for this journal, the most recent in 2003.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1212.1-1212
Author(s):  
V. Yang ◽  
C. Mcmaster ◽  
C. Owen ◽  
J. Leung ◽  
R. Buchanan ◽  
...  

Background:Giant cell arteritis (GCA), if left untreated, confers the threat of serious cranial ischaemic complications including permanent visual loss. Although achieving a prompt and accurate diagnosis remains challenging, early diagnosis is viewed as being paramount in preventing significant morbidity.1 This raises the question of whether GCA patients are at greater risk of developing visual sequelae if there is a longer window between symptom onset and presentation.Objectives:To compare the frequency of lasting visual loss in patients diagnosed with GCA undergoing temporal artery biopsy (TAB) within three months and after three months of symptom onset.Methods:Patients who underwent TAB from January 2011 to November 2020 were identified from the pathology database of an Australian rheumatology referral centre. The diagnosis of GCA was established for each patient based on either positive TAB or, in the setting of negative TAB, clinical diagnosis by a rheumatologist. Baseline demographics, symptoms and major confounders – including age, sex, history of polymyalgia rheumatica or inflammatory arthritis, headache, jaw pain, fatigue, temporal artery tenderness or diminished pulse, and number of 1990 American College of Rheumatology (ACR) classification criteria for GCA2 fulfilled – were manually extracted from electronic medical records, as was the duration between onset of GCA symptoms and TAB, and the presence of visual loss before and after TAB. Logistic regression log-likelihood tests were used to examine the two cohorts presenting before and after three months.Results:There were 167 patients who underwent TAB during the study period with accessible clinical information. Of these, 31 (19%) had a delayed presentation of greater than three months from symptom onset. There were no statistical differences in patient demographics between the two groups (Table 1). No patients with delayed presentation experienced lasting, objective visual loss. In contrast, there were three cases in the cohort of patients who presented more promptly; these included two patients who developed permanent unilateral blindness, and one who experienced unilateral vision loss with some improvement at three months of follow-up.Table 1.Patient characteristics by time from symptom onset to TAB.Presentation <3 monthsPresentation ≥3 monthsp-valueAge (years)73.45±10.0669.84±10.750.080Female92 (67.65%)20 (64.52%)0.738History of polymyalgia rheumatica23 (16.91%)4 (12.90%)0.586History of inflammatory arthritis6 (4.41%)2 (6.45%)0.633Headache110 (80.88%)23 (74.19%)0.406Jaw pain37 (27.21%)5 (16.13%)0.206Fatigue28 (20.59%)6 (19.35%)0.878Temporal artery tenderness or diminished pulse46 (33.82%)11 (35.48%)0.860ACR classification criteria2.83±0.992.58±0.890.199Conclusion:GCA patients with a lengthier course of symptoms before diagnosis did not experience any enduring visual loss. This may reflect a pattern of more aggressive disease leading to earlier presentation, but further study should explore whether longer symptom duration before diagnosis necessitates a higher degree of clinical concern.References:[1]Font C, Cid MC, Coll-Vinent B, López-Soto A, Grau JM. Clinical features in patients with permanent visual loss due to biopsy-proven giant cell arteritis. Br J Rheumatol. 1997 Feb;36(2):251-4. doi: 10.1093/rheumatology/36.2.251. PMID: 9133940.[2]Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990 Aug;33(8):1122-8. doi: 10.1002/art.1780330810. PMID: 2202311.Disclosure of Interests:None declared


