Giant Cell Arteritis Presenting Research Articles

The activation pattern of macrophages in giant cell (temporal) arteritis and primary angiitis of the central nervous system

To determine if the pattern of macrophage activation reflects differences in the pathogenesis and clinical presentation of giant cell arteritis and primary angiitis of the central nervous system, specimens of 10 patients with giant cell arteritis and five with primary angiitis of the central nervous system were immunohistochemically studied and the expression of the macrophage activation markers 27E10, MRP14, MRP8 and 25F9 was determined in the vasculitic infiltrates. Thus, a partly different expression pattern of macrophage activation markers in giant cell arteritis and primary angiitis of the central nervous system was observed. The group comparison revealed that giant cell arteritis cases had significantly higher numbers of acute activated MRP14-positive macrophages, whereas primary angiitis of the central nervous system is characterized by a tendency toward more MRP8-positive intermediate/late activated macrophages. Furthermore, in giant cell arteritis comparably fewer CD8-positive lymphocytes were observed. These observations suggest, that despite their histopathological similarities, giant cell arteritis and primary angiitis of the central nervous system appear to represent either distinct entities within the spectrum of granulomatous vasculitides or different stages of similar disease processes. Their discrete clinical presentation is reflected by different activation patterns of macrophages, which may characterize giant cell arteritis as a more acute process and primary angiitis of the central nervous system as a more advanced inflammatory process.

ORBITAL APEX SYNDROME AS THE PRESENTING FEATURE OF LARGE VESSEL VASCULITIS

Orbital apex syndromes are commonly due to metastases and orbital pseudotumour. We present a patient in whom an orbital apex syndrome was the initial manifestation of a large vessel vasculitis,...

Giant Cell Arteritis Presenting as Depressed Mood and Headache in an Elderly Patient

Giant cell arteritis (GCA) is an autoimmune disorder characterized by the inflammation of medium to large vessels. Although classic descriptions have centered on features such as vision loss, headaches, fevers, and jaw claudication, there is increasing recognition of more atypical manifestations. We report a case of GCA in a 77-year-old male patient initially presenting with depressed mood and headache. His psychiatric symptoms promptly responded to treatment with corticosteroids without the use of antidepressants. This case adds to the literature that GCA may initially present with affective or psychotic symptoms and highlights the need for clinicians to consider GCA in the differential diagnosis for new-onset psychiatric disorders in the elderly.

Giant Cell Arteritis Presenting as Critical Lower Limb Ischemia

A 62-year-old woman presented with ischemic pain of her lower limb on 2 occasions, without upper limb or cranial vessel involvement. She was treated with open bypass of her left lower limb when she presented with critical limb ischemia during her second admission. Although bilateral temporal artery biopsies were negative, superficial femoral artery biopsy at the time of surgery confirmed the diagnosis of giant cell arteritis. Corticosteroid therapy was promptly commenced.

AB0449 Isolated aortitis as an atypical presentation of giant cell arteritis

BackgroundThe most common causes of aortitis are the large-vessel vasculitis, although it also is associated with several other rheumatologic diseases. Giant cell arteritis (GCA) is the most common form of...

AB0773 Delays in recognition of giant cell arteritis presenting as fever of unknown origin: Evaluation of a hospital-based population

BackgroundGiant cell arteritis (GCA) represents the most common primary vasculitis of the elderly, that usually involves large and medium sized arteries. The wide spectrum of clinical manifestations can extensively vary,...

Initial presentation of giant cell arteritis with ischemic optic neuropathy without headache: Report of four cases

Initial presentation of giant cell arteritis with ischemic optic neuropathy without headache: Report of four cases

Bitemporal scalp, lip and tongue necrosis in giant cell arteritis: A rare presentation

A 74-year-old man was presented with fever, bilateral throbbing temporoparietal headache, jaw claudication, and bilateral loss of vision. On examination, he had bilateral scalp necrosis with impending necrosis of lip and tongue. Temporal artery biopsy was done, and it was compatible with active temporal arteritis. This is one of the rare presentations of giant cell arteritis where there is simultaneous necrosis of scalp, lip, and tongue, and to the best of our knowledge, it is the first case reported from India.

