Edit concept Question Editor Create issue ticket

Non-Cranial Giant Cell Arteritis

Giant Cell Arteritis


Presentation

  • Fatigue and malaise are often present and may be severe.[mdedge.com]
  • Presentation [ 4 ] GCA typically presents with recent onset of temporal headache, myalgia, malaise or fever. Typical features may be absent or subtle.[patient.info]
  • While GCA has a classic presentation occurring after the age of 50, atypical presentations (eg, fever of unknown origin, cough, low or normal erythrocyte sedimentation rate) may lead to a delay in diagnosis.[mayoclinic.pure.elsevier.com]
  • Key points Given the wide spectrum of presentations of giant cell arteritis, physicians need to be equally familiar with both typical and atypical presentations. The presenting manifestation of giant cell arteritis may be stroke.[cmaj.ca]
  • GCA was not diagnosed until she presented to hospital with sudden loss of vision in her left eye. An 85-year-old man on oral steroid treatment for PMR presented to A&E with sudden loss of vision in his right eye.[themdu.com]
Fever
  • While GCA has a classic presentation occurring after the age of 50, atypical presentations (eg, fever of unknown origin, cough, low or normal erythrocyte sedimentation rate) may lead to a delay in diagnosis.[mayoclinic.pure.elsevier.com]
  • Patients may present with systemic symptoms such as fever (usually low-grade), fatigue, malaise, unexplained weight loss, and sweats. Some patients are initially diagnosed as having fever of unknown origin.[merckmanuals.com]
  • Constitutional symptoms: fatigue, general malaise, fever, anorexia, weight loss, and night sweats Fever of Unknown Origin Rarely tongue pain, ulceration and necrosis, see Case Example Temporal Arteritis with Tongue Necrosis Review of Systems Polymyalgia[medicine.uiowa.edu]
  • The most common symptoms of GCA include persistent, throbbing headaches, tenderness of the temples and scalp, jaw pain, fever, joint pain, and vision problems. Early treatment is vital to prevent serious complications such as blindness or stroke.[vasculitisfoundation.org]
  • Early symptoms of giant cell arteritis resemble the flu: fatigue, loss of appetite, and fever.[medlineplus.gov]
Weight Loss
  • He had previously experienced several weeks of temporal headache and weight loss. Abnormal findings on neurologic examination included upbeat nystagmus, gaze palsy when looking to the right and gait ataxia.[cmaj.ca]
  • ., fever, weight loss, night sweats, fatigue, and malaise ), and new headaches.[amboss.com]
  • There are many possible clinical features that present in a subacute fashion 10 : a headache (most common) with or without scalp tenderness systemic symptoms (e.g. fever, fatigue, weight loss) jaw claudication transient vision loss ( amaurosis fugax )[radiopaedia.org]
  • loss Otorhinologic – jaw claudication Ophthalmologic – transient visual changes, diplopia, visual field cuts, permanent vision loss Cardiovascular – arm claudication, thoracic artery aneurysm Neurologic – headache, temporal artery pain, mononeuropathy[arupconsult.com]
Fatigue
  • Early symptoms of giant cell arteritis resemble the flu: fatigue, loss of appetite, and fever.[medlineplus.gov]
  • ., fever, weight loss, night sweats, fatigue, and malaise ), and new headaches.[amboss.com]
  • Fatigue and malaise are often present and may be severe.[mdedge.com]
Malaise
  • A 71-year-old woman with fever, general malaise and weight loss was found to have markedly elevated ESR and CRP. She denied any headache or scalp tenderness.[themdu.com]
  • ., fever, weight loss, night sweats, fatigue, and malaise ), and new headaches.[amboss.com]
  • Fatigue and malaise are often present and may be severe.[mdedge.com]
  • […] ischemic disease – most common in the optic nerve Can lead to vision loss, caused primarily by occlusive vasculopathy Granulomas form in arterial media Often associated with polymyalgia rheumatica Clinical Presentation Constitutional – fever, fatigue, malaise[arupconsult.com]
  • Fever, weight loss, malaise, and fatigue are also common. ESR and C-reactive protein are typically elevated. Diagnosis is clinical and confirmed by temporal artery biopsy.[merckmanuals.com]
Anemia
  • ., parvovirus B19 ) Association with polymyalgia rheumatica (PMR) : 40–50% of patients with giant cell arteritis also have PMR References: [1] [#1200 Clinical features Constitutional symptoms Fever, weight loss, night sweats Symptoms of anemia : fatigue[amboss.com]
