Giant Cell Temporal Arteritis Research Articles

Giant Cell Arteritis in Ophthalmology Practice: a Case Report

Introduction. Giant cell (temporal) arteritis refers to a group of chronic and acute systemic vasculitis mainly affecting the extracranial and intracranial arteries of large and medium caliber. Loss of vision due to anterior ischemic optic neuropathy (AION) or occlusion of the central retinal artery is one of the most severe and most common complications of giant cell arteritis. This case report describes a patient with giant cell arteritis, the outcome of it was a total vision loss in the right eye. The patient repeatedly visited the ophthalmologists in the outpatient clinics with complaints of intermittent episodes of vision loss.Purpose. To present methods of diagnosis and treatment, through which the doctor at the initial reception will be able to suspect the disease and start treatment timely.Conclusions. The anamnestic criteria for the GCA diagnosis in the practice of ophthalmologist are: female, age over 50 years, headaches with paresthesia, intermittent lameness of the mandible, short-term episodes of vision loss. The necessary laboratory methods of research include: clinical blood test with determination of erythrocyte sedimentation rate, determination of C-reactive protein level.

Diagnostic Relevance of 18F-FDG PET/CT in Newly Diagnosed Patients with Monoclonal Gammopathy of Undetermined Significance (MGUS) : Single- Center Experience

Introduction:The International Myeloma Working Group (IMWG) consensus aimed to provide recommendations for the optimal use of positron emission tomography-computed tomography (PET/CT) in patients with multiple myeloma (MM) and other plasma cell disorders, including smouldering multiple myeloma (SMM) and solitary plasmocytoma (Cavo M et al., Lancet Oncol. 2017). Monoclonal gammopathy of undetermined significance (MGUS) is one of the most common pre-malignant disorders, in which the probability of progression to MM or other lymphoproliferative disorders is 1% per year (Kyle RA. Blood 2003). MGUS patients have an increased risk of developing hematologic and non-hematologic malignancies (Mailankody S, et al. Blood. 2011; Gregersen H et al., Am J Hematol. 2000). Data for the role of ¹⁸F-FDG PET/CT in diagnostic relevance in newly MGUS patients are still limited. We think, that using ¹⁸F-FDG PET/CT imaging in newly MGUS patients may be useful in early detection of other serious pathologies, not only in predicting progression of MGUS to active MM.Patients and methods:We prospectively analyzed the diagnostic relevance of ¹⁸F-FDG PET/CT in 390 newly MGUS patients . All MGUS patients were diagnosed and monitored at the Department of Internal Hematology and Oncology in Brno, Czech Republic from January 2010 to December 2016. The diagnosis of MGUS was made according to updated 2010 International Myeloma Working Group diagnostic criteria (Kyle RA et al., Leukemia 2010). Imaging scans were reviewed by a radiologist and a nuclear medicine physician. The aim of this analysis was to evaluate the benefit of ¹⁸F-FDG PET/CT in early detection of other serious illnesses in newly MGUS patients. This study did not focuse on the MGUS patients re-classified as MM. Patients underwent ¹⁸F-FDG PET/CT within 6 months of MGUS diagnosis. At the time of ¹⁸F-FDG PET/CT imaging the patients showed no specific symptoms indicative of later diagnosed serious illnesses.Results:A total of 390 newly diagnosed MGUS patients were evaluated, and by IMWG definition had no lytic lesions on skeletal survey. On ¹⁸F-FDG PET/CT, presence of focal or diffuse areas of detectable increased tracer uptake on at least two consecutive slices was recorded in 36 (9.2%) of MGUS patients. The most frequent pathology was lymphadenopathy (3.8%), followed by thyreopathy (2.1%), rheumatologic diseases (1.8%) and others solid tumors (1.3%). Primary malignancy was confirmed by biopsy in 17 (4.4%) patients. This included 12 (3.1%) patients with lymphoproliferative diseases and 3 (0.8%) patients with colon cancers. In 1 case prostate and thyroid cancer was diagnosed.On ¹⁸F-FDG PET/CT focal lymph nodes abnormalities were detected in 15 (3.8%) evaluable patients. Twelve (3.1%) patients were reclassified as having lymphoproliferative diseases. Among the remaining 3 patients with lymph nodes abnormalities 1 patient was detected with Sjögren's syndrome, 1 patient with sarcoidosis, and 1 patient with reactive lymphadenopathy ,without detection of malignant cells after biopsy. None of the patients had symptoms typical for lymphoproliferative diseaseas at the time of ¹⁸F-FDG PET/CT imaging. Thyreopathy was the second most common pathology in our study, observed in 8 (2.1%) patients. In 1 patient thyroid carcinoma was diagnosed; among the remaining 7 (1.8%) patients with focal thyreoid abnormalities nontoxic struma was found. All patients' thyroid hormones tested within the normal range at the time of ¹⁸F-FDG PET/CT imaging. Rheumatologic diseases were the third most common pathology, occurring in 7 (1.8%) of all scanned patients. Specifically, there were 4 (1.1%) patients with rheumatoid arthritis, 2 (0.5%) patients with polymyalgia rheumatica and 1 patient with giant-cell temporal arteritis. In 1 patient thymoma and schwannoma of the femoral nerve were identified. For all these patients, all diseases were early-onset without typical symptoms.Summary:In conclusion, using newer sensitive imaging tool we detected 36 (9.2%) patients with serious pathologies before the first symptoms. Therefore we believe, that ¹⁸F-FDG PET/CT imaging can be recommended to be done not only in all MGUS patients with suspected SMM or active MM, but also for early identification of other serious pathologies. DisclosuresNo relevant conflicts of interest to declare.

