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Sections Giant Cell Arteritis (Temporal Arteritis)
- Overview
- Presentation
- DDx
- Workup
- Treatment
- Guidelines
- Medication
- Questions & Answers
- Media Gallery
- References
Diagnostic Considerations
Many of the clinical manifestations of giant cell arteritis (GCA) are nonspecific, and consequently the differential diagnosis is long. GCA can cause blindness, and this dreaded complication can often be prevented with prompt diagnosis and institution of therapy. Failure to do so represents a medicolegal pitfall in addition to suboptimal medical practice. Treatment is long and often complicated, however, so overdiagnosis also has hazards.
Polymyalgia rheumatica
Polymyalgia rheumatica (PMR) is a clinical syndrome characterized by aching in the proximal portions of the extremities and torso. Diagnostic criteria include aching and morning stiffness lasting half an hour or more in 2-3 commonly affected areas (eg, neck, shoulder girdle, hip girdle) for at least 1 month in a person aged 50 years or older. Aching and stiffness are also worse with exertion, and may be severe and incapacitating.
In PMR, muscles may be tender, disuse may lead to atrophy, and contractures may develop. Muscle strength is usually normal but is often difficult to evaluate because of pain.
In typical cases of PMR without vasculitic involvement of the peripheral nervous system, electromyographic (EMG) results are normal. Mild EMG abnormalities in an elderly patient that suggest a mild peripheral neuropathy are generally unrelated to PMR or vasculitis. Erythrocyte sedimentation rate (ESR) elevation (40-50 mm/h via Westergren method) is typically found, with a rapid response to small doses of corticosteroids (prednisone 10 mg/d).
PMR is a diagnosis of exclusion. The presence of other specific disease entities, such as GCA, rheumatoid arthritis, polymyositis, chronic infection, or malignant neoplasm, must be ruled out.
PMR often coexists with GCA. In some series, 10-15% of patients with pure PMR had associated GCA based on temporal artery biopsy findings. Conversely, 50-70% of patients with GCA had associated PMR. No difference is evident in the degree of ESR elevation, the presence of minor visual symptoms, sex, age, or the duration of symptoms between patients with PMR and GCA and those with PMR only; furthermore, approximately 10% of patients with PMR and localized temporal artery signs have negative biopsy findings.
Other disorders
Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis) and polyarteritis nodosa may occasionally affect the temporal artery and cause clinical findings similar to GCA. Other vasculitides, such as systemic lupus erythematosus (SLE), rheumatoid arthritis, and Takayasu arteritis, may have similar signs and symptoms.
GCA and Takayasu arteritis have overlapping symptoms in varying frequencies, and when GCA involves larger vessels, the lesions are indistinguishable from those observed in Takayasu arteritis. Thus, these two disorders may represent a spectrum of presentation rather than distinct disease entities. [84]
Amyloidosis rarely affects temporal arteries. It may cause jaw or arm claudication. [85]
Other problems to be considered include the following:
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Dental conditions
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Sinus disease
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Carotid disease and stroke
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Dissection
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Connective tissue disease
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Systemic infections
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Neoplasms
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Arteriosclerotic vascular disease
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Arteriovenous fistulas
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Other forms of vasculitis
Differential Diagnoses
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Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis)
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Non-arteritic ischemic optic neuropathy
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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.
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Lumbar angiogram showing stenosis and occlusion of femoral artery branches due to vasculitis.
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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.
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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).
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Anterior ischemic optic neuropathy. Image courtesy of Richard Kho, MD, Q.C. Eye Center, Quezon City, Philippines.
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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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Central retinal artery occlusion (CRAO).
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Prominent temporal artery is visible on the temple of a 76-year-old woman with temporal arteritis. Courtesy of ScienceSource (https://www.sciencesource.com/).
Tables
Mythili Seetharaman, MD Consultant Rheumatologist, Orthopedic Associates of Allentown, Affiliated with St Luke's University Hospital
Mythili Seetharaman, MD is a member of the following medical societies: American College of Rheumatology, American Medical Association, Pennsylvania Rheumatology Society
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; Past President, 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: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: QualDerm Partners; Novascan<br/>Have a 5% or greater equity interest in: QualDerm Partners - North Carolina.
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.
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, The Scientific Research Honor Society
Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Aldeyra Therapeutics (Lexington, MA); Bausch & Lomb Surgical, Inc (Rancho Cucamonga, CA); Eyegate Pharma (Waltham, MA); Novartis (Cambridge, MA); pSivida (Watertown, MA); Xoma (Berkeley, CA); Allakos (Redwood City, CA)<br/>Serve(d) as a speaker or a member of a speakers bureau for: Alcon (Geneva, Switzerland); Allergan (Dublin, Ireland); Mallinckrodt (Staines-upon-Thames, United Kingdom)<br/>Received research grant from: Alcon; Aldeyra Therapeutics; Allakos Pharmaceuticals; Allergan; Bausch & Lomb; Clearside Biomedical; Dompé pharmaceutical; Eyegate Pharma; Mallinckrodt pharmaceuticals; Novartis; pSivida; Santen; Aciont<br/>Stock for: Eyegate Pharma.
Manolette R Roque, MD, MBA, FPAO Section Chief, Cataract and Refractive Surgery, Department of Ophthalmology, Asian Hospital and Medical Center; Section Chief, Ocular Immunology and Uveitis, International Eye Institute, St Luke's Medical Center Global City
Manolette R Roque, MD, MBA, FPAO is a member of the following medical societies: American Academy of Ophthalmology, American Uveitis Society, European Society of Cataract and Refractive Surgery, International Ocular Inflammation Society, Philippine Academy of Ophthalmology, Philippine College of Surgeons, Philippine Medical Association, Philippine Ocular Inflammation Society, Philippine Society of Cataract and Refractive Surgery, University of the Philippines Medical Alumni Society
Disclosure: Nothing to disclose.
Edsel B Ing, MD, PhD, MBA, MEd, MPH, MA, FRCSC Professor, Department of Ophthalmology and Vision Sciences, Sunnybrook Hospital, University of Toronto Faculty of Medicine; Incoming Chair of Ophthalmology, University of Alberta Faculty of Medicine and Dentistry, Canada
Edsel B Ing, MD, PhD, MBA, MEd, MPH, MA, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Medical Association, Canadian Ophthalmological Society, Canadian Society of Oculoplastic Surgery, Chinese Canadian Medical Society, European Society of Ophthalmic Plastic and Reconstructive Surgery, North American Neuro-Ophthalmology Society, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Statistical Society of Canada
Disclosure: Nothing to disclose.
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.
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
Disclosure: Nothing to disclose.
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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Sections Giant Cell Arteritis (Temporal Arteritis)
- Overview
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