  • Elevated inflammatory marker(s) Criteria for Diagnosis Histologic diagnosis – temporal artery biopsy showing segmental inflammation Laboratory Testing Nonspecific testing – helpful in excluding other diagnoses CBC – thrombocytosis, normochromic normocytic anemia[arupconsult.com]
  • There may also be fatigue, joint pain, and anemia. Making the Diagnosis GCA is diagnosed by a thorough physical examination, blood tests, and sometimes by taking a biopsy (tissue sample) of the affected temporal arteries.[medbroadcast.com]
  • In most patients, ESR and C-reactive protein are elevated; anemia of chronic disease is common. Occasionally, platelets are elevated, and serum albumin and total protein, if measured, are low.[merckmanuals.com]
  • Anemia, usually caused by anemia of inflammation, is common in affected patients. Less common, elevations in liver transaminases or a low serum albumin may occur.[rheumatologyadvisor.com]
Loss of Appetite
  • Early symptoms of giant cell arteritis resemble the flu: fatigue, loss of appetite, and fever.[medlineplus.gov]
  • Symptoms of fatigue, loss of appetite, weight loss and fever are often found. Headache, with pain and tenderness over the temples, is a prominent feature of this disease due to inflammation of the temporal arteries.[vasculitis.org.uk]
  • These may include: Jaw pain - particularly when chewing Hearing problems Vision problems eg: blurred or double vision, loss of visual field Aching muscles about the neck and shoulders Loss of appetite Fatigue Depression Other symptoms can include tenderness[southerncross.co.nz]
Vascular Disease
  • ) disease NOS systemic autoimmune disease systemic collagen (vascular) disease Type 1 Excludes autoimmune disease, single organ or single cell-type -code to relevant condition category Systemic connective tissue disorders Approximate Synonyms Giant cell[icd10data.com]
  • Temporal arteritis is a chronic vascular disease of unknown origin occurring in the elderly, characterized by granulomatous inflammation in the wall of medium-size and large arteries (1).[gpnotebook.co.uk]
  • Occasionally, symptoms relate to intermittent or persistent brain ischaemia, due to a subclavian steal syndrome (SSS), narrowing of other aortic arch vessels or intracerebral vascular disease.[patient.info]
  • disease of unknown origin, occurring in the elderly, characterized by severe headache, fever, and accumulation of giant cells in the walls of medium-sized arteries, especially the temporal arteries.[medical-dictionary.thefreedictionary.com]
  • Coronary Artery Disease or Peripheral Vascular Disease No change in standard management E.[clinicaladvisor.com]
Vascular Disease
  • ) disease NOS systemic autoimmune disease systemic collagen (vascular) disease Type 1 Excludes autoimmune disease, single organ or single cell-type -code to relevant condition category Systemic connective tissue disorders Approximate Synonyms Giant cell[icd10data.com]
  • Temporal arteritis is a chronic vascular disease of unknown origin occurring in the elderly, characterized by granulomatous inflammation in the wall of medium-size and large arteries (1).[gpnotebook.co.uk]
  • Occasionally, symptoms relate to intermittent or persistent brain ischaemia, due to a subclavian steal syndrome (SSS), narrowing of other aortic arch vessels or intracerebral vascular disease.[patient.info]
  • disease of unknown origin, occurring in the elderly, characterized by severe headache, fever, and accumulation of giant cells in the walls of medium-sized arteries, especially the temporal arteries.[medical-dictionary.thefreedictionary.com]
  • Coronary Artery Disease or Peripheral Vascular Disease No change in standard management E.[clinicaladvisor.com]
Chest Pain
  • […] well-known complication of giant cell arteritis, 21.2% of patients with giant cell arteritis and visual symptoms have no other cranial or systemic manifestations. 2 Arteritis may involve the aorta and its main branches, so patients may present with chest[cmaj.ca]
  • Impact of a public campaign on pre-hospital delay in patients reporting chest pain. Heart. 1996;76(2):150–5. View Article PubMed PubMed Central Google Scholar Fry CW, Perrow R, Paul SP. Brain tumours in children: importance of early identification.[bmcmedicine.biomedcentral.com]
Vision Disorder
