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Giant Cell Arteritis (Temporal Arteritis) Workup

  • Author: Mythili Seetharaman, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Nov 04, 2015
 

Approach Considerations

The laboratory hallmark of giant cell arteritis (GCA) is an elevation in the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level. The ESR usually exceeds 50 mm/h and may exceed 100 mm/h, but may be normal in 7-20% of patients with GCA.[85, 86, 87] Therefore, a normal ESR does not rule out GCA, and the level of elevation of ESR does not correlate reliably with the severity of the disease. Because normal values of ESR are known to increase with age and are higher in women, the ESR should be adequately adjusted.[88]

The CRP is of hepatic origin, usually rises before ESR in most disease states, and is often elevated in GCA. It has higher sensitivity and specificity than ESR (98.6% and 75.7%, respectively) and is relatively unaffected by age, gender, and other hematologic parameters.[89]

Nonconcordance between ESR and CRP can occur (ie, either an elevated ESR with normal CRP or a normal ESR with an elevated CRP). The use of both tests provides a slightly greater sensitivity for the diagnosis of GCA (99%) than the use of either test alone.[90]

Alpha-2 globulin, fibrinogen, and other acute-phase reactants are elevated mildly but nonspecifically in 72% of patients with GCA. A study of 26 markers related to immune cells identified serum B-cell activating factor (BAFF) and interleukin-6 (IL-6) as having the strongest association with GCA; elevated levels of BAFF and IL-6 accurately distinguished patients with newly diagnosed GCA from healthy controls.[91] However, currently these tests are not readily available in most laboratories.

A complete blood cell count (CBC) should always be obtained. CBC reveals mild normochromic normocytic anemia in most patients, with a mean hemoglobin level of 11.7 ± 1.6 g/dL. Anemia has been shown to have a good negative predictive value for severe ischemic complications in GCA.[92] The leukocyte and differential counts are generally normal. Platelet counts are mildly elevated in most patients (mean platelet count, 427 ± 116 x 10 x 109/L).[93]

Thrombocytosis (platelet count >375,000/µL), although not in itself diagnostic, is more helpful than an elevated ESR for ruling in GCA. Conversely, a normal platelet count is more accurate than a normal ESR for ruling out GCA.[94] Additionally, in the largest population-based GCA study to date in the United States (3001 patients), the combination of elevated platelet counts and CRP levels greater than 2.45 mg/dL was associated with a positive temporal artery biopsy, while ESR results were not as specific a predictor.[95]

Liver function test results (eg, alkaline phosphatase and aspartate aminotransferase), are elevated in 20-30% and 15% of GCA cases, respectively. A prolonged prothrombin time also may be found. Muscle enzyme levels (eg, creatine kinase, aldolase) are normal.

In GCA, the frequency of rheumatoid factor, antinuclear antibodies, and other autoreactive antibodies is not higher than that of age-matched controls. Complement levels are normal, and cryoglobulins and monoclonal immunoglobulins are absent.

Automated visual field testing typically reveals an inferior altitudinal defect, inferior nasal sectorial defect, or central scotoma.

Temporal artery biopsy

Superficial temporal artery biopsy (TAB) is the criterion standard for diagnosing temporal arteritis. TAB should be obtained almost without exception in patients in whom GCA is suspected clinically. It is important because the treatment course for GCA is long and often complicated, and many of the nonspecific symptoms of GCA (eg, headache, body aches, fatigue) occur in myriad other disorders. A positive TAB has 100% specificity but relatively low sensitivity (15-40%) for the diagnosis of GCA.

Imaging studies

Color duplex ultrasonography of the temporal artery has emerged as a promising alternative or complement to TAB.[96, 97, 98, 99, 100, 101] Its specificity is 80-100% when a dark halo (classic halo sign) is seen about the vessel.[98] This key diagnostic feature is believed to represent vessel wall edema. Its sensitivity is limited, however; in particular, early inflammatory changes that can be seen on TAB specimens do not produce the characteristic halo effect.

Prospective study is necessary to validate the utility of the test, but a possible application includes confirming the diagnosis of GCA without performing a biopsy in persons with clinically evident disease. Color duplex ultrasonography is user dependent and is not yet sufficiently sensitive to replace TAB.