Cerebral Vasculitis

Introduction:Vasculitis is an inflammation systems may be involved of blood vessels due to various origins. Vessels of the peripheral and/or central nervous. Vasculitis of the CNS is rare and occurs in the context of systemic diseases or as primary angiitis of the CNS.Epidemiology:The overall incidence of primary vasculitis is about 40/1,000,000 persons [excluding giant cell (temporal) arteritis, GCA]. Its incidence increases with age. The incidence of GCA is much higher (around 200/1,000,000 persons in the age group[50 years).Clinical Presentation:Clinical and pathological presentation in CNS vasculitis represents a wide spectrum. Among others, headache, cranial nerve affections, encephalopathy, seizures, psychosis, myelitis, stroke, intracranial haemorrhage and aseptic meningoencephalitis are described. Primary and secondary vasculitides leading more frequently to CNS manifestations are discussed.Primary and secondary Vasculitides:Including Giant Cell (Temporal) Arteritis , Takayasu arteritis, Polyarteritis nodosa, Primary angiitis of the CNS, Wegener’s granulomatosis, and Connective tissue diseases, such as systemic lupus erythematosus (SLE), scleroderma, rheumatoid arthritis, mixed connective disease and Sjögren syndrome, are systemic immune-mediated diseases that lead to multiple organ affections.Cerebral Vasculitis: Imaging and Differential Diagnosis:Vasculitides represent a heterogeneous group of inflammatory diseases that affect blood vessel walls of varying calibers (inflammatory vasculopathy). Since the devastating symptoms of CNS vasculitis are at least partially reversible, early diagnosis and appropriate treatment are important. In order to establish a differential diagnosis clinical features, disease progression, age of onset, blood results, as well as CSF examinations have to be taken into consideration. Neuroimaging techniques, such as MRI and DSA, play a central role in the diagnosis and disease monitoring .The diagnostic protocol for cerebral vasculitis should include initial MRI to assess the degree of parenchymal damage and to detect vessel wall changes, in particular in large-vessel vasculitis. If the results are ambiguous or medium-sized arteries are affected (beyond the spatial resolution of MRI) DSA should follow. Small-vessel vasculitides entirely evade detection by vascular imaging and consequently require brain or leptomeningeal biopsy . Exclusion of differential diagnoses: Important differential diagnoses include RCVS, intracranial atherosclerosis Moyamoy disease, autoimmune encephalopathies and infectious disorder such as varicella zoster virus (VZV) vasculopathies or endocarditis.Therapy:Vasculitis is a serious disease that is potentially fatal or leads to permanent disability and requires rapid institution of immunosuppressive treatment. Possible therapeutic options include glucocorticoids, cyclophosphamide, azathioprine, intravenous immunoglobulins and mycophenolate mofetil.