  • These include internists, who treat a broad range of diseases; rheumatologists, who focus on rheumatic diseases; geriatricians, who treat older people; ophthalmologists, who treat eye and vision disorders; neurologists, who treat headaches and problems[medical-dictionary.thefreedictionary.com]
Myalgia
  • Headache and myalgia are frequently seen, but they are not specific to temporal arteritis. The manifestations of temporal arteritis are variable and often transient.[sharinginhealth.ca]
  • Presentation [ 4 ] GCA typically presents with recent onset of temporal headache, myalgia, malaise or fever. Typical features may be absent or subtle.[patient.info]
  • Classic features of giant cell arteritis include headache, claudication of the jaw and tongue, loss of vision in one eye, fever, myalgia, weight loss, anorexia and fatigue ( Table 1 ). 2 – 4 Diagnostic criteria established by the American College of Rheumatology[cmaj.ca]
  • Distinct tongue numbness and vertigo have also been reported. [38] Myalgias, especially of the proximal muscles, are also associated with GCA.[eyewiki.aao.org]
  • Patients with giant cell arteritis often have a variety of other symptoms, such as malaise, fatigue, low-grade fever, anorexia, weight loss, myalgias or arthralgias. They may also have various visual symptoms, including blurring and scotomas.[aafp.org]
Arthralgia
  • Patients with giant cell arteritis often have a variety of other symptoms, such as malaise, fatigue, low-grade fever, anorexia, weight loss, myalgias or arthralgias. They may also have various visual symptoms, including blurring and scotomas.[aafp.org]
  • In NAION, patients will usually not note any associated systemic symptoms such as headache, jaw claudication, scalp tenderness, weight loss, anorexia, fever, or myalgias/arthralgias.[eyewiki.aao.org]
Facial Pain
  • Less common non-visual manifestations include fever, altered mental state, stroke, angina, myocardial infarction, pericarditis, limb claudication, abdominal pain, facial pain and/or swelling, tongue pain and/or swelling or necrosis, ear pain, cough and[themdu.com]
  • However, the common signs and symptoms of GCA that may be present include: Headache (can be generalized) Scalp tenderness Tiredness Facial pain, jaw pain due to jaw movement Flu-like symptoms with low-grade fever Muscle pain (commonly felt in the neck[dovemed.com]
  • GCA should be suspected in anyone over the age of 50 years with headache, scalp tenderness, transient visual symptoms or unexplained facial pain. Acute severe sight impairment occurs in up to 20% of patients.[patient.info]
  • Pain Polymyalgia Rheumatica Postherpetic Neuralgia Retinal Artery Occlusion (RAO) Retinal Vein Occlusion (RVO) Rheumatoid Arthritis Transient Ischemic Attack Hoffman GS.[emedicine.staging.medscape.com]
Headache
  • JF - Headache SN - 0017-8748 IS - 8 ER -[mayoclinic.pure.elsevier.com]
  • Resources Organizations National Headache Foundation. 428 W. St.[medical-dictionary.thefreedictionary.com]
  • Just a Headache? Headache is the most common symptom of Giant Cell Arteritis.[thedoctorwillseeyounow.com]
  • She had also consulted her GP about recent onset occipital headaches. GCA was not diagnosed until she presented to hospital with sudden loss of vision in her left eye.[themdu.com]
  • Other useful predictive features include: Temporal headache. Scalp tenderness. ESR significantly elevated. Anaemia. Symptoms The history is usually short and the most common symptoms include: Headache: present in more than 85% of patients.[patient.info]
Peripheral Neuropathy
  • neuropathy Claudication of extremities: upper and/or lower, uni/bilateral Aortitis or aortic aneurysm, usually clinically silent or result in fever of unknown origin; rarely may manifest in aortic dissection Normochromic, normocytic anemia Speech and[medicine.uiowa.edu]
  • Neurological manifestations can occur in one-third of patients with giant cell arteritis, most commonly cranial nerve palsies, peripheral neuropathies and, rarely, strokes in the region of the carotid or vertebrobasilar artery. 5 Perform auscultation[bpac.org.nz]
  • Less often, neurological symptoms, such as peripheral neuropathies [39] [40], strokes [41], scalp necrosis [42] and dementia [43] [44] have all been attributed to GCA.[eyewiki.aao.org]
  • Mild EMG abnormalities in an elderly patient that suggest a mild peripheral neuropathy are generally unrelated to PMR or vasculitis.[emedicine.staging.medscape.com]