At the very least, however, the use of ultrasonography to “map” the path of the artery is a very useful adjunct to the evaluation of a patient with suspected GCA, making considerably more straightforward the choice of incision site and the subsequent location of the artery.[102]

The diagnostic power of high-resolution magnetic resonance imaging (MRI) is comparable to that of color duplex ultrasonography in detecting GCA. Decisions regarding which technique to use may depend on the clinical setting.[103]

Because aneurysms of the aorta or other vessels are often asymptomatic, unless they rupture, screening all patients with GCA for large-vessel disease with computed tomography (CT) is prudent. MRI can also be used to evaluate aortic involvement in GCA.[104] Vessel wall thickening and edema may be evident on T2-weighted images, and increased mural enhancement may be visible on postcontrast T1-weighted images, indicative of regional inflammation. Ultrasonography may be helpful for diagnosing and monitoring aortic involvement in GCA, including aortic aneurysms.[105]

In rare cases, serious suspicion for aortic or carotid artery disease may warrant invasive imaging studies. Aortic arch and cerebral angiography may show occlusion or alternating stenotic areas. However, temporal artery arteriography has no diagnostic value in GCA and does not aid in predicting the proper biopsy site for the temporal artery.

CT and MRI of the brain are not first-line diagnostic procedures for GCA. On CT and MRI, the brain is typically unaffected by GCA, but in patients with a multi-infarct state due to cervicocephalic arteritis, CT and MRI demonstrate multiple areas of infarction.[106, 107, 108, 109]

Positron emission tomography (PET) scanning with 18-fluorodeoxyglucose uptake has been used to evaluate the vasculitic process within large vessels such as the thoracic aorta.[110] PET scanning shows promise in the diagnosis of GCA, but its role is currently unclear.[111]

American College of Rheumatology diagnostic criteria

The following are diagnostic criteria for GCA issued by the American College of Rheumatology in 1990 (the presence of 3 or more yields a diagnostic sensitivity of 93.5% and specificity of 91.2%)[48] :

  • Age 50 years or older
  • New-onset localized headache or localized head pain
  • Temporal artery tenderness to palpation or decreased pulsation
  • ESR of 50 mm/h or higher
  • Positive arterial biopsy results (vasculitis characterized by mononuclear infiltration or granulomatous inflammation, usually with multinucleated giant cells)
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Temporal Artery Biopsy

Superficial temporal artery biopsy (TAB) is the criterion standard for making a diagnosis of temporal arteritis. (See the image below.) TAB should be obtained almost without exception in patients in whom GCA is suspected clinically. It is important because the treatment course for GCA is long and often complicated, and many of the nonspecific symptoms of GCA (eg, headache, body aches, fatigue) occur in myriad other disorders.

Hematoxylin- and eosin-stained superficial tempora Hematoxylin- and eosin-stained superficial temporal artery biopsy specimen, cross section. The hallmark histologic features of GCA shown here include intimal thickening with luminal stenosis, mononuclear inflammatory cell infiltrate with media invasion and necrosis, and giant cell formation in the media.

A positive TAB is diagnostic of GCA (100% specificity). The reported sensitivity of TAB has ranged widely, from as low as 15 to as high as 87%.[112] The histopathological changes on TAB often correlate with clinical features of severity.[8]

TAB should be performed as soon as possible after clinical suspicion is raised. If the index of suspicion is high, the clinician should not delay starting therapy while awaiting TAB. Failure to treat increases the risk of ischemic ocular and cerebral complications, and TAB results remain positive for characteristic GCA pathology for some time after treatment is started.

The reported chances of obtaining a positive biopsy after initiation of steroid treatment vary widely in the literature, from only 10% after 1 week to 86% after 4 or more weeks of treatment.[113, 114] This wide variation may relate to issues such as patient selection, differences in biopsy or pathology techniques, or differences in the disease itself in different populations.

In one large clinical series, TAB was confirmatory in only about one third of patients in whom GCA initially was suspected. Because of this high negative rate, and because GCA produces "skip lesions" with normal intervening segments, large (5 cm) and, if necessary, bilateral TAB specimens should be obtained.

Biopsy of the most symptomatic side should be performed first and, if frozen sections are negative, a contralateral specimen should also be obtained. In cases in which a large TAB section is obtained from the most symptomatic side and multiple thin sections obtained, diagnosis can be made in 86% of cases with a unilateral TAB.

