scholarly journals P040 An unusual case of giant cell arteritis

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Jessica Ellis ◽  
Keziah Austin ◽  
Sarah Emerson

Abstract Background/Aims  A 49-year-old female of Nepalese heritage was referred with right-sided headache, scalp tenderness, and a painful swelling overlying the right temple. She denied any visual or claudicant symptoms but felt systemically unwell with a fever. There were no symptoms suggestive of an inflammatory arthritis, underlying connective tissue disease or vasculitis. She was normally fit and well with no past medical history. She did not take any regular medications and denied using over the counter or illicit drugs or recent travel. On review she had a low grade fever. There was a large tender, erythematous swelling overlying the right temple. Bilaterally the temporal arteries were palpable and pulsatile. Peripheral pulses were normal with no bruits. There was no evidence of shingles (HSV) or local infection. Full systemic examination revealed no other abnormalities. Laboratory tests showed: PV 2.56, CRP 101, total white cell count 14.38 (eosinophils 0.4), albumin 33, Hb 115. Urine dip was normal. Renal function, liver function and immunoglobulins were normal. ANCA was negative. Hypoechogenicity surrounding the right frontal branch of the right temporal artery was seen on ultrasound. There were no discrete masses suggestive of cysts, abscess or tumours. Temporal artery biopsy confirmed the presence of vasculitis; histology demonstrated transmural lymphohistiocytic inflammation, disruption of the elastic lamina and intimal proliferation. Prednisolone was started at 40mg daily. Four weeks after initially presenting she was asymptomatic and her inflammatory markers had normalised. Methods  The case is discussed below. Results  Temporal arteritis, or GCA, is primarily a disease of older adults; with age 50 often used as an inclusion criteria, and is more common in Caucasian populations. Limited reports exist of GCA in younger cohorts, but these are rare. An important differential in younger patients, such as ours, is juvenile temporal arteritis. This rare localised vasculitis affects almost exclusively the temporal artery. It is typically a disease of young males, who present with non-tender temporal swelling. Systemic symptoms are unusual and inflammatory markers are normal. Clinical or laboratory evidence of organ involvement, peripheral eosinophilia or fibrinoid necrosis on histology should prompt consideration of an AAV or PAN. Incidence of GCA increases in correlation with Northern latitude, with highest rates reported in Scandinavian and North American populations. GCA is rare in Asian populations. Higher diagnostic rates in countries where physicians have increased awareness of GCA proposed as an explanation for this difference; however differences in incidence are still observed between Asian and Caucasian populations presenting to the same healthcare providers. Conclusion  GCA is an uncommon diagnosis in younger and non-Caucasian patients. Thorough investigation through ultrasound and biopsy helped increase our diagnostic confidence in this unusual case. Rheumatologists must be alert to atypical presentations in order to deliver prompt and potentially sight-saving treatment. Disclosure  J. Ellis: None. K. Austin: None. S. Emerson: None.

2015 ◽  
Vol 209 (2) ◽  
pp. 338-341 ◽  
Author(s):  
Khoi Le ◽  
Lindsay M. Bools ◽  
Allison B. Lynn ◽  
Thomas V. Clancy ◽  
W. Borden Hooks ◽  
...  

2018 ◽  
Vol 89 (10) ◽  
pp. A17.3-A17
Author(s):  
Mehta Dwij ◽  
Wade Charles

An 80 year old gentleman presented with bilateral, sequential ischaemic optic neuropathy. He initially developed progressive loss of vision in left eye with loss of colour vision and subsequently developed similar symptoms in the right eye with headaches, weight loss, malaise and lethargy. His visual acuity dropped to 6/12 on the right and counting fingers on the left. He had a dense central scotoma in left eye with left-sided RAPD but no other focal neurological deficits.Blood tests revealed an ESR of 107 with an MPO ANCA titre of 19. MRI brain with contrast showed prominent meningeal enhancement and infiltration with ischaemic changes in the brain. CSF analysis revealed WCC of 24 (95% lymphocytes), RCC 22 and protein 0.4 g/L with negative bacterial culture. Temporal artery biopsy was normal.He was treated initially with IV methylprednisolone and 6 cycles of IV cyclophosphamide and subsequently put on methotrexate. His systemic symptoms have resolved completely and his visual acuity continues to gradually improve.MPO-ANCA vasculitis can mimic temporal arteritis and should be considered in patients presenting with an ischaemic optic neuropathy. It is also a treatable cause of meningeal disease.


