Roverano Susana, Ortiz Alberto, Henares Eduardo, Eletti Mónica, Paira Sergio
Rheumatology Section, J.M. Cullen Hospital, Santa Fe, Argentina.
Pathology Section, J.M. Cullen Hospital, Santa Fe, Argentina.
Clin Rheumatol. 2015 Nov;34(11):1985-8. doi: 10.1007/s10067-015-2942-x. Epub 2015 Apr 24.
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.
一位82岁女性因左眼突然视力丧失前来就诊。15天前,她曾抱怨复视。她有疑似巨细胞动脉炎的症状,但体格检查正常。实验室检查结果显示红细胞沉降率(ESR)=62mm/1°h;尿毒症=0.56g/dl(正常<0.45);血清肌酐=1.7mg%(正常<1.4);钙和磷水平低;尿钙和血清甲状旁腺激素正常。眼底检查和脑部磁共振成像(MRI)显示视神经、视交叉、视交叉后区域、眼肌、眼球、泪腺、眶周脂肪、海绵窦和枕叶皮质均正常。怀疑为颞动脉炎,因此进行了活检。结果显示动脉中膜和内弹性膜有大量钙沉积,管腔狭窄,无炎症和多核巨细胞。组织学诊断为钙化防御。尽管钙化防御是一种在终末期肾病患者中已被充分描述的病症,但许多病例是由非尿毒症原因引起的。迄今为止,文献中仅描述了6例酷似巨细胞动脉炎的钙化防御病例。