Studies have found that bilateral biopsies do not increase the diagnostic yield in the vast majority of patients (99%).[115, 116, 117, 118] Nevertheless, most physicians with high clinical suspicion despite an initial negative biopsy would still recommend a second contralateral biopsy, given the consequences of a missed diagnosis of GCA.[119]

The clinical significance of giant cells seen on TAB in temporal arteritis is unknown. Armstrong et al found that patients whose TAB contained giant cells had 3 times the rate of blindness and polymyalgia rheumatica compared with the group with no giant cells; although the difference was not statistically significant, it does suggest an association with giant cells and more aggressive disease.[120]

Alberts et al reported that in clinical practice, bilateral temporal artery duplex ultrasonography can serve the same function as biopsy, but without subjecting patients to the potential morbidity of a surgical procedure. TAB could be reserved for cases in which the ultrasonographic result is inconsistent with the clinical picture, in which case the biopsy result, if different, might influence the treatment decision.[97]

Several clinical studies demonstrate that the likelihood of a positive TAB in patients with GCA is greatly enhanced if temporal artery pulses are absent or diminished, even in the absence of other localizing signs. The presence of a headache and jaw claudication may also increase the yield.[121]

TAB is a safe procedure; however, risks include temporary or permanent damage to the temporal branch of the facial nerve, infection, bleeding, hematoma, and dehiscence. Isolated case reports exist of ischemic stroke or skin ulceration from disruption of collateral flow from an asymptomatic carotid occlusion.

Temporal artery biopsy procedure

TAB may be performed by ophthalmologists, general surgeons, head and neck surgeons, plastic or vascular surgeons, or dermatologic surgeons. The frontal branch of the superficial temporal artery is preoperatively identified by using Doppler ultrasonography and then marked with a pen or with dye. In approximately 16% of cases, the frontal branch is atrophic or absent, in which case a biopsy should be performed on the main trunk of the superficial temporal artery using a preauricular approach.[122]

To improve the yield and to avoid complications, proper site selection is important. Focal symptoms or signs, such as erythema, tenderness, absent pulsations, arterial nodularity or swelling, inflammation, bruit, or thickening, help guide biopsy site selection and may improve the yield of the biopsy.

Localizing findings are often absent or misleading, however; frequently, the physical examination findings correlate poorly with the biopsy results. In the absence of localizing findings, the danger zone of the temporal branch of the facial nerve is avoided. Knowledge of the anatomy and careful dissection above and within the superficial temporal fascia help prevent nerve damage during the procedure.

Of particular importance, the zygomatic arch is the most risky location for injury to the temporal branch of the facial nerve. Injury to this nerve leads to an inability to elevate the eyebrows, brow ptosis, paralysis, and a possible asymmetric appearance of the forehead due to loss of the lines and wrinkles on the affected side. A line drawn from the earlobe to the lateral edge of the eyebrow, and from the tragus to just above and behind the highest forehead crease (or 2 cm above supraorbital rim) helps to outline the danger zone where the nerve is superficial.

With the site selected and the patient under local anesthesia, a shallow incision just into the underlying fat is made directly over the artery. The artery is bluntly dissected free from within the superficial temporal fascia. To avoid nerve injury, it is important to undermine just below the dermis in the superficial fat and above the superficial fascia.[123]

A segment of artery is ligated proximally and distally, removed, and sent for histopathologic review. Hemostasis is obtained with electrocoagulation, and a layered closure is performed.

Most authors recommend excision of 2-5 cm of artery to provide accurate diagnosis of temporal arteritis because studies have noted higher positive rates with longer specimens. Temporal arteritis may have a patchy distribution among extracranial arteries and within small segments of these arteries. Longer biopsy specimens provide more tissue in which to demonstrate short, noncontiguous foci of giant cell arteritis, the so-called skip areas.

Skip areas are not commonly identified, but patches of arteritis as short as 0.29 mm have been clearly demonstrated on serial sectioning. Serial sectioning, proper tissue handling, and adequate specimen length are critical to ensure maximum yield from the biopsy. Surgeons should be aware that fixation results in shrinkage of the biopsy specimen.[124]

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Ultrasonography

Although superficial temporal artery biopsy remains the standard for diagnosis of giant cell arteritis (GCA), color duplex ultrasonography can be used to diagnose GCA. A hypoechoic halo around the temporal artery lumen on color duplex sonograms has demonstrated high specificity for GCA, but limited sensitivity; in particular, early inflammatory changes that can be seen on TAB specimens do not produce the characteristic halo effect.[125]

Steroid treatment results in disappearance of the halo. Rarely, the halo may disappear before 2 weeks of steroid treatment; often, it persists for up to 2 months.[126]

The halo represents edema in the artery wall. Some centers consider this finding a major decision-maker in their diagnostic equation, while others do not believe that such a halo is definitive for GCA.

