Amaurosis as an initial presentation of Takayasu arteritis in children

Rheumatol Int. 2023 Mar;43(3):575-587. doi: 10.1007/s00296-022-05115-2. Epub 2022 Apr 2.

Abstract

Visual disturbances in Takayasu arteritis (TA) are common but tend to be late manifestations of the disease. However, its presence at diagnosis must alert TA to avoid sight disabilities. Herein, we present two children with TA that debuted with vision loss, and the results of the literature review displayed 58 subjects with vision loss before the diagnosis of TA. The world English literature was reviewed by searching the PubMed database of the National Library of Medicine for the terms "Takayasu Arteritis" and "Blindness" or "Amaurosis fugax", from 190 to 2021. Cases eligible must present vision loss before or at TA diagnosis. Our two patients who presented with amaurosis fulfilled the criteria for TA diagnosis. The first patient had a bilateral and transient visual loss, whereas the second had monocular and permanent amaurosis. Both patients were cursed with hypertension and demonstrated large vessel compromise; their clinical picture improved with corticosteroids and immunosuppressant therapy. We identified in the literature review sixteen patients with TA in case reports and 42 in case series, plus our two cases presented herein with monocular or bilateral vision loss at the time of diagnosis. Previous literature indicated that amaurosis represents a severely advanced disease. Herein, we reported two children with amaurosis as their pivotal symptom; they had significant head and neck vascular alterations, so prompt and aggressive treatment is needed to prevent disease progression and disability. Transient or permanent vision loss must alert the physician to include Takayasu arteritis in the differential diagnosis.

Keywords: Amaurosis fugax; Blindness; Case report; Pulseless disease; Takayasu arteritis.

Publication types

  • Review

MeSH terms

  • Blindness / diagnosis
  • Child
  • Diagnosis, Differential
  • Disease Progression
  • Humans
  • Immunosuppression Therapy*
  • Takayasu Arteritis* / drug therapy
  • United States