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At a glance

A severe throbbing headache syndrome characterized by inflammation of the temporal (pulseless, enlarged superficial artery) and other cranial arteries. Patients are affected with anorexia, insomnia, and low-grade fever. There is often accompanying eye watering, nasal congestion, or swelling around the eye, on the affected side. These symptoms typically last from 15 minutes to 3 hours. Blindness may occur if process reaches the ophthalmic artery. Rarely presents before the sixth decade of life.

Synonyms

Arteritis Temporalis (of Horton); Cluster Headache Syndrome; Horton Arteritis; Horton Disease I; Horton Giant Cell Arteritis; Horton Temporal Arteritis; Horton-Gilmour Disease; Horton-Magath-Brown Syndrome; Hutchinson-Horton Syndrome; Giant Cell Arteritis (GCA); Granulomatosis Arteritis; Senile Arteritis; Temporal Arteritis; Temporal Megacellular Arteritis.

N.B.: Do not confuse with Bing-Horton Syndrome or Horton disease II (cluster headache and erythroprosopalgia).

History

The typical clinical presentation of Horton’s headache was first described in 1641 by the Dutch physician Nicolaas Tulp (1593-1674). He obtained fame with his Anatomy Lecture that was painted in 1632 by Rembrandt Harmenszoon van Rijn (1606-1669). It is believed the initial description of the disease was made by Thomas Willis (1621-1675). The first extensive report was made by Wilfred Harris (1926), who did not, however, distinguish clearly between the headache and other painful conditions of long duration and alternate hemispheres. Systemic arteritis affects major and small arteries. It was first described by Jonathan Hutchinson in 1890. However, it was BT Horton (1939) who first described the attacks and their associated symptoms as well as described an attractive pathological theory and etiology. Subsequently, Klunke et al in 1952 first described the recurrent nature that distinguishes Horton’s headache from other forms of headache and the term cluster headache was implanted.

Incidence

More common in the northern latitudes (15-30:100,000 persons) compared to southern latitudes (<2:100,000 persons). Rather common in northern Europe. Rare in nonwhites. Both sexes affected, but females twice as often as males.

Genetic inheritance

Not a genetic disorder even though genetic factors may predispose to the disease (three allelic variants of the HLA-DRB1*04 family are overrepresented in patients with biopsy-proven disease). Familial aggregation has been observed. People of Hispanic descent seem genetically protected against the syndrome.

Pathophysiology

The pathogenesis is not fully understood, but the underlying cause of the inflammation is an autoimmune reaction to the lining of temporal and related arteries (mainly the adventitia); however, Horton Syndrome cannot be considered an autoimmune disease. Histologically, there is lymphocyte, plasma cell, and multinucleated giant cell infiltration of the vessel wall. A cell-mediated autoimmune mechanism against elastin is suggested. The cellular infiltrate predominantly consists of CD4 + T lymphocytes and monocytes. High levels of IL-1 and IL-2 have been identified in the lesions of Horton Syndrome, as well as in polymyalgia rheumatica, suggesting a common pathogenesis. Chlamydia pneumoniae may play a significant role (data suggest the organism could be viable and undergo active vegetative growth in temporal ...

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