2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Samir Patel ◽  
Israa Al-Shakarchi ◽  
Dobrina Hull

Abstract Introduction Giant cell arteritis (GCA) represents a common cause of uncertainty for physicians. GCA can present as an indolent or aggressive process with a wide spectrum of symptoms and signs. The key to diagnosis is good clinical acumen and awareness of both common and rare associated conditions. Here we describe a rare and atypical presentation of a patient with GCA and chest pain. Case description An 83-year old male was admitted to the medical take with 3 hours of crushing chest pain associated with diaphoresis and nausea. He had been started on 60mg prednisolone by his GP 3 days preceding this for suspected GCA (scalp tenderness, jaw claudication, polymyalgia). Past medical history included: hypertension, recent strokes (bilateral cerebellar infarcts 3 months prior), previous DVTs and COPD.  Examination revealed no abnormalities. Admission ECGs showed sinus rhythm with fixed T-wave inversion in aVL and V3. Chest radiograph was normal. Bloods tests revealed mildly raised inflammatory markers (CRP, ESR 24) and a dynamic troponin (324 – 1241 – 1431 ng/L). He was diagnosed with a non-ST elevation myocardial infarction (NSTEMI) and was referred to Cardiology for an angiogram. He was started on dual antiplatelets and high dose atorvastatin.  On review by Rheumatology, his recent history of bilateral posterior circulation strokes was investigated further. Multi-level bilateral vertebral artery stenosis was seen on CT angiogram at the regional hyper-acute stroke unit and diagnosed as atheromatous in origin due to: a negative right-sided temporal artery biopsy, no GCA symptoms and modest inflammatory markers (CRP in the 30s and ESR in the 20s).  Due to the onset of clear and acute GCA symptoms a left-sided temporal artery biopsy and PET scan was scheduled. Prednisolone was reduced to 40mg OD due to concern over its atherogenicity during a suspected NSTEMI.  Coronary angiogram went on to show clear arteries and a subsequent cardiac MRI confirmed acute myocarditis. The left temporal artery biopsy revealed chronic inflammation with scattered giant cells.  The patient was diagnosed with: GCA, extra-cranial vasculitis and myocarditis. He was treated with intravenous methylprednisolone, a weaning course of prednisolone and started on methotrexate (15mg weekly).  The patient’s chest pain settled soon after admission and his troponin and inflammatory markers fell with prednisolone.  Discussion GCA seldom presents with myocarditis and can lead to significant morbidity and mortality. This remains an important differential in a patient with clear GCA features and chest pain. This case report adds to the growing body of evidence that myocarditis is a recognised feature of GCA. A literature review revealed 8 articles and a total of 9 patients with GCA who presented with or developed myocarditis. The initial diagnosis of an NSTEMI was reasonable in this case and the most probable cause of the patient’s symptoms, signs and blood results at the time. To this end, starting dual anti-platelets was justified. His prednisolone was reduced to 40mg due to concerns over accelerated atherosclerosis with high dose glucocorticoids in an already high-risk male with smoking history, hypertension and a stroke history. In retrospect, this was an unnecessary step, but arguably reasonable when weighing the risks and benefits of one treatment against a simultaneous acute illness (NSTEMI). Each subsequent investigation helped to diagnose and exclude certain conditions: the angiogram was necessary to exclude an acute coronary syndrome; cardiac MRI then went on to provide evidence of myocarditis; temporal artery biopsy confirmed GCA and a PET scan excluded any significant large vessel involvement. Interestingly, the first right-sided temporal artery biopsy 3 months prior was negative but the second left-sided biopsy was diagnostic. The BSR states that “contralateral biopsy is usually unnecessary”; however, a few studies have found discordant results when carrying out simultaneous bilateral biopsies with an increased diagnostic yield (Boyev et al., 1999; Breuer et al., 2009 and Durling et al., 2014), suggesting bilateral temporal artery biopsies could lead to less treatment delays and more accurate diagnoses. Key learning points Myocarditis is a recognised complication of giant cell arteritis. The key to diagnosis here was a thorough history and an open mind with regards to his background, particularly the bilateral strokes. Investigations initially did not support GCA-associated myocarditis. But the preceding typical GCA symptoms and recent strokes raised the suspicion of a more systemic vasculitis. Working off others’ diagnosis can be misleading. The very recent bilateral posterior circulation strokes and vertebral artery abnormalities were overlooked on admission due to being thoroughly worked up and disregarded as vasculitic by a tertiary stroke centre. Conflicts of interest The authors have declared no conflicts of interest.