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Cerebral Angiography

The sensitivity of cerebral angiography in GCA affecting the brain is as low as 10–20%. Consequently, angiography is not the procedure of choice in such cases. However, involvement of multiple vessels in multiple vascular beds (a high-probability angiogram) raises the possibility of RCVS (reversible cerebral vasoconstriction). Documentation of reversibility of the angiographic abnormalities, during the course of the disease, will eventually secure the diagnosis of RCVS.[127]

GCA causes granulomatous inflammation in the wall of medium-size and large arteries and preferentially affects extracranial branches of the carotid artery.[128] Occlusion of the posterior ciliary arteries occurs more commonly. Involvement of intracranial arteries is rare, and cerebral infarctions are the hemodynamic consequences of occlusion of cervical arteries.

Magnetic resonance angiography (MRA) and cerebral angiography reveal occlusion of the affected arteries. While MRA can give useful information in GCA, cerebral angiography is the criterion standard to obtain optimum information, but at the cost of potential complications.

In GCA, vertebral arteries are more likely affected than internal carotid arteries, especially in the extradural portion, where there is more elastic tissue.[129] The vertebral and external carotid arteries (including the superficial temporal artery) may show vasculitic changes of alternating stenotic segments or occlusion. The internal carotid arteries may be occluded, but they rarely demonstrate a characteristic vasculitic pattern.

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Electromyographic Studies

Electromyography (EMG) is rarely needed in patients with a clinical presentation of giant cell arteritis. Elderly patients frequently have mildly abnormal nerve conduction studies and EMG findings that suggest a mild peripheral neuropathy, regardless of whether they have GCA. The relationship of such neuropathies to the systemic inflammatory state in some patients is uncertain. In other patients, antecedent ischemic mononeuropathies may occur and eventually resemble a "diffuse," severe, peripheral neuropathy.

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Histologic Findings

The histopathology of the diagnostic arterial lesion in giant cell arteritis (GCA; see the image below) includes the following:

  • Intimal proliferation with resulting luminal stenosis
  • Disruption of the internal elastic lamina by a mononuclear cell infiltrate
  • Invasion and necrosis of the media progressing to involvement of the entire vessel wall (ie, panarteritis) with an inflammatory infiltrate consisting predominantly of mononuclear cells
  • Giant cell formation with granulomata within the mononuclear cell infiltrate
  • Intravascular thrombosis (less consistently found)
    Hematoxylin- and eosin-stained femoral artery bran Hematoxylin- and eosin-stained femoral artery branch, cross section, taken from a lower limb amputation specimen. Mononuclear cell invasion and necrosis in the media of this large artery can be observed. Extensive lower limb vasculitis from GCA resulted in ischemic necrosis of the lower limb, necessitating amputation.

Involvement of an affected artery is patchy, with skip lesions and normal intervening segments. It is commonly accepted that because of the patchy involvement of the arteries, biopsies may be nondiagnostic in many patients, and nondiagnostic biopsy specimens do not exclude the diagnosis of GCA. Some authors even suggest that biopsy may not be necessary.[113]

Early cases or regions with minimal involvement

Collections of lymphocytes are confined to the region of the internal or external elastic lamina or adventitia in early cases or regions of arteries with minimal involvement. Intimal thickening, with prominent cellular infiltration, is typically present.

Late cases or regions with marked involvement

All layers are affected in late cases or regions of arteries with marked involvement. There are widespread areas of necrosis of portions of the arterial wall. The elastic laminae usually are involved, and granulomas containing multinucleated histiocytic and foreign body giant cells, histiocytes, predominantly helper T-cell lymphocytes, and some plasma cells and fibroblasts are usually present.[7]

Weyand and colleagues have extensively described the distribution and function of inflammatory cells in the artery wall.[130, 9] Eosinophils may be seen in the specimen section, but polymorphonuclear leukocytes (PMNs) are rare. Thrombosis may develop at the sites of active inflammation. These areas with thrombosis may recanalize later.

It has been observed that the inflammatory process is usually most marked in the inner portion of the media adjacent to the internal elastic lamina. This has led to the belief that the internal elastic lamina plays a central role in the initiation of the inflammatory process. Fragmentation and disintegration of elastic fibers occur. This is closely associated with an accumulation of giant cells, which often seem to engulf parts of the internal elastic lamina.

