428. Sudden blindness in a young female
Creators: name: Dixit1, Juhi
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Presentation of Case:</strong> 15-year-old female presented to Rheumatology clinic with 4-year history of B/L lower limb claudication and recurrent episodes of episodic abdominal pain associated with misophobia. Over the past 2-3 years both her claudication and pain abdomen had worsened restricting her activities of daily living and schooling. Also, there was new onset gradually worsening B/L upper limb claudication making it difficult for her to reach objects above head due to pain. There was history of intermittent anginal chest pain, palpitations, significant loss of weight and appetite. In June 2021 she had 1 episode of sudden onset weakness in left upper and lower limb without facial nerve palsy or seizure that had resolved within few minutes without residual neurological damage. There was no other history suggestive of Connective tissue disorder and no family history of autoimmune disorders, young hypertension / CVA. Personal history was negative for tuberculosis. On evaluation patient was found to have absent pulses in B/L upper limbs with B/L subclavian and carotid bruit and normal B/L lower limb pulses. She was hypertensive with blood pressures of 170/104 &amp; 176/110 in right and left lower limbs respectively. Her cardiovascular, pulmonary, abdomen and CNS examination was normal. Two days later during hospital stay she developed acute onset painless loss of vision bilateral eyes not associated with floaters or restricted eye movements. At the time of episode patient did not have perception of light in both eyes, no signs of ocular inflammation and fundus examination was normal. <strong>Diagnostic Testing:</strong> Her blood investigations revealed Hb 9.9 gm%, TLC 8400/mm<sup>3</sup>, Platelet count 4,60,000/mm<sup>3</sup>, SGOT/SGPT 14/27 IU/dL, Urea/creatinine 23/0.49 mg/dL, HIV/HBsAg/Anti HCV non reactive, ESR 20 mm at 1<sup>st</sup> hour (&lt;15), CRP 3.26 mg/dL(0-6). Her CT aortography revealed diffuse circumferential mural thickening involving arch of aorta and descending thoracic aorta leading to mild luminal narrowing. Diffuse circumferential mural thickening with severe luminal narrowing was noted for bilateral common carotid and subclavian arteries prior to origin of vertebral arteries. For her ocular complaints MRI brain and FFA was done and was normal. <strong>Differential &amp; Final Diagnosis:</strong> In view of her clinical and radiological features she was diagnosed as Takayasu arteritis Type V (ITAS 2010 score 20). Possibility of CVA, CRAO, retinal vasculitis and ocular ischemic syndrome was ruled out in view of normal fundus examination, FFA and MRI brain. A possibility of subclavian steal syndrome was considered due to severe luminal narrowing of first part of bilateral subclavian arteries causing compromised vascular flow to B/L central retinal arteries and amaurosis fugax. <strong>Discussion of Management:</strong> She was started on 1 mg/kg oral prednisolone along with anti-hypertensives, methotrexate 10 mg/week and low dose aspirin. Digital subtraction angiography was done along with stenting of right subclavian artery with subsequent resolution of eye symptoms. On follow up she had no recurrence of visual symptoms and right upper limb claudication had improved. <strong>Conclusions: </strong>Ocular manifestations in Takayasu arteritis can result from disease activity or as complication of long term steroid use. Our patient had subclavian steal syndrome leading to amaurosis fugax which improved after right subclavian artery stenting. Normal inflammatory markers despite clinically active disease was unusual. <strong>Disclosures:</strong> None