Giant Cell Arteritis Presenting with Bilateral Subdural Haematomas of Arterial Origin.

Giant cell arteritis or temporal arteritis is an inflammatory condition affecting medium to large sized vessels, particularly the cranial arteries. A 76-year-old man with no significant past medical history presented to the emergency department with a 3-week history of diffuse headaches associated with fever, loss of appetite, weight loss and general malaise. A CT scan of the brain showed bilateral shallow chronic low density subdural haematomas. A complete laboratory panel was unremarkable except for a raised erythrocyte sedimentation rate and elevated C-reactive protein. A transthoracic echocardiogram and CT scan of the body were unremarkable. MRI of the brain confirmed bilateral old subdural collections and showed marked vessel wall enhancement in the frontal branches of the left superficial temporal artery. A left temporal artery biopsy confirmed giant cell temporal arteritis. We speculate that a vasculitic process in the small subdural arteries may have contributed to our patient’s spontaneous subdural haematomas.LEARNING POINTSA contrast-enhanced T1 axial fat-suppressed MRI of the brain is a non-invasive modality of diagnostic value in giant cell arteritis (GCA); increased access to high resolution MRI technology may reduce the requirement for invasive temporal artery biopsy in the future.Subdural hematomas may be of arterial origin; GCA has been reported in association with subdural hematomas but causation is not proven.GCA must be suspected in patients presenting with headaches and raised inflammatory markers even in the absence of classic features or dual pathology as failure to recognize and treat promptly could lead to permanent visual loss.

Diagnosis value of high frequency ultrasonography and MRI in giant cell arteritis

Objective To study the diagnosis value of giant cell temporal arteritis with high frequency ultrasonography and magnetic resonance imaging.Methods The total of 29 cases testified as giant cell temporal arteritis clinically were assessed by high frequency ultrasonography and magnetic resonance imaging.The 29 cases were assessed by high frequency ultrasonography,the 11 cases were assessed by magnetic resonance imaging,mural thickness and lumen diameter of temporal arteries were examined,and were compared with the biopsy specimens.Meanwhile 30 healthy volunteers were randomly selected as control group,the ultrasonography of normal temporal arteries were analyzed.Results All temporal arteries were diaplayed clearly.27 cases were diagnosed for giant cell temopral arteritis by high frequency ultrasonography in 29 cases,the diagnostic accuracy was 93.1％.The 11 cases were diagnosed by magnetic resonance imaging,the diagnostic accuracy was 100％.The imaging features were mural thickness,the lumen stenosis,and partial temporal arteries occluded,the mural was contrasted by Gd-DTPA.Conclusions High frequency ultrasonography and magnetic resonance imaging are noninvasive methods for giant cell temporal arteritis diagnosis,it is important clinical value for therapy. Key words: Ultrasonography; Magnetic resonance imaging; Temporal arteries ; Giant cell arteritis

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.

Giant cell arteritis associated with chronic active Epstein-Barr virus infection

Giant cell arteritis is an inflammatory vasculopathy that preferentially affects medium-sized and large arteries. A viral cause has been suspected but not confirmed in polymyalgia rheumatica and giant-cell arteritis. We report the case of a 81-year-old female who suffered from chronic active Epstein-Barr virus infection and developed giant cell temporal arteritis.

Bitemporale Skalpnekrose

A 71-year-old woman developed progressive spreading of bitemporal scalp necrosis within 4 weeks accompanied by headaches, myalgia of the shoulder girdle and muscle weakness that had started a few months previously. No additional diseases were reported. The suspected temporal giant cell arteritis could be confirmed by temporal artery biopsy. Therapy with glucocorticoids led to a rapid resolution of clinical symptoms and was tapered over 18 months. Recovery of the scalp necrosis emerged following second intention healing and split-skin transplantation of necrotic areas after successful wound conditioning. The case study demonstrates a rare and serious complication of temporal arteritis which is often accompanied by a poor prognosis.

