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Giant Cell Ar teritis (Temporal Arteritis)

Author: Mythili Seethar aman, MD; Chief E ditor: Herbert S Diamond, MD  more...
 

Updated: Nov 04, 2015

 

Background
Giant cell ar teritis (GCA), or tempor al arteritis, is a systemic inflammat ory
vasculitis of unknown etiology that occurs in older persons and can r esult in a wide
variety of systemic, neur ologic, and ophthalm ologic complications. GCA is the
most common form of systemic v asculitis in adults. Other names for GCA include
arteritis cranialis, Hor ton disease, granulomatous arteritis, and ar teritis of the aged.

GCA typically aff ects the superficial tempor al arteries—hence the term tempor al
arteritis. In addition, GCA most commonly aff ects the ophthalmic, occipital,
vertebral, posterior ciliar y, and proximal vertebral arteries. It has also been shown
to involve medium- and lar ge-sized vessels, including the aor ta and the car otid,
subclavian, and iliac ar teries. [1, 2, 3]

Histopathologically, GCA is mark ed by transmural inflammation of the intima,

media, and adv entitia of affected arteries, as well as patchy infiltr ation by
lymphocytes, macr ophages, and multinucleated giant cells. Mur al hyperplasia can
result in ar terial luminal narr owing, resulting in subsequent distal ischemia. (See
Pathophysiology.)

Age and female sex are established risk fact ors for GCA, a genetic component
seems likely, and infection may have a role (see Etiology). One school of thought
considers GCA and polymyalgia rheumatica to be different manifestations of the
same disease pr ocess, while others see them as closely r elated but diff erent
diseases. [4]

Common signs and sympt oms of GCA r eflect the involvement of the tempor al
artery and other medium-siz ed arteries of the head and the neck and include visual
disturbances, headache, jaw claudication, neck pain, and scalp tenderness.
Constitutional manif estations, such as fatigue, malaise, and f ever, may also be
present. (See Pr esentation.)

GCA should alwa ys be consider ed in the differential diagnosis of a new-onset

headache in patients 50 y ears of age or older with an ele vated erythrocyte
sedimentation r ate. Temporal artery biopsy remains the criterion standar d for
diagnosis of this gr anulomatous vasculitis (see the image below).

Hematoxylin- and eosin-stained superficial tempor al 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 ni vasion and necrosis, and giant cell
formation in the media.

Visual loss is one of the most significant causes of morbidity in GCA. P ermanent
visual impairment ma y occur in as many as 2 0% of patients, and, in some cases,
GCA can cause bilater al blindness. [5] Newly recognized GCA should be consider ed
a true neuro-ophthalmic emer gency.

Prompt initiation of tr eatment may prevent blindness and other potentially

irreversible ischemic sequelae of GCA. [6] Corticosteroids are the mainsta y of
therapy. In steroid-resistant cases, drugs suc h as cyclosporine, azathioprine, or
methotrexate may be used as ster oid-sparing agents. The typical patient with GCA
remains on ster oid therapy for roughly 2 years. (See Treatment and Medication.)
 

Contributor Information and Disclosures

Author
Mythili Seethar aman, MD Consultant Rheumat ologist, OAA; Clinical As sistant Professor, Thomas Jeff erson
University Hospital, St Christ opher's Hospital for Children

Mythili Seethar aman, MD is a member of the following medical societies: American College of Rheumat ology,
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, Depar tment of Ophthalmology , Massachusetts E ye and Ear Infirmar y; Founder and Pr esident,
Ocular Immunology and Uv eitis Foundation, M assachusetts E ye Research and Surgery Institution

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO is a member of the following medical so cieties: Alpha Omega
Alpha, American Academy of Ophthalmology , American Association of Immunologists , American College of
Rheumatology, American College of Sur geons, American Federation for Clinical Resear ch, American Medical
Association, American Society for Micr obiology, American Uveitis Society , Association for Resear ch in Vision
and Ophthalmology , Massachusetts Medical Society , Royal Society of Medicine , Sigma Xi

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Alde yra
Therapeutics (Lexingt on, MA); Bausch & Lom b Surgical, Inc (Rancho Cucamonga, CA); E yegate Pharma
(Waltham, MA); Novartis (Cambridge, M A); pSivida (Watertown, MA); Xoma (Berkeley, CA)<br/>Receiv ed
research grant from: Alcon; Alde yra Therapeutics; Bausch & Lomb; Clearside Biomedical; Dompé
pharmaceutical; E yegate Pharma; Mallinckr odt pharmaceuticals; No vartis; pSivida; Santen.
John G Alber tini, MD Private Practice, The Skin Sur gery Center; Clinical Associate Pr ofessor (Volunteer),
Department of Plastic and Reconstructiv e Surgery, Wake Forest University School of Medi cine; President-Elect,
American College of Mohs Sur gery

John G Alber tini, MD is a member of the following medical societies: American Academy of Dermat ology,
American College of Mohs Sur gery

Disclosure: Received grant/research funds from Genentech for inv estigator.

