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Abstract: TH-PO0241

Vision Loss in ESRD: A Case Series of Mönckeberg Medial Sclerosis Mimicking Giant-Cell Arteritis

Session Information

Category: Bone and Mineral Metabolism

  • 502 Bone and Mineral Metabolism: Clinical

Authors

  • Hussain, Irshad, SUNY Upstate Medical University, Syracuse, New York, United States
  • Aslam, Muhammad Haseeb, SUNY Upstate Medical University, Syracuse, New York, United States
  • Shafqat, Mahzaib, Allama Iqbal Medical College, Lahore, Punjab, Pakistan
  • Hashemi, Sara, SUNY Upstate Medical University, Syracuse, New York, United States
  • Ali, Asim, SUNY Upstate Medical University, Syracuse, New York, United States
  • Zaccarini, Daniel J., SUNY Upstate Medical University, Syracuse, New York, United States
  • Mobeen, Haris, SUNY Upstate Medical University, Syracuse, New York, United States
Introduction

Mönckeberg medial calcific sclerosis (MCS) is a vascular condition characterized by calcification of the tunica media of small to medium-sized arteries, sparing the intima. It is commonly seen in patients with end-stage renal disease (ESRD) and contributes to arterial stiffness and increased cardiovascular morbidity. Rarely, MCS may involve the temporal arteries and mimic giant cell arteritis (GCA), presenting with acute vision loss. Differentiating between MCS and GCA is critical, as the management strategies differ significantly.

Case Description

Case 1: A 68-year old man with ESRD presented with sudden, painless left monocular vision loss. He denied systemic symptoms. Labs showed ESR 17mm/hr, CRP 5.8 mg/L. CT angiogram ruled out stroke. Retinal angiography was consistent with anterior ischemic optic neuropathy. He received high-dose IV methylprednisolone followed by oral prednisone. Temporal artery biopsies (TABs) were negative for arteritis, but one showed medial calcification consistent with MCS. Vision did not recover.

Case 2: A 79-year old male with ESRD developed progressive left eye pain and vision loss with CN VI palsy. CT orbit revealed optic nerve calcification. ESR was 18 mm/hr, CRP 86 mg/L. ANA + (1:80). He was treated with steroids for presumed GCA. TAB showed medial calcification of the internal elastic lamina, diagnostic of MCS. Vision loss was permanent.

Discussion

Both patients were initially treated for GCA but were ultimately diagnosed with MCS. MCS can appear in 6% of GCA-negative TABs and is more prevalent in ESRD and diabetic patients. Unlike GCA, MCS does not respond to corticosteroids. Early recognition is critical to prevent unnecessary immunosuppression and to preserve vision in the unaffected eye.

Digital Object Identifier (DOI)