Giant Cell Temporal Arteritis Associated with Overlying Basal Cell Carcinoma: Co-Incidence or Connection?

Giant cell arteritis is a granulomatous vasculitis of large and medium sized arteries manifesting as temporal arteritis and/or polymyalgia rheumatica. The histological assessment of temporal artery biopsies is frequently encountered in anatomical pathology and has important diagnostic consequences in patients clinically suspected of having giant cell arteritis. We present an intriguing case of giant cell arteritis associated with a Basal cell carcinoma and discuss the ongoing controversy pertaining to the association of giant cell arteritis/polymyalgia rheumatica with malignancy.

Association between human papillomavirus DNA and temporal arteritis

BackgroundTo examine the relationship between human papillomavirus (HPV) and giant cell arteritis (GCA) of the temporal artery.MethodsThe study group consisted of 22 cases of histologically positive/biopsy confirmed GCA. The control groups consisted of 21 histologically negative temporal artery biopsies and fifteen cases of vascular margins of nephrectomies. For detection of the presence of HPV, two methods were used: 1) polymerase chain reaction (PCR) with INNO-LiPA HPV Genotyping Extra, 2) Cervista™ HPV HR. All cases were from the files of the Barnes-Jewish Hospital and Washington University in St. Louis.ResultsHPV DNA was detected by PCR and genotyping in 16 of 22 (73%) histologically positive cases of GCA and in only five of 21 (24%) histologically negative temporal artery biopsies. Among the vascular margin controls, only three of 15 (20%) were positive for HPV DNA. The second, independent method (CervistaTM) confirmed the aforesaid results with 100% concordance with the exception of three cases which had low genomic DNA for which it was not possible to perform the test. The differences in HPV positivity between the histologically positive and negative temporal artery biopsies and between the histologically positive temporal artery biopsies and controls were both statistically significant (p = 0.001 and 0.002, respectively).ConclusionsThe results of our study revealed a statistically significant association between HPV positivity and biopsy confirmed temporal giant cell arteritis GCA (p = 0.001). Further studies are necessary to elucidate the pathophysiology underlying this association.