Giant Cell Arteritis Presenting as Choroidal Infarction

Background. Giant cell arteritis (GCA) is a systemic granulomatous vasculitis that affects large- and medium-sized arteries of the head and neck. Ocular manifestations of GCA usually are anterior ischaemic optic neuropathy (AION) or retinal vessel occlusion. Case Report. We report an interesting case of a 70-year-old man who presented with sudden vision loss and choroidal infarction in his left eye. Thorough clinical and paraclinical evaluation revealed an underlying GCA, the treatment of which prevented further vision loss and systemic complications. Conclusion. This is an unusual presentation of choroidal infarction associated with CGA and emphasizes the need of thorough systemic evaluation in patients with choroidal infarction.

Giant cell arteritis presenting as tongue necrosis

Giant cell arteritis (GCA) is a large and medium-vessel systemic vasculitis affecting predominantly the internal and external carotid arteries with particular affinity for its extra-cranial branches (especially, the superficial temporal artery). The most frequent complication of this disorder is visual loss. We report the case of a patient who suffered tongue necrosis (a rare complication) secondary to giant cell arteritis. Case Report: A 71-year-old female who after having her dentures refitted developed severe pain, edema of the tongue, odynophagia, dysphagia and resultant anorexia. She was admitted with the diagnosis of glossitis and initially treated with antifungals and later antiviral medications without improvement. She subsequently developed tongue ulcerations, severe pain in temporomandibular joint (TMJ) with right sided headache. Temporal arteritis was suspected at this point, re-examination revealed palpable and tender temporal arteries (R>L). Tongue examination demonstrated dark greyish plaques in the dorsum with areas of ulceration without palatal involvement. An elevated ESR and the right sided temporal artery biopsy that followed demonstrating chronic inflammation with the presence of giant cells confirmed the diagnosis of GCA. Treatment with oral prednisone (1 mg/kg/day) produced a rapid improvement of her symptoms and ulcers with complete resolution at three months. Conclusion: The presence of glossitis in GCA represents a severity marker of this disease and is associated with a higher risk of vision loss and mortality. As such a high level of suspicion is necessary for the early recognition of this rare presentation and prompt institution of treatment to prevent the aforementioned complications.

Third cranial nerve palsy (ptosis, diplopia) accompanied by orbital swelling: case report of unusual clinical presentation of giant cell arteritis associated with polymyalgia rheumatica

IntroductionGiant cell arteritis (GCA) is the most common systemic vasculitis in older individuals, characterized by granulomatosus inflammation of the wall of large and medium-sized arteries. The wide spectrum of arterial sites involved leads to ischemia of different organs resulting in a wide range of clinical signs and symptoms. Temporal artery is commonly involved (temporal arteritis). Unusual patterns of presentation, such as extraocular motility disorders and orbital swelling, may be early and transient manifestations of GCA and precede the permanent visual loss due to ischemic optic neuropathy.Case reportWe describe a patient with uncommon manifestations of GCA consisting of transient recurrent diplopia, ptosis, orbital swelling together with more typical clinical features of the disease such as musculoskeletal manifestations (polymyalgia rheumatica) and facial pain: all signs and symptoms promptly resolved under corticosteroid therapy without relapse.Conclusions A high level of suspicion of GCA in individuals over the age of 50 years is needed to prevent the development of severe complications. Clinicians should be aware of uncommon manifestations of the disease such as head–neck swelling and ophthalmoplegia: management guidelines have stated that prompt administration of adequate dose of corticosteroids as soon as ocular manifestations of GCA are noted may almost totally prevent blindness.

A Case of Giant Cell Arteritis Presenting as Thunderclap Headache

A Case of Giant Cell Arteritis Presenting as Thunderclap Headache

GIANT CELL ARTERITIS IN TRIGEMINAL AUTONOMIC CEPHALGIA

Clinical assessment is the cornerstone of diagnosis in headache but can prove misleading when more than one type of headache coexists. We describe two patients with phenotypically classical cluster headache...

