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Giant-Cell Arteritis and Polymyalgia Rheumatica
Giant-Cell Arteritis and Polymyalgia RheumaticaGiant-Cell Arteritis and Polymyalgia Rheumatica
Posted by Sara Fazio • July 4th, 2014
Both giant-cell arteritis and polymyalgia rheumatica are immune-mediated diseases that are treated with glucocorticoids, with higher doses used for giant-cell arteritis. In our latest Clinical Practice article, prompt initiation of high doses and a biopsy are recommended when ischemic optic neuropathy is suspected.
Giant-cell arteritis is an inflammatory vasculopathy that typically occurs in medium and large arteries with well-developed wall layers and adventitial vasa vasorum. The vascular beds that are usually affected include the external carotid branches (e.g., temporal and occipital arteries), the ophthalmic, vertebral, distal subclavian, and axillary arteries, and the thoracic aorta. Polymyalgia rheumatica causes aching and stiffness in selected muscle groups, predominantly in the neck, shoulders, upper arms, and pelvic girdle. Symptoms are most pronounced in the morning.
Clinical Pearls
• What is the epidemiology of giant-cell arteritis and polymyalgia rheumatica, and how often do the diagnoses overlap?
Giant-cell arteritis and polymyalgia rheumatica have multiple risk factors and pathogenic abnormalities in common. Approximately 50% of patients with giant-cell arteritis present with polymyalgia rheumatica before, at the time of, or after the diagnosis of vasculitis. Symptoms of polymyalgia rheumatica often appear when the therapy for giant-cell arteritis is being tapered. Both giant-cell arteritis and polymyalgia rheumatica are diseases that affect the elderly, with a peak incidence at the age of 70 to 80 years; age (50 years or older) is considered a criterion for the diagnosis. Women account for 65 to 75% of patients. Polymyalgia rheumatica occurs at a frequency that is 3 to 10 times that of giant-cell arteritis.
• What are laboratory results in patients with giant-cell arteritis and polymyalgia rheumatica?
Marked elevations in the erythrocyte sedimentation rate (ESR) and the level of C-reactive protein (CRP) are common in giant-cell arteritis and polymyalgia rheumatica, as are the presence of thrombocytosis and anemia. In a cohort of 764 patients with suspected giant-cell arteritis who underwent biopsy, with the diagnosis confirmed in 177 patients, the sensitivity of an elevated ESR was 84% and that of an elevated CRP level was 86%; the specificity of these markers was low, however, at 30%. Only 4% of patients with confirmed giant-cell arteritis had both a normal ESR and a normal CRP level at the time of diagnosis. Assessment of inflammatory markers is helpful during diagnostic evaluation and long-term monitoring, but elevated levels of these markers should not be the only indication for immunosuppressive therapy. No highly specific biomarkers for giant-cell arteritis and polymyalgia rheumatica have been validated.
Morning Report Questions
Q: What is the standard for diagnosis of suspected giant-cell arteritis?
A: In cases of suspected giant-cell arteritis, histologic verification of vasculitis should be sought by means of a temporal-artery biopsy with assessment of a vascular segment that is 1.5 to 2.0 cm in length. Histologic analysis is the standard for diagnosis; it can detect small inflammatory infiltrates and can also distinguish giant-cell arteritis from non-giant-cell arteritis arteritides (e.g., ANCA-associated vasculitis). High-field-strength MRI may emerge as a method that is sensitive to the detection of temporal-artery inflammation, but neither ultrasonography nor MRI has yet replaced temporal-artery biopsy, which is highly sensitive for even minor inflammatory changes.
Q: What is the optimal treatment for giant-cell arteritis and polymyalgia rheumatica?
A: Giant-cell arteritis and polymyalgia rheumatica are responsive to glucocorticoids. Most treatment recommendations are based on clinical experience rather than the results of randomized, controlled trials. Therapy for giant-cell arteritis is initiated with prednisone at a dose of 1 mg per kilogram of body weight per day. Given the risk of irreversible ischemic complications, new-onset clinical manifestations of disease indicating an unstable supply of blood to the eyes or the central nervous system (e.g., arteritic optic neuropathy) are typically managed with intravenous pulse therapy (e.g., 1000 mg of methylprednisolone per day for 3 consecutive days) to optimize immunosuppression and suppress tissue edema. The doses of glucocorticoids used to treat polymyalgia rheumatica are much lower than those used for the treatment of giant-cell arteritis. In the majority of patients, a dose of 15 to 20 mg of prednisone per day is sufficient to control myalgia. No glucocorticoid-sparing agents have been approved for the treatment of giant-cell arteritis or polymyalgia rheumatica.