Retinal oximetry in central retinal artery occlusion

A 69-year-old woman experienced dizziness when she woke up and realized that she had almost completely lost her vision on the left eye. She has suffered from polymyalgia rheumatica and had received oral prednisolone until 2 months earlier. The following day she had bare light perception and a pupillary afferent defect on the left eye. The left fundus showed central retinal artery occlusion with segmented blood columns (‘boxcarring’, Fig. 1) in the arterioles and venules, marked ischaemic oedema and a cherry red spot in the fovea. The right eye had normal visual acuity of 1.0. The intraocular pressure was normal in both eyes. Sedimentation rate was only 26 and 29 mm/h on repeated measurements (Elfarsson et al. 2010). Top left: the patient’s left eye approximately 30 h following the onset of the central retinal artery occlusion. The interrupted blood column (‘boxcarring’) in retinal arterioles and venules indicates the total occlusion of the central retinal artery. The box indicates the area shown on the oximetry images. Top right: temporal artery biopsy showing intimal thickening with inflammation adjacent to the internal elastic membrane and in the adventitia. Bottom left: the initial spectrophotometric retinal oximetry images taken 30 h after the onset of central retinal artery occlusion. The retinal arterioles have only 71 ± 9% oxygen saturation (mean ± SD for major arterioles) and the oxygen saturation in the venules is slightly lower; 63 ± 9%. Bottom right: retinal oximetry images taken 1 month following the onset of central retinal artery occlusion. The retinal arterioles now show oxygen saturation of 100 ± 4%, consistent with uninterrupted arterial blood flow. Given the history and arterial occlusion and a normal ultrasound examination of heart and carotid arteries, a tentative diagnosis of temporal arteritis was made. Treatment was started with prednisolone tablets 1 mg/kg and a temporal artery biopsy obtained. Histological examination confirmed temporal giant cell arteritis (Fig. 1) and prednisolone treatment was continued. Ultrasound examination of the heart and carotid arteries was performed, keeping in mind that Miyazawa et al. (2011) reported that carotid stenosis is frequently associated with central retinal artery occlusion. Also, Mansour & Younis (2011) and Pournaras et al. (2010) suggested that ocular paracentesis and hypotensive treatment may be considered, even this late (>24 h) following the onset of central retinal artery occlusion. Spectrophotometric retinal oximetry (Hardarson et al. 2006) was performed on both eyes approximately 30 h after she noticed the vision loss. Retinal arterioles showed oxygen saturation of 71 ± 9% (mean ± SD for major arterioles) and the venular oxygen saturation was 63 ± 9% (Fig. 1). The corresponding values for the normal fellow eye were 95 ± 5% (arterioles) and 66 ± 8% (venules). The low arteriovenous difference in the affected eye is consistent with stagnant blood in the vasculature, cell death and decreased oxygen consumption. One month later the eye remained blind, although the perfusion of the retinal circulation had been restored. The oxygen saturation in the arterioles had returned to 100 ± 4%, while the venous saturation was 54 ± 5% (Fig. 1). At this time, the arteriolar saturation in the fellow eye was 100 ± 4% and the venular saturation 60 ± 6%. The pathogenesis of central retinal artery occlusion has been extensively studied (Hayreh 2011) although few studies are available on the influence of occlusion on human retinal oxygenation. Hammer et al. (2009) investigated branch and central retinal artery occlusions with a two wavelength oximeter. At the time of diagnosis, which was within 48 h of the occlusion, they found low arterial saturation (78%) in the occluded branch artery, similar to our findings, and a considerable increase (91%) 5 days later, after treatment with pentoxifylline for improvement of blood flow. Saturation after central retinal artery occlusion was, however, initially close to normal (93%) although it did increase after 5 days and treatment (103%). Venous saturation in central retinal artery occlusion was initially slightly below normal (55%) and had increased to 70% 5 days later. A later paper by the same group confirmed the results for occluded branch retinal arterioles (Gehlert et al. 2010). Spectrophotometric retinal oximetry is a relatively new form of diagnostic imaging. It can clearly show decreased arterial oxygen saturation following central retinal artery occlusion and may thus become helpful in diagnosing this disorder. It also demonstrates the return of blood flow and may therefore be useful for follow-up of patients with arterial occlusions. To realize fully the usefulness of this method for diagnosis and follow-up of central retinal artery occlusion and other retinal vasculopathies, further clinical studies are needed.

Abstract 17956: Serpin Treatment Reduces Inflammation in Transplanted Temporal Artery Biopsy Specimens from Patients with Suspected Giant Cell Arteritis in Immunodeficient Mice

Background - Giant cell / temporal arteritis (GCTA) is a severe inflammatory vasculitic syndrome (IVS), which causes arterial occlusion and sudden loss of vision. Treatment for IVS remains limited. Intra-aortic full thickness implants of human TA biopsy and left internal mammary artery (LIMA) specimens were surgically engrafted into the abdominal aorta of immunodeficient SCID mice as a new model for analyzing development of vascular inflammation, as well as response to new treatments. Serp-1 is a myxomaviral-derived serine protease inhibitor (serpin) that blocks thrombotic and thrombolytic protease pathways, arterial inflammation, and plaque growth. Serp-1 was recently tested in acute coronary syndromes (ACS), where reduced markers of myocardial damage were detected after stent implant. Objective - We assessed the effects of Serp-1 or saline treatment on inflammatory cell invasion and plaque growth after TA and LIMA engraftment in NOD-SCID mice. Results - Normal arterial (LIMA) specimens were harvested during bypass surgery and implanted into 24 SCID mice. Half the mice had saline (N=12) and half had Serp-1 treatment (N=12). In each group 6 mice had 5x106 peripheral blood mononuclear cells (PBMCs) infusions from the same patient as the donor LIMA graft, given immediately postop by tail vein and 6 had no cell infusion. TA grafts from patients with suspected GCA were implanted into a separate group of 24 SCID mice - 12 mice had saline (100µl) and 12 Serp-1 (100ng/g/100µl) treatment (one i.v. injection followed by daily i.p. injection for 10 days). in each treatment group 4 mice had infusion of non-matched PBMCs. At 4 weeks LIMA (P < 0.034) or TA (P < 0.0001) grafts, had significantly reduced plaque growth with Serp-1 treatment when human PBMC were also infused (p<0.05). In the absence of PBMC, Serp-1 had minimal effect. Mononuclear cell invasion was reduced in Serp-1 treated mice with PBMCs. Flow cytometry did not detect significant changes in splenocytes. Conclusions - Aortic implant of human LIMA or TA xenografts in SCID mice provides a physiological model for investigating inflammatory vasculitis. With PBMC infusion, treatment with the myxomaviral Serp-1, reduced inflammatory cell invasion and plaque growth in arterial xenografts.