Workup

  • […] with acute third nerve palsy, physicians must be alert to elements in the history and physical examination that point to a serious neurologic or medical condition, such as intracranial aneurysm or GCA, respectively. 14 Failure to undertake an adequate workup[mdmag.com]
Thrombocytosis
  • […] changes or new onset localized headache Elevated inflammatory marker(s) Criteria for Diagnosis Histologic diagnosis – temporal artery biopsy showing segmental inflammation Laboratory Testing Nonspecific testing – helpful in excluding other diagnoses CBC – thrombocytosis[arupconsult.com]
  • […] can lead to Rouleaux formation of RBCs CRP Mild thrombocytosis Normochromic anemia No autoantibodies Temporal artery biopsy (gold stand ard) : mandatory in all patients Extended biopsy sample ( 1 cm, ideally 2 cm ) Specificity : 100% Duplex ultrasonography[amboss.com]
  • Anaemia, thrombocytosis, leucocytosis and elevated serum alkaline phosphatase are common in untreated GCA.[themdu.com]
  • Laboratory tests typically show anemia, leukocytosis, and thrombocytosis. The erythrocyte sedimentation rate and the C-reactive protein level are usually very high.[mdedge.com]

Treatment

  • If GCA is a possibility, treatment should start right away.[medicalnewstoday.com]
  • This article reviews the sequelae, diagnosis and treatment of GCA.[reviewofoptometry.com]
  • If it is suspected that your sight loss is due to GCA, you may be admitted to hospital for treatment or given treatment to use at home straight away.[rnib.org.uk]
  • In the early period following initiation of steroid treatment, patients with GCA need to be regularly reviewed to ensure that treatment is adequate.[themdu.com]
  • Diagnosis and treatment Treatment for giant cell arteritis will usually begin as soon as possible. This may mean that treatment begins before a diagnosis is confirmed.[your.md]

Prognosis

  • The prognosis is good with correct and early diagnosis and treatment.[vasculitis.org.uk]
  • This is important in ensuring a favorable prognosis In most cases, corticosteroid treatment can be stopped after 1-2 years.[dovemed.com]
  • Links: epidemiology clinical features investigations steroids in treatment of temporal arteritis prognosis[gpnotebook.co.uk]
  • While some anecdotal cases report significant visual improvement with prompt treatment, the prognosis for visual recovery from AAION is generally poor.[reviewofoptometry.com]
  • […] shows mural inflammation very well 2,4 mean wall thickness increased in the affected region luminal diameter correspondingly decreased in the affected region reported approximate sensitivity and specificity is 80% and 97%, respectively 2 Treatment and prognosis[radiopaedia.org]