The giant cells are difficult to find in some cases, and their absence does not exclude the diagnosis if other features are present. Fibrinoid necrosis is seen less commonly in necrotizing arteritis.

The more sections that are examined in the area of arteritis, the more likely it is that giant cells will be found. What is needed is transmural acute and chronic inflammation for acute diagnosis or evidence of previous repair. Healed or subacute phase shows fibrosis, fragmented internal elastic lamina, chronic inflammatory cells in the intima or media, and ideally neovascularization. Long breaks in internal elastic lamina favor healed arteritis over atherosclerosis.

Intimal proliferation is often marked. However, intimal proliferation is a nonspecific feature in the elderly, and does not suggest past or present arteritis if found alone.

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Contributor Information and Disclosures
Author

Mythili Seetharaman, MD Consultant Rheumatologist, OAA; Clinical Assistant Professor, Thomas Jefferson University Hospital, St Christopher's Hospital for Children

Mythili Seetharaman, MD is a member of the following medical societies: American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, Sigma Xi

Disclosure: Nothing to disclose.

John G Albertini, MD Private Practice, The Skin Surgery Center; Clinical Associate Professor (Volunteer), Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine; President-Elect, American College of Mohs Surgery

John G Albertini, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery

Disclosure: Received grant/research funds from Genentech for investigator.

Stephen A Paget, MD Physician-in-Chief Emeritus, Joseph P Routh Professor of Medicine, New York Hospital, Weill Cornell Medical College; Program Director, Cornell Arthritis and Multipurpose Arthritis and Musculoskeletal Diseases Center (MAMDC), Hospital for Special Surgery

Stephen A Paget, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Manolette R Roque, MD, MBA, FPAO Section Chief, Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center; Section Chief, Ocular Immunology and Uveitis, International Eye Institute, St Luke's Medical Center Global City; Senior Eye Surgeon, The LASIK Surgery Clinic; Director, AMC Eye Center, Alabang Medical Center

Manolette R Roque, MD, MBA, FPAO is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Philippine Medical Association, American Uveitis Society, International Ocular Inflammation Society, Philippine Ocular Inflammation Society, American Society of Ophthalmic Administrators, American Academy of Ophthalmic Executives, Philippine Society of Cataract and Refractive Surgery

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Acknowledgements

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting; Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching

Richard J Caselli, MD Professor, Department of Neurology, Mayo Medical School; Chair, Department of Neurology, Mayo Clinic of Scottsdale

Disclosure: Nothing to disclose.

Steve Charles, MD Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society

Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Other; Topcon Medical Lasers Consulting fee Consulting

Hyland Cronin, MD Resident Physician, Dermatology Department, Geisinger Health System

Disclosure: Nothing to disclose.

Ann G Egland, MD Consulting Staff, Department of Operational and Emergency Medicine, Walter Reed Army Medical Center

Disclosure: Nothing to disclose.

Dirk M Elston, MD Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Gino A Farina, MD, FACEP, FAAEM Associate Professor of Emergency Medicine, Hofstra North Shore LIJ School of Medicine and Albert Einstein College of Medicine; Program Director, Department of Emergency Medicine, Long Island Jewish Medical Center

Gino A Farina, MD, FACEP, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Kilbourn Gordon III, MD, FACEP Urgent Care Physician

Kilbourn Gordon III, MD, FACEP is a member of the following medical societies: American Academy of Ophthalmology and Wilderness Medical Society

Disclosure: Nothing to disclose.

Russell Hall, MD J Lamar Callaway Professor And Chair, Department of Dermatology, Duke University Medical Center, Duke University School of Medicine

Russell Hall, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Federation for Medical Research, American Society for Clinical Investigation, and Society for Investigative Dermatology

Disclosure: Novan Consulting fee Consulting; Stieffel, a GSK company Consulting fee Consulting; Society for Investigative Dermatology Salary Board membership

Jean Marie Hammel, MD Assistant Professor, Associate Residency Director of Emergency Medicine Residency Program, Department of Surgery, Section of Emergency Medicine, Yale University School of Medicine

Jean Marie Hammel, MD is a member of the following medical societies: Alpha Omega Alpha and Phi Beta Kappa

Disclosure: Nothing to disclose.

Leslie W Jackson, MD, LTC, MC Assistant Professor, Department of Medicine, Uniformed Services University of the Health Sciences; Assistant Chief, Rheumatology Service, Department of Medicine, Walter Reed Army Medical Center

Disclosure: Nothing to disclose.

