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| Giant Cell Arteritis (Temporal arteritis) |
| Overview |
 | Rare before 50 years old, mean age of onset 70 year old |
| 10 times more common in over 80 years old as in 50 - 60 years old. |
| Female affected twice as often as men. |
| More common in northern latitudes (15-30/ 100,000 person) as compared to
southern latitudes (less than 2/100,000). |
| More common in Northern European
ancestry. |
| Most frequently involve: Branches of the external carotid artery,
including the posterior ciliary arteries that supply the optic nerve. |
| May involve aorta and its upper extremity branches, vertebral artery.
Lower extremity arteries and mesenteric arteries is rarely affected. Intracranial arteries
are essentially spared. |
|
| Signs and Symptoms |
Clinical Findings in 100
patients: from Calamia KT, Hunder GG: Clinical
manifestations of giant cell arteritis. Clin Rheum Dis 6:389, 1980.
| Weight loss or anorexia |
50 |
| Malaise, fatigue, or weakness |
40 |
| Fever |
42 |
| Polymyalgia rheumatica |
39 |
| Other musculoskeletal pains |
30 |
| Synovitis |
15 |
| Symptoms related to arteries |
83 |
| Headache |
68 |
| Visual symptoms |
| Transient |
16 |
| Fixed |
14 |
| Jaw claudication |
45 |
| Swallowing claudication or dysphagia |
8 |
| Tongue claudication |
6 |
| Limb claudication |
4 |
| Signs related to arteries |
66 |
| Artery tenderness |
27 |
| Decreased temporal artery pulsations |
46 |
| Erythematous, nodular, or swollen scalp
arteries |
23 |
| Large artery bruits |
21 |
| Decreased large artery pulses |
7 |
| Ophthalmologic |
20 |
| Visual loss |
14 |
| Ophthalmoscopic |
18 |
| Extraocular muscle weakness |
2 |
| Raynaud's phenomenon |
3 |
| Central nervous system abnormalities |
15 |
| Sore throat |
9 |
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| Visual loss |
| Highest risk for visual loss is present within the first two months even
on adequate corticosteroid dose. |
| After two months of adequate high dose corticosteroid therapy, visual
loss is very unlikely. |
|
| Lab findings |
| Elevated ESR in 85%. Combination of an elevated ESR with an elevated
C-reactive protein ( >2.45 mg/dl) may help to discriminate GCA from other causes of
elevated ESR |
| Normochromic, normocytic anemia with a low reticulocyte count and
adequate iron stores |
| 25% may have elevated Alkaline phosphatase |
| Tests for antinuclear antibodies and rheumatoid factor are generally
negative |
|
| Diagnostic Criteria: Am College of Rheumatology
1990 |
Sensitivity of 93.5%, specificity of 91.2% when at least
3/5 criteria are present:
| Age of onset >50 years |
| New Headache: new type of localized pain in the head |
| Temporal artery abnormality: Temporal artery tenderness to palpation or
decreased pulsation, unrelated to arteriosclerosis of cervical arteries |
| Elevated ESR > 50 mm/hour by Westergren method |
| Abnormal artery biopsy: artery showing vasculitis characterized by a
predominance of mononuclear cell infiltration or granulomatous inflammation, usually with
multinucleated giant cells. |
|
| Temporal artery biopsy |
| Biopsy within 14 days of initiation of treatment establishes the
diagnosis if positive. |
| Short course of corticosteroid therapy prior to biopsy probably do not
significantly affect the chance of positive biopsy, but may change the histologic
characteristics. |
| Adequate temporal biopsy specimen (usually greater than 2 cm) should be
obtained and examined in its entirety after transverse sectioning is performed and the
tissue stained with hematoxylin and eosin, and elastin stains. |
| The superficial temporal artery is the typical site biopsied. The
dissection can be guided by the use of direct palpation of the artery as it courses
through the temporalis fascia, or by Doppler ultrasound, particularly color duplex to
locate the vessel. |
| Surgical risk is low: includes infection, bleeding, scalp necrosis, and
damage to branches of the facial nerve. |
| A potentially serious complication: ipsilateral hemispheric infarction in
the rare patient where the superficial temporal artery provides a significant collateral
via the palpebral arteries of the ophthalmic artery to reconstitute the intracranial
segment of an occluded carotid artery. |
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| Differential diagnosis |
| Rheumatoid arthritis:
 | 15% of patients with giant cell arteritis have synovitis, but only the
large and sternoclavicular joints are involved. |
| Titers of rheumatoid factor are not increased. |
| May have both diseases. |
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| Takayasu's disease:
| A rare form of arteritis, causes stenosis and occlusion of the major
aortic branches |
| This disease affects primarily young women. |
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| Periarteritis nodosa:
| Can involve any system, it usually causes fever, abdominal pain,
hypertension, edema, and a polyneuropathy. |
| Albuminuria and hematuria are prominent. |
| Renal biopsy should clarify the diagnosis. |
|
| Wegener's granulomatosis:
| Severe upper respiratory involvement |
| Deteriorating renal function |
| Temporal arteries are rarely affected. |
| Positive cytoplasmic antineutrophil antibodies |
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| Treatment |
| Start treatment ASAP |
| Prednisone 30 mg bid or 1.0-2.0 mg/kg/day. |
| If symptoms fail to improve within 24-72 hours, doses are increased as
necessary until symptoms are resolved or normalized by therapy. |
| If eye affected, consider Solumedrol 1 g iv qd X 3 days. |
| Note: treatment for polymyalgia rheumatica without GCA is Prednisone 2 mg
to 20 mg per day |
| Taper steroid slowly for Temporal arteritis: Start with 60 mg/d, reduce
by 20 mg/d every month. 5th month 10 mg per day. Then reduce by 1-2 mg/day every month. |
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| Prevent side effects from long term
steroid use |
| Monitor weight. Need Diet, exercise. |
| Calcium 1,500 mg qd. Vitamin D 400 IU qd |
| Monitor for glucose, hypertension, cataract, gastritis |
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| References and Further reading |
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