Author(s):  
Marília A. Dagostin ◽  
Rosa M.R. Pereira

Giant cell arteritis (GCA) is the most common vasculitis in adults, with the incidence increasing with the advancing age. The aorta and its branches, especially the carotid extracranial branches, are the classic targets of inflammation in GCA. Visual loss, upper limb ischemia, and stroke are complications described. Suspicion of GCA is a medical emergency, and patients need to be quickly diagnosed/treated to prevent irreversible damage. Headache is the most common symptom, and a new-onset headache in older adults should always raise the suspicion of GCA. Patients may also present with scalp tenderness or tongue/jaw pain. GCA is often found to be the cause of an obscure-origin fever in older patients. A positive temporal artery biopsy is considered the gold standard for the diagnosis, but imaging techniques enable the assessment of cranial and extracranial arteries and the aorta. Ultrasound of temporal arteries is recommended and noncompressible “halo” sign is the typical finding. PET, MRI, or CT may be useful for the detection of the disease in the aorta and other vessels. The treatment must be started promptly with prednisone 1 mg/kg/day. When visual symptoms/unilateral visual loss is present, methylprednisolone pulse is recommended. Methotrexate, leflunomide and tocilizumab may be effective and well-tolerated glucocorticoid-sparing agents in GCA. Cardiovascular diseases are the leading causes of death in patients.


2019 ◽  
Vol 12 ◽  
pp. 117954761985769
Author(s):  
Logan Christopher DeBord ◽  
Ilene Chiu ◽  
Nelson Eddie Liou

Background: Lingual necrosis is a rare complication of giant cell arteritis (GCA). Methods: A 77-year-old woman presented for treatment of a painful and discolored tongue, odynophagia, and dehydration refractory to antimicrobials over 2 weeks. An extensive, well-demarcated necrotic area was visualized on the anterior tongue upon admission. Leukocytosis, thrombocytosis, and elevated erythrocyte sedimentation rate were present. Computed tomography angiogram of the head and neck revealed an undulated-beaded appearance of the distal internal carotid arteries and vertebral arteries bilaterally. Results: High-dose intravenous steroids were initiated for suspected vasculitis. Temporal artery biopsy confirmed the diagnosis of GCA. The patient’s condition improved and the anterior tongue was well healed at 1 month follow-up. Conclusions: An atypical presentation of GCA (eg, isolated lingual necrosis) risks a delay in diagnosis and increased morbidity. Any patient above the age of 50 years presenting with tongue necrosis, in the absence of known cause, should undergo expedited workup for GCA.


2008 ◽  
Vol 68 (1) ◽  
pp. 84-88 ◽  
Author(s):  
D Chatelain ◽  
P Duhaut ◽  
J Schmidt ◽  
R Loire ◽  
S Bosshard ◽  
...  

Background:Permanent visual loss (PVL) is the most feared complication of giant cell arteritis (GCA), and its risk factors are still unclear.Objectives:The aim of our study was to assess the pathological features predictive of PVL on temporal artery biopsy (TAB) specimens in patients with GCA.Methods:The slides of 391 TAB specimens from patients with GCA were reviewed by two pathologists without clinical information.Results:A total of 29 patients (26 females and 3 males, mean age 78.3 years) presented with unilateral PVL at the onset of the disease, and 362 patients (258 females, 104 males, mean age 74.7 years), did not. The pathological features strongly predictive for PVL were the presence (p = 0.003), number (p = 0.001) and aggregates of giant cells (p = 0.001), presence of plasmocytes (p = 0.002), thickened intima (p = 0.007), neoangiogenesis (p = 0.001) and degree of arterial occlusion (p = 0.006). Presence of neutrophils, eosinophils, parietal necrosis, calcification in the arterial wall and disruption of the internal elastic membrane were similar in both groups. Total obstruction of the arterial lumen by a thrombus, intensity of the inflammatory cells infiltration and inflammation of small vessels, nerves and veins surrounding the temporal artery were not associated with blindness. In multivariate analysis, only giant cells remained significantly associated with PVL.Conclusion:Giant cells are strongly associated with PVL, with a significant gradient between great risk and large number of giant cells. However, PVL was neither associated with the intensity of the inflammatory infiltrate, nor with the presence of arterial thrombosis.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 95.3-95
Author(s):  
A. Sachdev ◽  
S. Dubey ◽  
C. Tiivas ◽  
M. George ◽  
P. Mehta

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


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 682.1-682
Author(s):  
S. Chrysidis ◽  
U. Møller Døhn ◽  
L. Terslev ◽  
U. Fredberg ◽  
T. Lorenzen ◽  
...  

