Calciphylaxis of the temporal artery masquerading as temporal arteritis: a case presentation and review of the literature

Abstract

An 82-year-old woman came to consultation with sudden visual loss in her left eye. Fifteen days before, she complained of diplopia. She had doubtful symptoms of giant cell arteritis and showed a normal physical exam. Lab results showed erythrosedimentation rate (ESR) = 62 mm/1°h; uremia = 0.56 g/dl (normal <0.45); serum creatinine = 1.7 mg% (normal <1.4); low calcium and phosphorus; and normal urine calcium and serous PTH. Fundus exam and brain magnetic resonance imaging (MRI) showed normality of optic nerves, chiasma, retrochiasmatic area, ocular muscles, eyeballs, lacrimal glands, periorbital fat, cavernous sinuses, and occipital cortex. A temporal arteritis was suspected; therefore, a biopsy was carried on. It showed the presence of large calcium deposits in the artery's tunica media and internal elastic lamina, with narrowing of the lumen, with no inflammation and multinuclear giant cells. Histological diagnosis is calciphylaxis. Although calciphylaxis is a well-described entity that occurs in end-stage renal patients, many cases are due to non-uremic causes. To date, there are only six cases described in literature of calciphylaxis mimicking GCA.

Systematic Review of the Yield of Temporal Artery Biopsy for Suspected Giant Cell Arteritis

“…2 A multivariable prediction model with geographic external validation has been shown to outperform the pre-TAB ACR criteria, 18 and may be more accurate in selecting candidates for TAB. Although some feel that a high ACR risk score may obviate TAB, 14 we advocate confirmatory TAB given the risks of systemic glucocorticoid treatment, and because other diseases can mimic or overlap the clinical presentation of GCA, including amyloidosis, 21 granulomatosis with polyangiitis and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, 22 calciphylaxis, 23 Mönckeberg's medial calcific sclerosis, 24 and zoster sine herpete. 25 Without TAB, these alternative diagnoses may not be determined in an expedient fashion.…”

Too Young to Undergo Temporal Artery Biopsy? Calciphylaxis-Related Anterior Ischemic Optic Neuropathy

“…Rarely, calciphylaxis can be associated in elderly patients with AION [8][9][10] or with retinal ischaemia, 9 thus mimicking giant cell arteritis 8,9,11,12 and leading to diagnostic temporal artery biopsy. Pathology features in temporal artery calciphylaxis include calcifications of the tunica media and internal elastic media, narrowing of the lumen, but no inflammation nor multinuclear giant cells.…”

“…Pathology features in temporal artery calciphylaxis include calcifications of the tunica media and internal elastic media, narrowing of the lumen, but no inflammation nor multinuclear giant cells. 11,12 There are less than a dozen described patients with AION due to calciphylaxis. [7][8][9][10][11][12] The majority of described patients were old, with end-stage renal disease, arterial hypertension, and high inflammatory markers, justifying temporal artery biopsy and leading to the correct diagnosis.…”

Temporal Artery Vascular Diseases

“…An ophthalmologic examination may demonstrate authentic anterior ischemic optical neuropathy. Although GCA is more common in women, the majority of cases of TA calciphylaxis occur in men around 70 years of age, who are hypertensive and have renal insufficiency [ 97 ].…”