Etiology

  • Etiology Unknown; possible contributing factors are: Genetic predisposition (e.g., human leukocyte antigen HLA -DR4 ) Viral infections (e.g., parvovirus B19 ) Association with polymyalgia rheumatica (PMR) : 40–50% of patients with giant cell arteritis[amboss.com]
  • […] should be performed to measure cerebrospinal fluid (CSF) opening pressure (normal, 2 O) and to examine CSF composition for signs of meningitis and subarachnoid hemorrhage. 10 Although the management of increased ICP should be directed at the underlying etiology[mdmag.com]
  • (Etiology) The exact cause of Giant Cell Arteritis is largely unknown However, it is known that the risk of developing GCA increases with an advancing age.[dovemed.com]
  • […] return to top Causes and Risk Factors The etiology and pathogenesis of giant cell arteritis remain unclear. Amongst the possible causes is genetic susceptibility since the disease is more common in people with HLA-DR4 or HLA-DRB1.[sharinginhealth.ca]
  • Etiology The underlying etiology of GCA is is complex and has been widely researched, yet is still not well understood.[eyewiki.aao.org]

Epidemiology

  • References: [1] [2] Epidemiological data refers to the US, unless otherwise specified.[amboss.com]
  • Links: epidemiology clinical features investigations steroids in treatment of temporal arteritis prognosis[gpnotebook.co.uk]
  • An epidemiologic and histopathologic analysis. Arthritis Rheum 1994 ; 37 : 1007 –12. Salvarani C , Machioni P, Zizzi F, et al . Epidemiologic and immunogenetic aspects of polymyalgia rheumatica and giant cell arteritis in Northern Italy.[pmj.bmj.com]
  • Giant Cell Arteritis Epidemiology and Treatment. Drugs Aging. 1994;4:135-44. Kyle V et al. The clinical and laboratory course of polymyalgia rheumatica/giant cell arteritis after the first two months of treatment. Ann Rheum Dis. 1993;52:847-50.[rarediseases.org]
  • […] myopathy Osteoarthritis Other vasculitic disease Granulomatosis with polyangiitis (formerly Wegener granulomatosis) Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) Microscopic polyangiitis Polyarteritis nodosa Background Epidemiology[arupconsult.com]
Sex distribution
Age distribution

Pathophysiology

  • .; most common primary vasculitis in older patients Age – peak age 70-80 years Rare – Sex – M Ethnicity – more common in Caucasians than African or Asian Americans; rare in Hispanic population (Weyand, 2014) Pathophysiology Medium- and large-vessel vasculitis[arupconsult.com]
  • The pathophysiology of GCA is a result of luminal occlusion of inflamed arterial walls, and may affect a number of vessels supplying the eye.[journals.lww.com]
  • […] female:male ratio is 2:1) Non-black Scandinavian descent Having polymyalgia rheumatica (10 to 20% of patients with polymyalgia rheumatica develop temporal arteritis) Heavy smoking and atherosclerosis, a risk factor for women, but not men return to top Pathophysiology[sharinginhealth.ca]
  • Giant cell arteritis: a review of classification, pathophysiology, geoepidemiology and treatment. Autoimmun Rev. 2012 May;11(6-7):A544-54. Epub 2012 Jan 21. Kesten F, Aschwanden M, Gubser P, Glatz K, Daikeler T, Hess C.[medicine.uiowa.edu]
  • The Heart in Rheumatic, Autoimmune and Inflammatory Diseases: Pathophysiology, Clinical Aspects and Therapeutic Approaches. Academic Press. p. 367. ISBN 9780128032688.[en.wikipedia.org]

Prevention

  • Prompt treatment of giant cell arteritis is critical in order to prevent permanent tissue damage and loss of vision. Corticosteroid medications usually relieve symptoms of giant cell arteritis and may prevent loss of vision.[blood-test.biz]
  • Low-dose aspirin (81 to 100 mg orally once a day) may help prevent ischemic events and should be prescribed for all patients unless contraindicated. 1.[merckmanuals.com]
  • Treatment and Prevention Giant cell arteritis (GCA) is treatable, and complications can be avoided when treatment is started early enough.[rexall.ca]
  • Prompt treatment of GCA with high dose steroids is essential to prevent permanent loss of vision.[sciencedaily.com]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!