Temporal arteritis: Difference between revisions

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{{Infobox_Disease |
  Name          = Temporal arteritis |
  Image          = Gray508.png |
  Caption        = The arteries of the face and scalp. |
  DiseasesDB    = 12938 |
  ICD10          = {{ICD10|M|31|5|m|30}} |
  ICD9          = {{ICD9|446.5}} |
  ICDO          = |
  OMIM          = 187360 |
  MedlinePlus    = |
  MeshID        = D013700 |
}}
{{Temporal arteritis}}
{{Temporal arteritis}}


{{CMG}} '''Associate Editor-In-Chief:''' {{CZ}}
'''For patient information, click [[Temporal arteritis (patient information)|here]]'''


{{SK}} GCA; giant cell arteritis; cranial arteritis; Horton's disease; Horton disease; Horton's arteritis; Horton syndrome; Horton's syndrome; granulomatous arteritis; polymyalgia arteritica
{{CMG}}; {{AE}} {{HQ}}


==Overview==
{{SK}} GCA; giant cell arteritis; cranial arteritis; Horton's disease; Horton disease; Horton's arteritis; Horton syndrome; Horton's syndrome; Horton-Magath-Brown syndrome granulomatous arteritis; polymyalgia arteritica
'''Temporal arteritis''', also called '''giant cell arteritis''' ('''GCA''') is an [[inflammation|inflammatory]] disease of [[blood vessel]]s (most commonly large and medium [[artery|arteries]] of the head). It is therefore a form of [[vasculitis]]. The name comes from the most frequently involved vessel ([[temporal artery]] which branches from the [[external carotid artery]] of the neck). The alternative name (giant cell arteritis) reflects the type of inflammatory cell that is involved (as seen on [[biopsy]]).


The disorder may coexist (in one quarter of cases) with [[polymyalgia rheumatica]] (PMR), which is characterized by sudden onset of pain and stiffness in muscles ([[pelvis]], [[shoulder]]) of the body and seen in the elderly. Other diseases related with temporal arteritis are systemic [[lupus erythematosus]], [[rheumatoid arthritis]] and severe [[infection]]s.
==[[Temporal arteritis overview|Overview]]==


This diagnosis should be considered in any patient over the age of 50 with the new onset of [[headache]], particularly is the [[erythrocyte sedimentation rate]] is elevated.
==[[Temporal arteritis historical perspective|Historical Perspective]]==


Prompt treatment with steroids is a medical emergency to reduce the risk of [[blindness]].
==[[Temporal arteritis classification|Classification]]==


==History==
==[[Temporal arteritis pathophysiology|Pathophysiology]]==
The disease was first described in 1890 by Hutchinson. The histopathology of the disorder was first described in 1932 by Horton. Visual loss was first reported by Jennings in 1938. Birkhead first described the use of steroids to prevent progression to blindness.


==Epidemiology and Demographics==
==[[Temporal arteritis causes|Causes]]==
It is more common in females than males by a ratio of 3.7:1.  The mean age of onset is about 70 years and is rare at less than 50 years of age. It is more prevalent in caucasians.


Most likely as a result of increase clinical recognition of the syndrome, the incidence of temporal arteritis increased between 1950 and 1975 from 5.1 to 17.4 incident cases per 100,000 population per year in persons aged 50 years and older in Olmsted County, Minnesota. More recently in this population,  the prevalence was even higher at 133 cases per 100,000 in persons aged 50 years and older.
==[[Temporal arteritis differential diagnosis|Differentiating Temporal Arteritis from other Diseases]]==


Because temporal arteritis is a disease of the elderly, the prevalence of the disease is very dependent upon the age of the population. As a result, there is a lower prevalence in countries with a shorter or reduced life expectancy.
==[[Temporal arteritis epidemiology and demographics|Epidemiology and Demographics]]==


There is little data regarding the prevalence of disease outside the US, but given the variation in life expectancy and demographics, the prevalence is thought to vary significantly. The highest reported rates of disease outside the US are in Scandinavia, where the prevalence is 23.3-33.6 per 100,000 people older than 50 years.
==[[Temporal arteritis risk factors|Risk Factors]]==


==Pathophysiology==
==[[Temporal arteritis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
 
The damage to the vasuclature is mediated by an attack on the internal elastica lamina by activated CD4+ [[T helper cell]]s.  This occurs in repsonse to the presentation of an [[antigen]] by [[macrophages]]. The inciting antigen has not been identified.
 
Because the disease involves only arteries with internal elastic lamina, the aortic arch and its branches are often involved. Intracranial arteries do not have internal elastic lamina and are not involved. The distribution of involved arteries are as follows:
 
==='''''Commonly involved sites:'''''===
 
'''Cervicocephalic arteries''': [[carotid artery]] and [[vertebral artery]].  The [[vertebral artery]] is involved as frequently as the temporal artery in fatal cases. Involvement of the [[basilar artery]] is rare.
 
'''Intraorbital branches''': Posterior ciliary artery and [[ophthalmic artery]].
 
'''External common, external, and internal carotid artery involvement:''' It is less common for proximal intracranial arteries to be involved.
 
'''External vertebral arteritis:''' It is less common though for the disease to extend more than 5 mm beyond the dural penetration.
 
'''Subclavian, axially and proximal brachial artery:''' There can be typical vasculitic lesions with long, smooth, lesions with tapered occlusions.
 
'''Coronary arteries''': for a full discussion of the involvement of the heart in this disorder see the chapter on [[The Heart in Temporal Arteritis / Giant Cell Arteritis]]
 
==='''''Less commonly involved sites:'''''===
 
'''Descending aorta:''' Mesenteric, iliac, femoral and renal arteries are less often involved. In these cases there can be [[mesenteric ischemia]], [[renal infarction]], and ischemic [[mononeuropathy]] can occur.
 