2021 ◽  
Vol 14 (1) ◽  
pp. e238514
Author(s):  
Rebecca Ceci Bonello ◽  
Etienne Ceci Bonello ◽  
Christian Vassallo ◽  
Edward Giles Bellia

A 76-year-old woman presented with a 2-hour history of pleuritic chest pain with no other associated symptoms. Blood investigations revealed raised inflammatory markers and an elevated white cell count. On chest radiograph, an airspace shadow indicative of a consolidation was prominent. This was followed by a CT scan of her thorax which showed a spiculated lesion in the right upper lobe, a lesion in the posterior segment of the left lower lobe and mildly enlarged right hilar lymph nodes. She was started on dual antibiotic therapy; however, the patient’s clinical status and inflammatory markers did not improve. A bronchoscopy was performed which excluded malignancy and atypical pathogens. CT-guided biopsy confirmed the presence of cryptogenic organising pneumonia. Prednisolone 50 mg daily was prescribed with quick resolution of symptoms.


2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Alexander G. Goglia ◽  
Michael Makar ◽  
Craig Vanuitert ◽  
Vadim Finkelstein

Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis. While the lungs and kidneys are the major organs affected by MPA, it is known to involve multiple organ systems throughout the body. Temporal artery involvement is a very rare finding in MPA. This report presents a patient whose initial presentation was consistent with giant cell arteritis but was ultimately found to have microscopic polyangiitis. It highlights the importance of considering alternative types of vasculitis in the differential diagnosis for patients with atypical temporal artery biopsy findings.


VASA ◽  
2001 ◽  
Vol 30 (3) ◽  
pp. 222-224 ◽  
Author(s):  
Alec Cikes ◽  
M. Depairon ◽  
R.-M. Jolidon ◽  
P. Wyss ◽  
H.-J. Lang

While blindness is one of the typical clinical presentations of temporal arteritis, tongue necrosis, on the other hand, is an unusual complication of the disease. An 80 year old male patient presenting a sudden massive swelling of the tongue was admitted to the Hospital of Yverdon. The swelling rapidly progressed to a complete necrosis of the tongue within a few days. The clinical presentation, the dramatic evolution of the necrosis, and sudden unilateral blindness despite prompt treatment confirmed our diagnosis of temporal arteritis. However, all the examinations, including biopsy of the right temporal artery, remained non-specific for the disease. Our diagnosis was based on the unusual clinical presentation of the disease.


Vascular ◽  
2013 ◽  
Vol 22 (6) ◽  
pp. 406-410 ◽  
Author(s):  
Sarantos Kaptanis ◽  
Joanne K Perera ◽  
Constantine Halkias ◽  
Nadine Caton ◽  
Lida Alarcon ◽  
...  

This study aimed to clarify whether positive temporal artery biopsies had a greater sample length than negative biopsies in temporal arteritis. It has been suggested that biopsy length should be at least 1 cm to improve diagnostic accuracy. A retrospective review of 149 patients who had 151 temporal artery biopsies was conducted. Twenty biopsies were positive (13.3%), 124 negative (82.1%) and seven samples were insufficient (4.6%). There was no clinically significant difference in the mean biopsy size between positive (0.7 cm) and negative samples (0.65 cm) ( t-test: p = .43 NS). Ninety-four patients fulfilled all three ACR criteria prior to biopsy (62.3%) and four patients (2.6%) changed ACR score from 2 to 3 after biopsy. Treatment should not be delayed in anticipation of the biopsy or withheld in the case of a negative biopsy if the patient’s symptoms improve.


Sign in / Sign up

Export Citation Format

Share Document