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Tags: C-reactive protein, CRP, erythrocyte sedimentation rate, ESR, Giant-Cell Arteritis, inflammatory vasculopathy, Polymyalgia Rheumatica
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Posted by Sara Fazio • July 4th, 2014
Both giant-cell arteritis and polymyalgia rheumatica are immune-mediated diseases that are treated with glucocorticoids, with higher doses used for giant-cell arteritis. In our latest Clinical Practice article, prompt initiation of high doses and a biopsy are recommended when ischemic optic neuropathy is suspected.
Giant-cell arteritis is an inflammatory vasculopathy that typically occurs in medium and large arteries with well-developed wall layers and adventitial vasa vasorum. The vascular beds that are usually affected include the external carotid branches (e.g., temporal and occipital arteries), the ophthalmic, vertebral, distal subclavian, and axillary arteries, and the thoracic aorta. Polymyalgia rheumatica causes aching and stiffness in selected muscle groups, predominantly in the neck, shoulders, upper arms, and pelvic girdle. Symptoms are most pronounced in the morning.
Clinical Pearls
• What is the epidemiology of giant-cell arteritis and polymyalgia rheumatica, and how often do the diagnoses overlap?
Giant-cell arteritis and polymyalgia rheumatica have multiple risk factors and pathogenic abnormalities in common. Approximately 50% of patients with giant-cell arteritis present with polymyalgia rheumatica before, at the time of, or after the diagnosis of vasculitis. Symptoms of polymyalgia rheumatica often appear when the therapy for giant-cell arteritis is being tapered. Both giant-cell arteritis and polymyalgia rheumatica are diseases that affect the elderly, with a peak incidence at the age of 70 to 80 years; age (50 years or older) is considered a criterion for the diagnosis. Women account for 65 to 75% of patients. Polymyalgia rheumatica occurs at a frequency that is 3 to 10 times that of giant-cell arteritis.
• What are laboratory results in patients with giant-cell arteritis and polymyalgia rheumatica?
Marked elevations in the erythrocyte sedimentation rate (ESR) and the level of C-reactive protein (CRP) are common in giant-cell arteritis and polymyalgia rheumatica, as are the presence of thrombocytosis and anemia. In a cohort of 764 patients with suspected giant-cell arteritis who underwent biopsy, with the diagnosis confirmed in 177 patients, the sensitivity of an elevated ESR was 84% and that of an elevated CRP level was 86%; the specificity of these markers was low, however, at 30%. Only 4% of patients with confirmed giant-cell arteritis had both a normal ESR and a normal CRP level at the time of diagnosis. Assessment of inflammatory markers is helpful during diagnostic evaluation and long-term monitoring, but elevated levels of these markers should not be the only indication for immunosuppressive therapy. No highly specific biomarkers for giant-cell arteritis and polymyalgia rheumatica have been validated.
Morning Report Questions
Q: What is the standard for diagnosis of suspected giant-cell arteritis?
A: In cases of suspected giant-cell arteritis, histologic verification of vasculitis should be sought by means of a temporal-artery biopsy with assessment of a vascular segment that is 1.5 to 2.0 cm in length. Histologic analysis is the standard for diagnosis; it can detect small inflammatory infiltrates and can also distinguish giant-cell arteritis from non-giant-cell arteritis arteritides (e.g., ANCA-associated vasculitis). High-field-strength MRI may emerge as a method that is sensitive to the detection of temporal-artery inflammation, but neither ultrasonography nor MRI has yet replaced temporal-artery biopsy, which is highly sensitive for even minor inflammatory changes.
Q: What is the optimal treatment for giant-cell arteritis and polymyalgia rheumatica?
A: Giant-cell arteritis and polymyalgia rheumatica are responsive to glucocorticoids. Most treatment recommendations are based on clinical experience rather than the results of randomized, controlled trials. Therapy for giant-cell arteritis is initiated with prednisone at a dose of 1 mg per kilogram of body weight per day. Given the risk of irreversible ischemic complications, new-onset clinical manifestations of disease indicating an unstable supply of blood to the eyes or the central nervous system (e.g., arteritic optic neuropathy) are typically managed with intravenous pulse therapy (e.g., 1000 mg of methylprednisolone per day for 3 consecutive days) to optimize immunosuppression and suppress tissue edema. The doses of glucocorticoids used to treat polymyalgia rheumatica are much lower than those used for the treatment of giant-cell arteritis. In the majority of patients, a dose of 15 to 20 mg of prednisone per day is sufficient to control myalgia. No glucocorticoid-sparing agents have been approved for the treatment of giant-cell arteritis or polymyalgia rheumatica.
