Temporal arteritis

James Goodwin MD (Dr. Goodwin of the University of Illinois at Chicago has no relevant financial relationships to disclose.)
Originally released May 14, 1996; last updated September 21, 2016; expires September 21, 2019

This article includes discussion of temporal arteritis, arteritis of the aged, Bagratuni syndrome, cranial arteritis, giant cell arteritis, polymyalgia arteritica, Rumbold disease, senile rheumatic gout, and polymyalgia rheumatica. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Temporal arteritis is an important cause of ischemic complications in elderly patients and timely diagnosis can prevent important morbidity, most notably blindness from either anterior ischemic optic neuropathy or central retinal artery occlusion. These patients, left untreated, are also at increased risk for ischemic stroke. Treatment with high-dose corticosteroids is effective in preventing these complications, and initial treatment with intravenous pulse corticosteroids leads to more sustained clinical remission and lower subsequent oral corticosteroid dosage requirement. Other adjunctive immunosuppressant drugs are also currently being used in this setting as steroid-sparing agents. In this update, the author cites studies showing increased risk for a variety of ischemic cardiovascular complications among patients with temporal arteritis and patients who were taking statin drugs, which had a somewhat lesser incidence of these complications. He also reviews advances in biological treatment of giant cell arteritis with monoclonal antibodies with and without concomitant corticosteroid treatment.

Key points

 

• Temporal arteritis is a systemic vascular disease in which inflammation, usually with giant cells, affects the internal elastic lamina, adventitia, and adjacent media of medium and large arteries with a predilection for those in the head and neck.

 

• It is a disease almost exclusively in the elderly (older than 60 years) and usually presents with headache along with manifestations of ischemia in tissues of the head including most commonly pain, ulceration, and necrosis involving the scalp and oral mucosa.

 

• Ischemia of the temporalis and masseter muscles produces a distinctive syndrome called jaw claudication with crescendo pain in the mandibular region while chewing solid food and gradual relief after cessation of muscular activity in the involved muscles.

 

• The most common serious complication is vision loss from ischemic optic neuropathy or central retinal artery occlusion.

 

• Ischemic stroke can occur secondary to emboli from involved extracranial carotid and vertebral arteries—intracranial segments of the arteries are not involved, possibly because they lack an internal elastic lamina.

 

• Serious complications can be prevented by treatment with systemic corticosteroids in adequate doses over prolonged periods.

Historical note and terminology

The earliest case report of what was probably temporal arteritis is attributed to Ali Ibn Isa, a 10th century oculist (Woods 1936). Hutchinson described another early case in a man of 80 named Rumbold, who presented with painful, swollen, and reddened temporal arteries in the apparent absence of systemic symptoms. The inflammation subsided gradually and left the arteries as "pulseless impervious cords" (Hutchinson 1889). Some authors have since referred to temporal arteritis as Rumbold disease.

Credit for the earliest complete description of the disease with pathologic documentation of characteristic inflammation and giant cells in the temporal artery must be accorded to Horton and colleagues from the Mayo Clinic. The following is their succinct clinical description derived from examining 2 patients:

 

Both patients were admitted to the Clinic in the spring of 1931 because of fever, weakness, anorexia, loss of weight, anemia, mild leukocytosis, and painful, tender areas over the scalp and along temporal vessels. These manifestations had been present for 4 weeks to 6 weeks. Temporal artery biopsy in these 2 cases yielded granulomatous arteritis and periarteritis (Horton et al 1932).

Since this report, temporal artery biopsy has remained as the mainstay of pathologic diagnosis, though biopsy of other accessible cranial vessels has occasionally been advocated.

As cases accumulated, it became apparent that temporal arteritis usually presents with headache and symptoms of systemic illness exclusively in elderly persons. Another painful syndrome, now called "polymyalgia rheumatica" after Barber (Barber 1957), shares with temporal arteritis the strong predilection for the elderly and the association with signs and symptoms of systemic illness. In the mid-1950s the concept that temporal arteritis and polymyalgia rheumatica are but 2 clinical manifestations of the same basic disease process was introduced (Paulley and Hughes 1960). Hamrin and Ostberg both provided landmark publications, the former reporting his series of 93 cases of polymyalgia rheumatica with analysis of the relationship with temporal arteritis and underlying vascular pathology, and the latter reporting an autopsy study that revealed the link between the clinical syndrome called "polymyalgia rheumatica and giant cell arteritis of the aorta and its main branches" (Hamrin 1972; Ostberg 1973).

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