Stephen A Paget, MD Physician-in-Chief Emeritus, Joseph P Routh Pr ofessor of Medicine, New Y ork Hospital,
Weill Cornell Medical College; Pr ogram Director, Cornell Ar thritis and Multipurpose Ar thritis and Musculosk eletal
Diseases Center (M AMDC), Hospital for Special Sur gery

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

Disclosure: Nothing to disclose.

Manolette R Roque, MD , MBA, FPAO Section Chief, Ocular Immunology and Uv eitis, Depar tment of
Ophthalmology, Asian Hospital and Medical Center; Section Chief, Ocular Immunology and Uveitis, International
Eye Institute, St L uke's Medical Center Global City; Senior E ye Surgeon, The L ASIK 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 Catar act and Refractive Surgery, Philippine Medical Association , American
Uveitis Society , International Ocular Inflammation Society , Philippine Ocular Inflammation Society , American
Society of Ophthalmic Administr ators, American Academy of Ophthalmic Ex ecutives, Philippine Society of
Cataract and Refractive Surgery

Disclosure: Nothing to disclose.

Chief Editor
Herbert S Diamond, MD  Visiting Professor of Medicine, Division of Rheumat ology, State University of New York
Downstate Medical Center; Chairman Emeritus, Depar tment of Internal Medicine, W estern Pennsylvania
Hospital

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

Disclosure: Nothing to disclose.

Acknowledgements
Lawrence H Brent, MD Associate Pr ofessor of Medicine, Jeff erson Medical College of Thomas Jeff erson
University; Chair, Program Director, Department of Medicine, Division of Rheumat ology, 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 Rheumat ology

Disclosure: Abbott Honor aria Speaking and te aching; Centocor Consulting f ee Consulting; Genentech
Grant/research funds Other; HGS/GSK Honor aria Speaking and teaching; Omnicar e Consulting f ee Consulting;
Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Sa vient Honoraria Speaking and
teaching; UCB Honor aria Speaking and teach ing

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

Disclosure: Nothing to disclose.

Steve Charles, MD Director of Charles Retina Institute; Clinical Pr ofessor, Department of Ophthalmology ,
University of Tennessee College of Medicine; Adjunct Pr ofessor of Ophthalmology , Columbia College of
Physicians and Sur geons; Clinical Pr ofessor Ophthalmology , Chinese Univ ersity of Hong K ong

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 Labor atories Consulting f ee Consulting; OptiMedica Ownership inter est Other; Topcon
Medical Lasers Consulting f ee Consulting

Hyland Cronin, MD Resident Physician, Dermat ology Depar tment, Geisinger Health System

Disclosure: Nothing to disclose.

Ann G Egland, MD Consulting Staff, Depar tment of Operational and Emergency Medicine, W alter Reed Army
Medical Center

Disclosure: Nothing to disclose.

Dirk M Elston, MD Director, Ackerman Academy of Dermat opathology, New York

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

Disclosure: Nothing to disclose.

Gino A Farina, MD, FACEP, FAAEM Associate Pr ofessor of Emer gency Medicine, Hofstr a North Shore LIJ School
of Medicine and Alber t Einstein College of Medicine; Pr ogram Director, Department of Emer gency 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 Emer gency Physicians , and Society for Academic Emer gency
Medicine

Disclosure: Nothing to disclose.

Kilbourn Gordon III, MD, FACEP Urgent Care Physician

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

Disclosure: Nothing to disclose.

Russell Hall, MD J Lamar Callawa y Professor And Chair, Department of Dermat ology, Duke University Medical
Center, Duke University School of Medicine

Russell Hall, MD is a member of the following medical societies: American Academy of Dermat ology, American
Dermatological Association , American Federation for Medical Resear ch, American Society for Clinical
Investigation, and Society for Inv estigative Dermatology

Disclosure: Novan Consulting f ee Consulting; Stieff el, a GSK company Consulting f ee Consulting; Society for
Investigative Dermatology Salar y Board membership

Jean Marie Hammel, MD Assistant Professor, Associate Residency Di rector of Emergency Medicine Residency
Program, Depar tment of Surgery, Section of E mergency Medicine, Y ale 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 Ser vices University of
the Health Sciences; Assistant Chief, Rheumat ology Service, Depar tment of Medicine, W alter Reed Army
Medical Center

Disclosure: Nothing to disclose.

B Mark Keegan, MD, FRCPC Associate Pr ofessor of Neur ology, College of Medicine, Ma yo Clinic; Master 's
Faculty, Mayo Graduate School; Consultant, Depar tment of Neur ology, Mayo Clinic, Rochester

B Mark Keegan, MD, FRCPC is a member of th e following medical societies: American Academy of Neur ology,
American Medical Association , and Minnesota Medical Association

Disclosure: Novartis Consulting f ee Consulting; Bionest Consulting f ee Consulting; Brist ol Meyers Squibb
Consulting fee Consulting; Caridian BC T Grant/research funds Other

Richard S Krause, MD Senior Clinical F aculty/Clinical Assistant Pr ofessor, Department of Emer gency Medicine,
University of Buffalo State Univ ersity of New York School of Medicine and Biomedical Sciences

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

Disclosure: Nothing to disclose.

