Merec Manual and Temporal Arteritis

Date: Fri, 3 Sep 1999 11:12:50 -0500

From: "Ruby Bartlett"

Please go here and enter the name in the top search space. There are many pages on this. Below is just one. Every one should really keep this "Merck Manual" at their finger tips. It is used by most doctors, I believe.

Hugs, Ruby

This Publication Is Searchable

The Merck Manual of Diagnosis and Therapy

Section 5. Musculoskeletal And Connective Tissue Disorders

Chapter 50. Diffuse Connective Tissue Disease

Topics

Rheumatoid Arthritis

Sjögren's Syndrome

Behçet's Syndrome

Relapsing Polychondritis

Systemic Lupus Erythematosus

Discoid Lupus Erythematosus

Systemic Sclerosis

Eosinophilic Fasciitis

Polymyositis And Dermatomyositis

Polymyalgia Rheumatica

Vasculitis

Temporal Arteritis

Polyarteritis Nodosa

Wegener's Granulomatosis

Mixed Connective Tissue Disease

(Giant Cell Arteritis; Cranial Arteritis)

A chronic inflammatory disease of large blood vessels, particularly those with a prominent elastica, occurring primarily in the elderly.

Etiology and pathogenesis of temporal arteritis (TA) are unknown. Estimated prevalence is about 1/1000 in patients > 50 yr. There seems to be a slight female preponderance. TA is often seen concomitantly with polymyalgia rheumatica (see above).

Pathology

TA most often involves arteries of the carotid system, particularly the cranial arteries. Segments of the aorta, its branches, the coronary arteries, and the peripheral arteries may also be affected. The disease has a predilection for arteries containing elastic tissue; it rarely occurs in veins. The histologic reaction is a granulomatous inflammation of the intima and inner part of the media; lymphocytes, epithelioid cells, and giant cells predominate. It causes marked thickening of the intimal layer with narrowing and occlusion of the lumen. The arteritis may be localized, multifocal, or widespread.

Symptoms and Signs

Presentations are diverse, depending on the distribution of the arteritis, but typically include severe headache (especially temporal and occipital), scalp tenderness, and visual disturbances (amaurosis fugax, diplopia, scotomata, ptosis, blurred vision). Pain on chewing in the masseter, temporalis, and tongue muscles is characteristic. Blindness caused by ischemic optic neuropathy probably occurs in <= 20% of patients but is very rare after high-dose corticosteroid treatment. Systemic symptoms are the same as for polymyalgia rheumatica, to which TA may be related. Patients may also present with arthritis, carpal tunnel syndrome, FUO, fatigue, unexplained weight loss, radiculopathy, and, rarely, pulseless disease (see Takayasu's Arteritis under Inflammation of the Aorta in Ch. 211). Physical examination may reveal swelling and tenderness with nodularity over the temporal arteries and, rarely, bruits over the large vessels.

Diagnosis

ESR is usually markedly elevated (often > 100 mm/h, Westergren method) during the active phase but is normal in about 1% of patients. Normochromic-normocytic anemia is often present and, at times, profound. Serum alkaline phosphatase may be elevated. Other nonspecific findings may include polyclonal hyperglobulinemia and leukocytosis.

TA can be diagnosed clinically but should be verified by temporal artery biopsy because of the need for prolonged corticosteroid treatment. Even a temporal artery that is normal on palpation and without tenderness or swelling may be abnormal on biopsy. Bilateral biopsy and removal of segments of >= 2 cm may increase the diagnostic yield. In patients with pulseless disease, angiography may identify areas of narrowing. Treatment should not be delayed to accommodate performing the biopsy or obtaining results. Histologic changes are not greatly altered by <= 3 days of high-dose corticosteroids.

Treatment

To prevent blindness, treatment should be started as soon as TA is suspected. Most patients respond to prednisone 60 mg/day, usually maintained for 2 to 4 wk. Based on response, the prednisone can be tapered gradually, usually by 5 to 10 mg/wk to 40 mg/day, then by 2 to 5 mg/wk to 20 mg/day, then by 1 mg/wk thereafter. Normalization of ESR is not necessary. If symptoms (predominantly headache, fever, and myalgia) flare with tapering, the prednisone can be slightly increased until symptoms are controlled. Some patients may be weaned from prednisone within 1 yr, but many require low doses of prednisone for years. Azathioprine, methotrexate, and dapsone have been used in patients with unacceptable corticosteroid side effects, although evidence of efficacy is sparse.

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