Giant cell arteritis presenting with a lateral medullary stroke in a patient with multiple atherosclerotic risk factors

Giant cell arteritis presenting with a lateral medullary stroke in a patient with multiple atherosclerotic risk factors

GCA diagnosis controversies

AbstractPurpose To report on different clinical presentations of giant cell arteritis (GCA, temporal arteritis) and diagnostic and therapeutic approach. GCA is the most common primary vasculitis of adulthood. Ocular symptoms present in 30‐75% of patients and include visual loss, amaurosis fugax, ophthalmoplegia, diplopia and ocular pain. Approximately 15‐50 % of patients suffer from permanent visual loss. Temporal artery biopsy and corticosteroid treatment is essential for diagnosis and preservation of vision.Methods Review and retrospective study of 50 eyes (36 patients) with giant cell arteritis treated at Eye Hospital Ljubljana. There were 67% female and 33% male patients with average age of 79 years, range 62‐92 years, with typical clinical picture and/or positive biopsy (78%, 18/23 performed).Results Loss of vision was unilateral (65%) or bilateral (35%). The most common ischaemic lesions were anterior ischaemic optic neuropathy (AION; 16 unilateral, 4 bilateral) and posterior ischaemic optic neuropathy (PION; 5 unilateral, 5 bilateral). Other lesions included central retinal artery occlusion and internuclear ophthalmoplegia. More than half of patients (53%) had visual acuity of counting fingers or less in affected eye. In AION various peripheral defects almost universally showed respect for horizontal meridian. Nasal and inferior halves of visual fields were most often affected. PION most often presented with scotomas with or without peripheral defect.Conclusion Severe loss of vision is present in affected eye in most patients. Bilateral loss of vision can occur if the diagnosis is not established early and corticosteroids given. Very few affected eyes recover to small extent. It is essential to recognise this disease even in absence of typical signs and symptoms. In elderly, all the patients to report amaurosis fugax, sudden loss of vision or diplopia should be suspected to have temporal arteritis.

Aortite inflammatoire au cours de la maladie de Horton

A sub-clinical inflammatory aortitis is very frequent in patients with giant cell arteritis, and can be the only localization of the disease. In most patients, this aortitis is asymptomatic and is of no consequence on the patient's survival. The relative risk of developing an aortic dissection or aneurysm is 17.3. Evolution towards an aneurysm or an aortic dissection is unpredictable and rare; and seems independent of the disease activity and the associated vascular risk factors. Isolated aortitis treatment is not consensual, but often similar to the treatment of giant cell arteritis and adapted to clinical and biological markers of disease activity. Screening for sub-clinical aortitis with FDG-PET should not be prescribed in patients with typical presentation of giant cell arteritis. A systematic screening of aortic complications in giant cell arteritis patients could be done with a chest X-ray and an abdominal ultrasound possibly completed with an aortic CT-scan at time of diagnosis, in order to look for aneurysms with possible surgical indication.

Dry cough is a frequent manifestation of giant cell arteritis

Physicians need to be familiar with the typical manifestations of giant cell arteritis. However, the challenge lies in recognizing atypical cases that lack the more specific manifestations or reflect vasculitis in less frequently involved territories. Among atypical clinical manifestations, dry cough has been reported in recent years. The literature contains sporadic reports mainly single case report. The objective of this study was to determine the frequency of dry cough in patients with giant cell arteritis. Clinical data were collected from 88 patients with giant cell arteritis. Relationships between dry cough and other clinical manifestations or biological data were analyzed. Dry cough of recent appearance was found at initial presentation of giant cell arteritis in 12 patients (13.6%). In 2 cases, dry cough was isolated. The 2 patients sought attention because of chronic dry cough associated with inflammation of unknown origin. In 10 cases, dry cough was associated with typical clinical manifestations of giant cell arteritis. A correlation was found between inflammatory biomarkers and presence of dry cough. The mean CRP was 153.8 mg/l (SD 85.1) in patients with dry cough and 94 mg/l (SD 72.2) in patients without dry cough (p = 0.0131). We conclude that the diagnosis of giant cell arteritis should always be considered in an elderly patient with an unexplained elevation of inflammatory markers and chronic dry cough. Dry cough in giant cell arteritis was not correlated with other clinical manifestations of this vasculitis, including pulmonary manifestations, but was correlated with inflammatory biomarkers.