Arterite temporal: confusão no diagnóstico.

Temporal arteritis is a chronic vasculitis of medium and large-size vessels and involves particularly extracranial branches of the aortic arch arteries. Authors report the case of a 73-year-old woman who presented to the hospital after looking for medical counselling three times because of unexplained fever, fatigue, nonproductive cough, and throat pain. She already completed two antibiotic prescriptions. This 3-week history completed in the last days with temporal bilateral headache and visual disturbance. Physical examination was notable for fever and bilateral thickened tender temporal arteries. The erythrocyte sedimentation rate was elevated. A biopsy specimen of the left temporal artery confirmed the diagnosis of giant cell temporal arteritis. The diagnostic suspicion of this disease is clinical and usually simple, but in 10% there are throat pain, non-productive cough and fever which are misunderstood as superior respiratory tract infections leading to diagnosis and treatment delay.

An elastic Van Gieson stain is unnecessary for the histological diagnosis of giant cell temporal arteritis

BackgroundElastic Van Gieson (EVG) stain is used routinely to identify the internal elastic lamina in biopsy specimens from clinically suspected cases of giant cell arteritis (GCA). It is widely recognised,...

Wertigkeit der farbkodierten Duplexsonographie in Abhängigkeit der klinischen Vortest-Wahrscheinlichkeit bei Riesenzellarteriitis

Colour Doppler sonography (CDS) is an established technique in the diagnosis of giant-cell (temporal) arteritis (GCA). The predictive value of its diagnostic criteria for GCA (halo sign or stenosis) is related to the pretest probability (PTP), a measure of probability of presence of a target disease before the result of a diagnostic test is known. A total of 182 (average age 69 years, 69% women) patients of the Rheumatology Center Baden-Württemberg were investigated. Based on the diagnostic criteria of the American College of Rheumatology (ACR) they were assigned to one of three groups, before a CDS was performed: group 1 (n= 139) patients with "isolated" polymyalgia rheumatica (PMR) and a low PTP for GCA; group 2 (n=19) patients with intermediate PTP and nonspecific headache and fewer than three ACR criteria for GCA); and group 3 (n=224) patients with a high PTP and new headache loclized to the temporal artery and at least three ACR criteria for GCA. The halo sign (periluminal dark halo) of more than 0.3 mm was present in 26% of group 1. 42% of those in group 2 and 83% of those in group 3. A stenosis or occlusion of the temporal artery was present in 3.5% (group 1), 5% (group 2) and 46% (group 3), respectively. 3 of 24 patients of group 3 also had a stenosis of the axillary or brachial artery. Concordance between clinical criteria and CDS (normal CDS in patients with PMR but no headache or abnormal CDS and clinically suspected BCA was found in 123 of 182 patients (67.5%). In these patients biopsy of the temporal artery ("gold standard" for the diagnosis of GCA) was not recommended. Temporal artery biopsy was, however, recommended in all patients with discordant findings (abnormal CDS with PMR but no headache or normal CDS with clinically suspected GCA, and also those with intermediary PTP (32%). A biopsy was performed in 42 of these patients after informed consent had been obtained. This demonstrated vasculitis in 11 of 25 patients with PMR (PPV in group 1: 0.44). But biopsies were negative in all four patients with clinically suspected GCA and normal CDS (NPV in group 3:1). In the intermediary group biopsy demonstrated vasculitits in 5 of 6 patients with an abnormal CDS (PPV 0.63), while 4 of 5 patients with a normal CDS had a normal biopsy (NPV 0.8). Taking into account pretest probability, an RCA can be accurately diagnosed or excluded by CDS in two thirds of patients without biopsy. When performed by an experienced investigator CDS is a basic part in the diagnosis of CDA.

