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Calciphylaxis, beware the ophthalmic mimic: A case series.

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Calciphylaxis can cause severe eye problems like vision loss, even without skin sores. Early diagnosis in kidney patients is key to proper treatment and avoiding misdiagnosis.

Keywords:
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Area of Science:

  • Ophthalmology
  • Nephrology
  • Pathology

Background:

  • Calciphylaxis is a rare condition causing skin necrosis due to vascular calcification.
  • Ocular involvement in calciphylaxis is uncommon, often presenting with severe visual impairment.

Observation:

  • Two cases of atypical calciphylaxis presenting with ocular ischemic events are described.
  • Case 1: A 76-year-old woman with pre-dialysis kidney failure experienced anterior ischemic optic neuropathy (AION) and cilioretinal artery occlusion.
  • Case 2: A 44-year-old man on hemodialysis developed AION and contralateral central retinal artery occlusion (CRAO).

Findings:

  • Both patients lacked the typical cutaneous manifestations of calciphylaxis.
  • The ocular findings included microvascular ischemic pathology, specifically AION and CRAO.
  • These presentations highlight the diagnostic challenge of calciphylaxis without skin lesions.

Implications:

  • Clinical suspicion of calciphylaxis is crucial in kidney failure patients with unexplained ophthalmic vascular events.
  • Temporal artery biopsy aids in confirming calciphylaxis and differentiating it from giant cell arteritis (GCA).
  • Timely, multidisciplinary treatment is essential for managing calciphylaxis and preventing irreversible vision loss, avoiding unwarranted corticosteroid use.