A 72-year-old woman comes to the office due to persistent headaches, fatigue, and muscle pain. She has a history of hypertension and osteoarthritis. The patient does not use tobacco, alcohol, or recreational drugs. Her father had a subarachnoid hemorrhage from a ruptured berry aneurysm. She is treated with prednisone, leading to marked and rapid improvement in her symptoms. Which of the following pathologic processes is most likely responsible for this patient's condition?
Giant cell arteritis | |
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This patient, an elderly woman with headaches, muscular pain, and a rapid response to glucocorticoids, has typical features of giant cell arteritis (GCA). GCA is a common form of vasculitis that occurs almost exclusively in patients age >50. About half of patients with GCA will also have polymyalgia rheumatica, which causes achy pain in the shoulder and hip girdles. GCA also confers a higher risk of thoracic aortic aneurysms, but not berry aneurysms (her father's history is likely unrelated to her presentation).
GCA is characterized by a T-cell-mediated inflammatory process of medium-to-large arteries. It may occur diffusely but predominantly affects the arteries of the head and neck, especially the temporal artery (ie, temporal arteritis). Biopsy of the temporal artery will show scattered, focal granulomatous inflammation (most pronounced in the media) with intimal thickening, elastic lamina fragmentation, and giant cell formation (without distinct granulomas). GCA is histologically identical to Takayasu arteritis, which typically involves the aortic arch and affects primarily younger patients.
(Choices B and D) Hyaline arteriolosclerosis is characterized by deposition of homogeneous hyaline material in the intima and media of small arteries and arterioles. It is associated with diabetes, hypertension, and advanced age. Severe hypertension also results in hyperplastic arteriolosclerosis, which manifests as onion-like concentric thickening of the walls of arterioles (eg, laminated smooth muscle cells, reduplicated basement membranes). Both forms of arteriolosclerosis can cause end-organ ischemic injury. However, the resulting symptoms/signs would not respond to glucocorticoids.
(Choice C) Medial band-like calcifications are characteristic of Mönckeberg's medial calcific sclerosis and present as pipestem calcifications on x-ray. They may be associated with atherosclerosis but do not directly cause symptoms and are usually not clinically significant.
(Choice E) Transmural inflammation of the arterial wall with fibrinoid necrosis is consistent with polyarteritis nodosa. This uncommon form of vasculitis typically occurs in young adults and presents with intermittent episodes of a variety of manifestations, including abdominal pain, peripheral neuropathy, renal insufficiency, and severe hypertension.
Educational objective:
Giant cell arteritis is characterized by granulomatous inflammation of the media with intimal thickening and predominantly involves branches of the carotid artery, especially the temporal artery. It is strongly associated with polymyalgia rheumatica; both conditions respond promptly to glucocorticoid therapy.