Systemic Calciphylaxis: Diffuse Cutaneous Involvement and Ischemic Optic Neuropathy a case report.

 

Nurshazwani MAT SALLEH 1, Yin Ping LIEW 2

1 Department of Internal Medicine and  2 Department of Renal Medicine, Raja Isteri Pengiran Anak Saleha (RIPAS) Hospital, Brunei Darussalam

 

ABSTRACT

Calciphylaxis, or calcific uraemic arteriolopathy, is a rare disease that is difficult and challenging to treat and has a high mortality rate. Its pathogenesis remains to be fully elucidated. Cutaneous manifestations usually predominate although systemic calciphylaxis has been reported, whereby calcifications occur in other organs, including brain, eyes, lungs, intestines and the mesentery. Treatment option is limited. The most common disease-modifying treatment used is intravenous sodium thiosulphate. We report a case of systemic calciphylaxis in a dialysis-dependent patient who presented with cutaneous lesions and later developed bilateral ischemic optic atrophy. Our case had a poor clinical outcome despite optimal supportive care with multi-disciplinary approach and four weeks of intravenous sodium thiosulphate treatment.

 

Keywords: calciphylaxis, systemic, diffuse, calcific uraemic arteriolopathy, ischaemic optic neuropathy, sodium thiosulphate

 

Correspondence author: Dr. Nurshazwani MAT SALLEH, Department of Internal Medicine, RIPAS Hospital, Bandar Seri Begawan BA1710, Brunei Darussalam.

Email: nurshazwani.salleh@moh.gov.bn

 

Brunei Int Med J. 2017; 13 (4): 126-130

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