'''Pulmonary artery'''
 
==Diganosis==
===Differential Diagnosis of Disorders that Temporal Arteritis Should be Distinguished From===
[[Cluster Headache]]
 
[[Migraine Headache]]
 
[[Multi-infarct Dementia]]
 
Persistent Idiopathic [[Facial Pain]]
 
[[Polyarteritis Nodosa]]
 
[[Postherpetic Neuralgia]]
 
[[Sinusitis]]
 
[[Trigeminal Neuralgia]]
 
[[Stroke]]
 
[[Wegener granulomatosis]]
 
===Symptoms===
Patients present with:
* [[fever]]
* [[headache]]
* tenderness and sensitivity on the [[scalp]]
* [[jaw]] [[claudication]] (pain in jaw when chewing)
* reduced [[visual acuity]] ([[blurred vision]])
* acute visual loss (sudden [[blindness]])
 
The inflammation may affect blood supply to the [[eye]] and [[blurred vision]] or sudden [[blindness]] may occur. In 76% of cases involving the eye, the [[ophthalmic artery]] is involved causing [[anterior ischemic optic neuropathy]].<ref>{{cite web |author=Hayreh |title=Ocular Manifestations of GCA |url=http://webeye.ophth.uiowa.edu/dept/GCA/04-ocular.htm |date=April 3, 2003 |publisher University of Iowa Health Care |accessdate=2007-10-15}}</ref> Loss of vision in both eyes may occur very abruptly and this disease is therefore a [[medical emergency]].


==Diagnosis==
==Diagnosis==
===Physical exam===
[[Temporal arteritis diagnostic study of choice|Diagnostic Study of Choice]] | [[Temporal arteritis history and symptoms|History and Symptoms]] | [[Temporal arteritis physical examination|Physical Examination]] | [[Temporal arteritis laboratory findings|Laboratory Findings]] | [[Temporal arteritis CT|CT]] | [[Temporal arteritis MRI|MRI]] | [[Temporal arteritis echocardiography or ultrasound|Ultrasound]] | [[Temporal arteritis other imaging findings|Other Imaging Findings]] | [[Temporal arteritis other diagnostic studies|Other Diagnostic Studies]]
Palpation of the head reveals sensitive and thick arteries with or without pulsation.
 
====Skin====
[[Image:temporal_arteritis.jpg|thumb|left|Temporal arteritis. <ref>http://picasaweb.google.com/mcmumbi/USMLEIIImages</ref>]]
{{clr}}
 
===Laboratory tests===
[[Erythrocyte sedimentation rate|Sedimentation rate]] is very high in most of the patients, but may be normal in approximately 20% of cases.
 
===Biopsy===
The [[Gold standard (test)|gold standard]] for diagnosing temporal arteritis is [[biopsy]], which involves removing of a small part of the vessel and examining it [[microscope|microscopically]] for giant cells infiltrating the tissue. Since the [[blood vessel]]s are involved in a patchy pattern, there may be unaffected areas on the vessel and the biopsy might have been taken from these parts. So, a [[false negative|negative result]] does not definitely rule out the diagnosis.
 
===Radiology===
Radiological examination of the temporal artery with [[ultrasound]] yields a [[halo sign]].
Contrast enhanced brain MRI and CT is generally negative in this disorder.


==Treatment==
==Treatment==
[[Corticosteroid]]s must be started as soon as the diagnosis is suspected (even before the diagnosis is confirmed by biopsy). Steroids do not prevent the diagnosis later being confirmed by biopsy, although certain changes in the histology may be observed towards the end of the first week of treatment and are more difficult to identify after a couple of months.<ref name="pmid16987903">{{cite journal |author=Font RL, Prabhakaran VC |title=Histological parameters helpful in recognising steroid-treated temporal arteritis: an analysis of 35 cases |journal=The British journal of ophthalmology |volume=91 |issue=2 |pages=204–9 |year=2007 |pmid=16987903 |doi=10.1136/bjo.2006.101725}}</ref>
[[Temporal arteritis medical therapy|Medical Therapy]] | [[Temporal arteritis surgery|Surgery]] | [[Temporal arteritis primary prevention|Primary Prevention]] | [[Temporal arteritis secondary prevention|Secondary Prevention]] | [[Temporal arteritis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Temporal arteritis future or investigational therapies|Future or Investigational Therapies]]
 
A 3 day course of pulse steroids with 250 mg of IV [[solumedrol]] BID is critical in reducing the risk of permanent [[visual loss]]. If there is progression of visual loss on steroids, IV [[heparin]] can be administered to reduce the risk of thrombotic occlusion.
 
Treatment should not be deferred while waiting on the results of a [[temporal artery]] biopsy.
 
==References==
{{reflist|2}}


==Additional Resources==
==Case Studies==
*[http://www.niams.nih.gov/hi/topics/polymyalgia/ Polymyalgia Rheumatica]
[[Temporal arteritis case study one|Case #1]]


{{Diseases of the musculoskeletal system and connective tissue}}
{{Diseases of the musculoskeletal system and connective tissue}}
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[[Category:Neurology]]
[[Category:Neurology]]
[[Category:Emergency medicine]]
[[Category:Emergency medicine]]
[[Category:Disease]]





Latest revision as of 19:07, 16 April 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Synonyms and keywords: GCA; giant cell arteritis; cranial arteritis; Horton's disease; Horton disease; Horton's arteritis; Horton syndrome; Horton's syndrome; Horton-Magath-Brown syndrome granulomatous arteritis; polymyalgia arteritica

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Temporal Arteritis from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice | History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

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