Inflammation and stroke

It is well-known that inflammation plays a major role in the genesis of the atherosclerotic plaque and thus favours the occurrence of stroke. But, inflammation is also involved after an ischaemic event affecting the brain. In this case, one can observe the infiltration of numerous inflammatory cells at the site of the lesion, the activation of the microglia and of pro-inflammatory cytokines, etc. It is usually thought that this poststroke inflammation is rather deleterious, as suggested by the fact that, after an experimentally-induced ischaemic stroke, blockade of the inflammatory response improves the neurological condition of mice. Nevertheless, until now, the application of such experimental treatments in humans has revealed unsuccessful. One of the possible explanations for this phenomenon might be that inflammation also has some beneficial effects, such as clearance of damaged tissue, promotion of angiogenesis, or still tissue remodelling and regeneration. After this first “basic science” part, we will briefly review some clinical aspects of the most significant inflammatory diseases that can cause stroke, i.e., the vasculitis. Among them, Takayasu’s arteritis, giant cell temporal arteritis (Horton’s disease), and primary angiitis of the central nervous system will be discussed. We will also shortly address the question of the antiphospholipid syndrome.

Giant cell temporal arteritis with a normal erythrocyte sedimentation rate: report of a case

Giant cell temporal arteritis (GCA) is a systemic vasculitis that affects individuals older than 50. Erythrocyte sedimentation rate (ESR) has typically been used in the ED as a screening test to rule out GCA. There have been documented cases in medicine and rheumatology journals of biopsy-proven giant cell arteritis with a normal or low ESR before steroid therapy. In the ED, the practitioner must use multiple resources to make the diagnosis of GCA, as there is no specific test that establishes the diagnosis. Delaying this diagnosis based on a laboratory value can create substantial morbidity and mortality for the patient. We report a case of an elderly woman who developed visual changes and had a delayed diagnosis because of a normal ESR value.

Ischaemic necrosis of the tongue secondary to cardiogenic shock: A case series

INTRODUCTION Ischemic tongue necrosis is rare, with fewer than 15 reported cases in the world literature, all related to giant cell (temporal) arteritis. We previously reported the first case of tongue gangrene as a sequela of profound cardiogenic shock, and now present our updated experience . Our institution’s cardiac surgery service has accrued a total of 4 patients with ischemic necrosis of the tongue as an end-organ manifestation of profound cardiogenic shock.

Poster 340: Rehabilitation Considerations for Ischemic Myelopathy Secondary to Giant Cell Arteritis: A Case Report

Setting: Freestanding acute rehabilitation hospital. Patient: A 60-year-old woman. Case Description: A previously independent, 60-year-old woman developed sudden lower limb (LL) weakness and anesthesia, imbalance, and loss of bowel and bladder function secondary to ischemic myelopathy from biopsy-confirmed giant cell temporal arteritis (GCA). Following high dose steroids within 36 hours of symptom onset, she was transferred for acute inpatient rehabilitation. On admission, most LL muscles were less than antigravity strength.

Polymyalgia rheumatica and giant cell arteritis

Among 47 patients with polymyalgia rheumatica there were 30 (63.8%) with temporal giant-cell arteritis. Rapidly progressing visual reduction occurred in 2, acute blindness in 10. Four forms of polymyalgia rheumatica were identified by their different clinical courses, with differing risk of blindness. Those patients with polymyalgia rheumatica and giant-cell arteritis are most endangered who have headaches and neck and shoulder pains, but no or only mild general symptoms.