A Case of Atypical Giant Cell Arteritis Presenting as a Fever of Unknown Origin

Giant cell arteritis (GCA) is a systemic vasculitis predominantly found in individuals of Northern European ancestry over 50 years of age. Typically it presents with new-onset persistent headache, claudication of jaws, and existence of an abnormal temporal artery. However, the diagnosis of GCA and the assessment of its activity remain challenging, especially in patients presenting with a variety of non-specific symptoms and laboratory tests. In those cases, F-fluorodeoxyglucose positron emission tomography (F-FDG-PET) is useful for the diagnosis of GCA. Recently, the number of foreign patients who visit domestic hospitals for medical care is increasing in Korea. Here-in, we report a Russian patient who was admitted to our hospital with fever of unknown origin (FUO). FUO study was performed to search for infection or malignancy and GCA was suspected by F-FDG-PET. The patient was eventually diagnosed with GCA by random temporal artery biopsy.

Large-Vessel Giant Cell Arteritis Presenting as Back Pain with Vertebral Body Infarction in a 73-year-old Caucasian Woman

To the Editor: Giant cell arteritis (GCA) is a chronic vasculitis that almost exclusively afflicts individuals age 50 years and older, with a prevalence of 1 in 500 individuals1,2. Early diagnosis is always the goal, so symptoms such as headache, scalp pain, jaw claudication, and amaurosis fugax should alert clinicians of the possibility of GCA and lead to early confirmatory biopsy3. GCA is also known to occasionally present in an unusual manner, which can cause significant confusion and ultimately delay the diagnosis, as was the case with our patient. A 73-year-old Caucasian woman with a history of lower back pain secondary to degenerative disc disease presented to the emergency department with a one-week history of incapacitating back and neck pain accompanied by stiffness. She described the pain as sharp, beginning in her mid-back, and then slowly progressing to both her lower cervical area and her upper sacrum. She could not find a position that was comfortable, and any movement made her symptoms much worse. She also complained of “shooting” pains in her forearms bilaterally, with transient numbness of her thumbs, as well as increasing stiffness in both shoulders. She specifically denied any jaw claudication, headaches, or visual disturbances. She had had a one-year history of lumbar degeneration for which she underwent a lumbar fusion of the L5 to S1 vertebrae 4 months prior to presentation. The medical history included known C4 through C6 spinal stenosis, a herpes zoster outbreak earlier that year, gastroesophageal reflux disease, mild interstitial pulmonary … Address correspondence to Dr. Maniaci; E-mail: maniaci.michael{at}mayo.edu

Giant cell arteritis in a neuro-ophthalmology clinic in Saskatoon, 1998-2003.

We present a retrospective review of all biopsy-positive cases of giant cell arteritis (GCA) presenting to a neuro-ophthalmology practice in Saskatoon, Saskatchewan. Records of 141 consecutive patients who underwent temporal artery biopsy at the Saskatoon Eye Centre from July 1998 through June 2003 were reviewed. Patients that were biopsy-positive for GCA were studied and an estimated regional incidence was calculated. Study variables included age at diagnosis, sex, ethnicity, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) level. Of 141 patients, 37 (26%) had a positive biopsy result for GCA; 11 underwent a second biopsy for a total of 152 biopsies. The average age of the biopsy-positive patients was 76.5 (SD 8.2) years, and the female-to-male ratio was 2.4:l. There were 35 patients (95%) of European descent and 2 patients (5%) of Aboriginal descent. Twenty-three patients had both ESR and CRP testing done before starting steroids. The ESR was elevated in 19 (83%) and the CRP in 22 (96%). The estimated incidence of GCA for Saskatoon and area was 9.4 per 100,000 for people over the age of 50 years. GCA occurs primarily in people of European descent; however, it can affect North American people of Aboriginal descent. Sensitivity for the detection of GCA is higher in CRP than in ESR. The estimated incidence of GCA in Saskatoon and surrounding referral area is moderate compared with other northern areas.