Christopher H Lee, MD Clinical Instruct or, Section of EMS, Depar tment of Emergency Medicine, Y ale University
School of Medicine

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

Disclosure: Nothing to disclose.

Evan Leibowitz, MD Fellow, Department of Internal Medicine, Divi sion of Rheumat ology, Valley Hospital

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

Disclosure: Nothing to disclose.

Victor J Marks, MD Associate, Depar tment of Dermat ology, Section Chief, Dermat ologic Surgery, Geisinger
Health System

Victor J Marks, MD is a member of the following medical societies: American Academy of Dermat ology,
American College of Mohs Micr ographic 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 Neur ology

Jorge E Mendizabal, MD is a member of the following medical societies: American Academy of Neur ology,
American Headache Society , National Stroke Association , and Stroke Council of the American Hear t
Association

Disclosure: Nothing to disclose.

Elisabetta Miser occhi, MD Fellow in Immunology and Uv eitis Service, Department of Ophthalmology , Harvard
Medical School

Disclosure: Nothing to disclose.

Julia R Nunle y, MD Professor, Program Director, Dermatology Residency, Department of Dermat ology, Virginia
Commonwealth Univ ersity Medical Center

Julia R Nunle y, MD is a member of the following medical societies: American Academy of Dermat ology,
American College of Physicians , American Society of Nephr ology, International Society of Nephr ology, Medical
Dermatology Society , Medical Society of Vir ginia, National Kidne y Foundation, Phi Beta Kappa , and Women's
Dermatologic Society

Disclosure: Nothing to disclose.

Arun Ramachandr an State University of New York Upstate Medica l University

Arun Ramachandr an is a member of the follo wing medical societies: American Medical Association

Disclosure: Nothing to disclose.

Tarakad S Ramachandr an, MBBS, FRCP(C ), FACP, FRCP Professor Emeritus of Neur ology and Psy chiatry,
Clinical Professor of Medicine, Clinical Pr ofessor of Family Medicine, Clinical Pr ofessor of Neurosurgery, State
University of New York Upstate Medical Univ ersity; Neuroscience Director, Department of Neur ology, Crouse
Irving Memorial Hospital

Tarakad S Ramachandr an, MBBS, FRCP(C ), FACP, FRCP is a member of the following medical societies:
American Academy of Neur ology, American Academy of P ain Medicine , American College of F orensic
Examiners, American College of International Physicians, American College of Managed Car e Medicine,
American College of Physicians , American Hear t Association , American Stroke Association , Royal College of
Physicians, RoyalCollegeofPhysicians and Sur geons of Canada , Royal College of Sur geons of England , and
Royal Society of Medicine

Disclosure: Boeringer-Ingelheim Honor aria Speaking and teaching

Barbara L Roque, MD Full Partner, Ophthalmic Consultants Philippines Co; Ser vice Chief, Pediatric
Ophthalmology and Str abismus, Depar tment of Ophthalmology, Asian Hospital and Medic al Center; Activ e Staff,
International E ye Institute, St L uke's Medical Center Global City; Visiting Ophthalmologist, AMC Eye Center,
Alabang Medical Center

Barbara L Roque, MD is a member of the follo wing medical societies: American Academy of Ophthalmology ,
American Association for P ediatric Ophthalmology and Str abismus, American Society of Catar act and
Refractive Surgery, Philippine Academy of Ophthalmology , Philippine Society of Catar act and Refractive Surgery,
and Philippine Society of P ediatric Ophthalmolo

Disclosure: Nothing to disclose.

Hampton Roy Sr, MD Associate Clinical Pr ofessor, 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 Sur geons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Pr ofessor, University of Nebraska

Medical Center College of Pharmacy; E ditor-in-Chief, Medscape Drug Ref erence

Disclosure: Medscape Salar y Employment

Florian P Thomas, MD , MA, PhD, Drmed Director, Regional MS Center of Ex cellence, St Louis Veterans Affairs
Medical Center; Dir ector, National MS Society Multiple Scler osis Center; Dir ector, Neuropathy Association Center
of Excellence, Professor, Department of Neur ology and Psy chiatry, Associate Pr ofessor, Institute for Molecular
Virology, St Louis Univ ersity School of Medicine

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

Disclosure: Nothing to disclose.

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

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermat ology,
Association of Militar y Dermatologists, Texas Dermatological Society , and Texas Medical Association

Disclosure: Nothing to disclose.

Acknowledgments

The authors and edit ors of Medscape Ref erence gratefully acknowledge the assistance of Ry an I Huffman, MD,
with the literature review and referencing for this article.

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