Diagnosis and Treatment of Giant Cell Arteritis (GCA)

Author: Jan Tore Gran Published year: 2017 Downloads: 11
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Brief content

Giant cell (temporal) arteritis (GCA) is characterized by a granulomatous inflammatory reaction that penetrates all layers of the wall of medium- to large-sized arteries. The transmural vasculitis is particularly prone to involve cranial arteries such as the superficial temporal artery (TA), but not infrequently the disease extends to involve large muscular arteries including the subclavian and axillary arteries and the aorta.

The earliest recorded description of GCA dates to the 10th century when Ali Ibn Isa of Baghdad in his memorandum book remarked on the relationship between inflamed arteries and visual symptoms. An even earlier case of GCA is disclosed in a photograph of carving of a blind harpist with swollen eyelids and prominent temporal arteries from an Egyptian tomb of Pa-Aton-Em-Heb dating back to 1350 BC. Moreover, the painting by the Flemish painter Jan van Eyck (1436) “The Holy Virgin with Canon van der Paele” may also represent an early case of GCA. The canon had prominent temporal arteries and diffuse swelling of the hand, the latter possibly indicating coexistent PMR. The tortuous and inflamed artery seen in the portrait of Lorenzo Gambietti by Piero di Cosimo in 1502 is also suggestive of GCA. Thus, although GCA may have existed for several centuries there is rather little definite historical documentation of its existence. As GCA preferentially affects persons aged 50 years and more, one possible explanation is the low average life expectancy in ancient times, exposing few individuals to the risk of contracting GCA.

The first clinical description of GCA was given by Jonathan Hutchinson in 1890 (St. Bartholomew`s Hospital, London) who was asked to examine Mr. Rumbold, an 80-year-old father of a porter at the London Hospital who had “red streaks on his head” which were so painful that they prevented him from wearing a hat (6). Forty-two years later (1932), the first histopathological evidence of a granulomatous vasculitis in the temporal arteries was reported by Horton, Magath, and Brown at a Mayo Clinic staff meeting in 1932 (7). In 1937, Horton and Magath added to their former report the prominence of headache, difficulty chewing food, and transient diplopia (8). Blindness as a complication of GCA was, however, first recognized by Jennings in 1938 (9). In 1941, Gilmore (10) suggested the presence of giant cells as characteristic of the disease which he called “giant cell chronic arteritis”. Advances in the Diagnosis and Treatment of Vasculitis During the 1930ies, the term “Temporal arteritis” was gradually introduced, whilst the designation “Cranial arteritis” was coined by Kilbourne and Wolff in 1946 (11) to point out that the temporal artery is not the only scalp artery affected in GCA.

The most frequent complaints of GCA are headaches and malaise. However, GCA may also present as a systemic inflammatory syndrome characterized by non-specific constitutional symptoms related to systemic inflammation in the absence of focal ischemic symptoms. This is often referred to as “silent or masked GCA” (12). On the other hand, the disease may also present with minimal or absent systemic manifestations, but with organ dysfunction such as visual loss or peripheral neuropathy. The term “Occult GCA”, coined by Simmons and Cogan in 1962 (13) is often used to denote such a disease variant (14). GCA rather frequently coexists with polymyalgia rheumatic (15), affects most often persons of Nordic origin (15), and almost exclusively involves individuals 50 years or older (15, 16).

The annual incidence of GCA among persons 50 years or older has been estimated to be 29/100000 (15), but autopsy studies have indicated a substantially higher incidence (17). It is possible that physician awareness is responsible for the progressive increase in the incidence that has been observed over the past two to five decades in different parts of the world (18, 19), conceivably remaining stable in the recent years (20). Mortality in GCA appears similar to that of the general population (21, 22). The cornerstone of treatment is represented by oral glucocorticosteroids (CS), but new therapeutic options that better control disease activity, exhibiting lower incidences of side effects, and reducing disease complications are highly warranted.