Background:Giant Cell Arteritis (GCA) is one of the most common systemic vasculitis. Temporal artery biopsy (TAB) has been the standard test to confirm the diagnosis of GCA. However, TAB has a lower sensitivity than clinical diagnosis and up to 44% of biopsy-negative patients are clinically diagnosed as having GCA.In a recent meta-analysis of the diagnostic performance of ultrasound (US) in GCA the sensitivity was 77 % (1). The included studies were performed by expert groups in single centres. In the to date only multicentre study (TABUL) investigating the diagnostic accuracy of US compared to clinical diagnosis after 6 months the sensitivity was lower (54%) (2)Objectives:To evaluate the diagnostic accuracy of vascular US compared to TAB in a multicentre study.Methods:In three Danish centres patients suspected for GCA were included during a period of two years. At baseline, clinical and laboratory data were collected and vascular US of temporal, facial, common carotid and axillary artery were performed. The US examinations were performed with high frequency transducers (15-18 MHZ) and followed by a TAB. All ultrasongraphers had participated in the same standardized US educational program and were blinded to clinical and laboratory data. An external expert blinded to clinical and laboratory data evaluated all images and made the final US diagnosis.A positive sign for vasculitis in cranial arteries was defined as a hypoechoic intima media complex (IMC) thickening (halo sign) and a positive compression sign. A homogeneous IMC increased thickness in axillary artery of ≥1mm and in common carotid artery ≥1.5mm was defined as vasculitis.The consultant rheumatologist’s diagnosis at 6 months after initial presentation was considered as the reference standard for the diagnosis of GCA.Results:During the recruitment period, 112 patients were included, 59% females, mean (SD) age 72.4(7.9) years, among which 91(81.3%) fulfilled the ACR 1990 classification criteria for GCA. 92% of the patients reported a newly emerged localized headache, while 49 (43.8%) experienced polymyalgia rheumatic symptoms.TAB was positive in 46(41.1%) and inconclusive in 6 patients, who were excluded from the analysis. Mean (SD) duration of glucocorticoid therapy prior to US and TAB was 0.91(1.55) and 4.02(2.61) days, respectively. In 62 patients, the final diagnosis was GCA.In all patients with a positive TAB, the US of the temporal artery was also positive for GCA. Of 19 cases with positive US and negative TAB, 12 were clinically diagnosed with GCA of whom 6 had isolated large vessel involvement on US. Among 41 patients with both negative US and TAB, 4 were clinically diagnosed with GCA (Box 1)US had a sensitivity of 93% and specificity of 84% for the diagnosis of GCA, while the sensitivity for TAB was lower (74%) with a specificity of 100%. For the diagnosis of GCA, US had a PPV of 89.2 % and a NPV of 90.2%, while for TAB the PPV was 100% and the NPV 73.3%.Conclusion:US evaluation of the temporal, facial and selected supraaortic arteries performed by trained ultrasonographers can replace biopsy in the diagnosis of GCA.Box.1References:[1]Duftner C, Dejaco C, et al. Imaging in diagnosis, outcome prediction and monitoring of large vessel vasculitis: a systematic literature review and metaanalysis informing the EULAR recommendations. RMD Open 2018;4:e000612.[2]Luqmani R et al. The Role of Ultrasound Compared to Biopsy of Temporal Arteries in the Diagnosis and Treatment of Giant Cell Arteritis (TABUL): a diagnostic accuracy and cost-effectiveness study. Health Technol Assess 2016;20:1_238.Disclosure of Interests:stavros chrysidis: None declared, Uffe Møller Døhn: None declared, Lene Terslev Speakers bureau: LT declares speakers fees from Roche, MSD, BMS, Pfizer, AbbVie, Novartis, and Janssen., Ulrich Fredberg: None declared, Tove Lorenzen: None declared, Robin Christensen: None declared, Per Søndergaard: None declared, Jakob Matthisson: None declared, Knud Larsen: None declared, Andreas Diamandopoulos: None declared


2011 ◽  
Vol 121 (S5) ◽  
pp. S264-S264
Author(s):  
Stephen V. Tornabene ◽  
Raymond Hilsinger ◽  
Raul M. Cruz

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