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Painless Visual Loss in a Septuagenarian

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A patient in their late 70s with a history of Crohn disease, coronary artery disease, hypercholesterolemia, and transient ischemic attack presented with sudden, painless, left-sided vision loss. They denied headache, scalp tenderness, and jaw claudication. The erythrocyte sedimentation rate (ESR) was 103 mm/h and a magnetic resonance angiographic examination of the brain was negative for mass, infarction, and hemorrhage. Examination results of a right, superficial, temporal artery biopsy specimen showed chronic, nongranulomatous inflammation surrounding adventitial vessels and extensive subintimal fibrosis, without giant cells or segmental loss of the internal elastic lamina, which was interpreted as compatible with temporal arteritis. High-dose corticosteroid therapy was initiated (1 g methylprednisolone intravenously for 3 days followed by 60 mg/d of oral prednisone).

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Systemic amyloidosis in the setting of lymphoplasmacytic lymphoma (Waldenström macroglobulinemia)

C. Order serum and urine protein electrophoresis

An older patient presenting with visual loss and pallid disc edema arouses suspicion for an anterior ischemic optic neuropathy. As the most common vasculitis of older adults in North America, giant cell arteritis (GCA) has become the poster child of the arteritic form of anterior ischemic optic neuropathy. Well established as visually morbid—with an approximate 8% rate of permanent vision loss and a 50% rate of fellow eye involvement—and responsive to corticosteroid therapy, GCA has a low threshold for diagnosis and treatment.1 However, the prevailing criteria for diagnosing GCA (the 1990 American College of Rheumatology classification) is relatively nonspecific, having a specificity of 91.3%.2 Other vasculitides, including eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, hypersensitivity vasculitis, Henoch-Schönlein purpura, polyarteritis nodosa, and Takayasu arteritis, are all cited as misclassifications for GCA in the original article by Hunder et al.2

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Article Information

Corresponding Author: Ralph C. Eagle Jr, MD, Department of Neurology and Ophthalmology, Wills Eye Hospital, Sidney Kimmel Medical College of Thomas Jefferson University, 840 Walnut St, Ste 1410, Philadelphia, PA 19107 (reagle@willseye.org).

Published Online: March 18, 2021. doi:10.1001/jamaophthalmol.2020.4638

Conflict of Interest Disclosures: None reported.

References
1.
Chen  JJ , Leavitt  JA , Fang  C , Crowson  CS , Matteson  EL , Warrington  KJ .  Evaluating the incidence of arteritic ischemic optic neuropathy and other causes of vision loss from giant cell arteritis.   Ophthalmology. 2016;123(9):1999-2003. doi:10.1016/j.ophtha.2016.05.008 PubMedGoogle ScholarCrossref
2.
Hunder  GG , Bloch  DA , Michel  BA ,  et al.  The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis.   Arthritis Rheum. 1990;33(8):1122-1128. doi:10.1002/art.1780330810 PubMedGoogle ScholarCrossref
3.
Jia  L , Couce  M , Barnholtz-Sloan  JS , Cohen  ML .  Is all inflammation within temporal artery biopsies temporal arteritis?   Hum Pathol. 2016;57:17-21. doi:10.1016/j.humpath.2016.07.004 PubMedGoogle ScholarCrossref
4.
Niederkohr  RD , Levin  LA .  Management of the patient with suspected temporal arteritis a decision-analytic approach.   Ophthalmology. 2005;112(5):744-756. doi:10.1016/j.ophtha.2005.01.031 PubMedGoogle ScholarCrossref
5.
Chen  J , Zhu  J , Chen  L , Hu  C , Du  Y .  Steroids in the treatment of nonarteritic anterior ischemic optic neuropathy: a PRISMA-compliant meta-analysis.   Medicine (Baltimore). 2019;98(46):e17861. doi:10.1097/MD.0000000000017861 PubMedGoogle Scholar
6.
Ghinai  RA , Mahmood  S , Mukonoweshuro  P , Webber  S , Wechalekar  AD , Moore  SE .  Diagnosing light chain amyloidosis on temporal artery biopsies for suspected giant cell arteritis.   J Neuroophthalmol. 2017;37(1):34-39. doi:10.1097/WNO.0000000000000447 PubMedGoogle ScholarCrossref
7.
Bellavia  D , Pellikka  PA , Al-Zahrani  GB ,  et al.  Independent predictors of survival in primary systemic (Al) amyloidosis, including cardiac biomarkers and left ventricular strain imaging: an observational cohort study.   J Am Soc Echocardiogr. 2010;23(6):643-652. doi:10.1016/j.echo.2010.03.027 PubMedGoogle ScholarCrossref
8.
Gertz  MA , Comenzo  R , Falk  RH ,  et al.  Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18-22 April 2004.   Am J Hematol. 2005;79(4):319-328. doi:10.1002/ajh.20381 PubMedGoogle ScholarCrossref
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