B Mark Keegan, MD, FRCPC Associate Professor of Neurology, College of Medicine, Mayo Clinic; Master's Faculty, Mayo Graduate School; Consultant, Department of Neurology, Mayo Clinic, Rochester

B Mark Keegan, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Minnesota Medical Association

Disclosure: Novartis Consulting fee Consulting; Bionest Consulting fee Consulting; Bristol Meyers Squibb Consulting fee Consulting; Caridian BCT Grant/research funds Other

Richard S Krause, MD Senior Clinical Faculty/Clinical Assistant Professor, Department of Emergency Medicine, University of Buffalo State University of New York School of Medicine and Biomedical Sciences

Richard S Krause, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Christopher H Lee, MD Clinical Instructor, Section of EMS, Department of Emergency Medicine, Yale University School of Medicine

Christopher H Lee, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, Society for Academic Emergency Medicine, and Wilderness Medical Society

Disclosure: Nothing to disclose.

Evan Leibowitz, MD Fellow, Department of Internal Medicine, Division of Rheumatology, Valley Hospital

Evan Leibowitz, MD is a member of the following medical societies: Alpha Omega Alpha and American Medical Association

Disclosure: Nothing to disclose.

Victor J Marks, MD Associate, Department of Dermatology, Section Chief, Dermatologic Surgery, Geisinger Health System

Victor J Marks, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physicians, American Medical Association, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Jorge E Mendizabal, MD Consulting Staff, Corpus Christi Neurology

Jorge E Mendizabal, MD is a member of the following medical societies: American Academy of Neurology, American Headache Society, National Stroke Association, and Stroke Council of the American Heart Association

Disclosure: Nothing to disclose.

Elisabetta Miserocchi, MD Fellow in Immunology and Uveitis Service, Department of Ophthalmology, Harvard Medical School

Disclosure: Nothing to disclose.

Julia R Nunley, MD Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center

Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Arun Ramachandran State University of New York Upstate Medical University

Arun Ramachandran is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Tarakad S Ramachandran, MBBS, FRCP(C), FACP, FRCP Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, FRCP(C), FACP, FRCP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, RoyalCollegeofPhysicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine

Disclosure: Boeringer-Ingelheim Honoraria Speaking and teaching

Barbara L Roque, MD Full Partner, Ophthalmic Consultants Philippines Co; Service Chief, Pediatric Ophthalmology and Strabismus, Department of Ophthalmology, Asian Hospital and Medical Center; Active Staff, International Eye Institute, St Luke's Medical Center Global City; Visiting Ophthalmologist, AMC Eye Center, Alabang Medical Center

Barbara L Roque, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Cataract and Refractive Surgery, Philippine Academy of Ophthalmology, Philippine Society of Cataract and Refractive Surgery, and Philippine Society of Pediatric Ophthalmolo

Disclosure: Nothing to disclose.

Hampton Roy Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska

Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Florian P Thomas, MD, MA, PhD, Drmed Director, Regional MS Center of Excellence, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, St Louis University School of Medicine

Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, National Multiple Sclerosis Society, and Sigma Xi

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Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of Medscape Reference gratefully acknowledge the assistance of Ryan I Huffman, MD, with the literature review and referencing for this article.

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Hematoxylin- and eosin-stained superficial temporal artery biopsy specimen, cross section. The hallmark histologic features of GCA shown here include intimal thickening with luminal stenosis, mononuclear inflammatory cell infiltrate with media invasion and necrosis, and giant cell formation in the media.
Lumbar angiogram showing stenosis and occlusion of femoral artery branches due to vasculitis.
Hematoxylin- and eosin-stained femoral artery branch, cross section, taken from a lower limb amputation specimen. Mononuclear cell invasion and necrosis in the media of this large artery can be observed. Extensive lower limb vasculitis from GCA resulted in ischemic necrosis of the lower limb, necessitating amputation.
Hematoxylin and eosin stain, low power. Temporal artery. Note the thrombosis in the lumen, intimal hyperplasia, and infiltration of the arterial wall muscular layers with inflammatory cells. A multinucleated giant cell is seen internal to the muscularis at the area of the internal elastic lamina (upper right).
Anterior ischemic optic neuropathy. Image courtesy of Richard Kho, MD, Q.C. Eye Center, Quezon City, Philippines.
Branch retinal vein occlusion in a patient with giant cell arteritis. Image courtesy of Manolette Roque, MD, Roque Eye Clinic, Manila, Philippines.
 
 
 
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