ShareThis
Tags: C-reactive protein, CRP, erythrocyte sedimentation rate, ESR, Giant-Cell Arteritis, inflammatory vasculopathy, Polymyalgia Rheumatica
Posted in Physicians-In-Training | Permalink | No Comments
This entry was posted on Friday, July 4th, 2014 at 11:00 am and is filed under Physicians-In-Training. You can follow any responses to this entry through the RSS 2.0 feed. You can leave a response, or trackback from your own site.
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NEJM in the News
The Boston Globe referencedthe 2009 NEJM Perspective article, “Ounces of Prevention — The Public Policy Case for Taxes on Sugared Beverages,” in a story about Boston’s “Sugar Smarts” campaign, which health officials hope will curb city residents’ consumption of sugary beverages. (July 1)
The New York Times referenced the 1977 NEJM Sounding Board, “The Frankenstein Factor,” in a story about three-parent IVF. (June 29)
The AP referenced the January 2013 NEJM Original Article, “Duodenal Infusion of Donor Feces for Recurrent Clostridium difficile,” in a story about how the FDA is regulating fecal transplantation treatment as an experimental drug. (June 26)
A New Yorker contributor referenced the 1972 NEJM Original Article, “Prolonged Extracorporeal Oxygenation for Acute Post-Traumatic Respiratory Failure (Shock-Lung Syndrome),” in a blog post entitled, “My New Iron Lung.” (June 26)
The AP referenced the January 2013 NEJM Original Article, “Duodenal Infusion of Donor Feces for Recurrent Clostridium difficile,” in a story about how the FDA is regulating fecal transplantation treatment as an experimental drug. (June 26)
A New Yorker contributor referenced the 1970 NEJM Original Article, “Prolonged Extracorporeal Oxygenation for Acute Post-Traumatic Respiratory Failure (Shock-Lung Syndrome),” in a blog post entitled, “My New Iron Lung.” (June 26)
@NEJM
@NEJMGroup
NEJM on Youtube
NEJM on Facebook
CareerCenter
PHYSICIAN JOBS
July 6, 2014
Infectious Disease
Infectious Disease Specialist
MAINE
Chiefs/Directors/Dept. Heads
DIRECTOR OF THE ADVANCED LUNG DISEASES PROGRAM (ALDP)
CONNECTICUT
Nephrology
SEEKING EIGHTH NEPHROLOGIST
NEW YORK
Geriatrics
GERIATRICS/PALLIATIVE MEDICINE
MASSACHUSETTS
Gastroenterology
MAINE
MAINE
Family Medicine
FAMILY MEDICINE, BOSTON AREA
MASSACHUSETTS
nejmcareercenter.org
Meet Us
NEJM Group will be exhibiting at the upcoming conferences below.
European Society of Cardiology (ESC): August 31- September 4, 2013 in Amsterdam—booth #A-464
ID Week: October 3-5, 2013 in San Francisco, CA—booth #108
Association of American Medical Colleges (AAMC): November 2-4, 2013, in Philadelphia, PA—booth #409
American Heart Association (AHA): November 17-19, 2013 in Dallas, TX—booth #1024
American Society of Hematology (ASH): December 7-10, 2013 in New Orleans, LA—booth #2506
Tags
abdominal pain AIDS anemia asthma breast cancer cardiac arrest Case Records Case Records of the Massachusetts General Hospital chemotherapy chronic kidney disease Clinical Decisions Clinical Practice Clinical Problem-Solving COPD CPR Critical Care Challenge current concepts Diabetes dyspnea fever global health glucocorticoids hepatitis C HIV hypertension Interactive Medical Cases Massachusetts General Hospital myocardial infarction NEJM nejm 200th anniversary NEJM 200th Anniversary website obesity pneumonia pregnancy prostate cancer ribavirin sepsis septic shock stroke thrombocytopenia tuberculosis type 2 diabetes Venous thromboembolism video weight loss
Blogroll
CardioExchange
Clinical Conversations: A Journal Watch Podcast
Gutcheck on Gastroenterology: A Journal Watch Blog
HIV and ID Observations: A Journal Watch Blog
Insights on Residency: A Journal Watch Blog
NEJM RSS Feeds
Physician's First Watch
Archives
Follow NEJM on Twitter Be a Fan of NEJM on Facebook
- See more at: http://blogs.nejm.org/now/index.php/giant-cell-arteritis-and-polymyalgia-rheumatica/2014/07/04/#sthash.Ukt8OkGs.dpuf
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