A Man With Bilateral Corneal Verticillata and Corneal Crystals | Cornea | JN Learning | AMA Ed Hub [Skip to Content]
[Skip to Content Landing]

A Man With Bilateral Corneal Verticillata and Corneal Crystals

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

A 77-year-old man was referred to the cornea service for bilateral corneal verticillata noted first by his general ophthalmologist. One year prior to presentation, the patient had undergone coronary artery bypass grafting (CABG). He was discharged with amiodarone and used the medication for 6 weeks. He did not use amiodarone for the 10 months prior to presentation. Prior to his CABG procedure, the patient only used a low dose of lisinopril until he began experiencing progressive dyspnea and fatigue. He was found to have a 5-vessel blockage once he became symptomatic. His symptoms resolved after the CABG surgery, and he remained otherwise healthy. He denied any other symptoms, including headache, rash, joint pain, or chest pain. His medications at time of presentation were aspirin, atorvastatin, and carvedilol. His best-corrected visual acuity was 20/20 OD and 20/25 OS. A slitlamp examination revealed bilateral corneal verticillata and numerous fine anterior stromal crystals (Figure). He had mild nuclear sclerosis in both eyes. His pupils were round and equally reactive, and the remainder of the slitlamp examination and dilated fundus examination were within normal limits.

Please finish quiz first before checking answer.

You answered correctly!

Read the answer below and download your certificate.

You answered incorrectly.

Read the discussion below and retake the quiz.

Paraproteinemic keratopathy from monoclonal gammopathy of unknown significance

D. Initiate laboratory testing for immunoglobulins

Although most patients taking amiodarone will eventually develop corneal verticillata, amiodarone does not classically cause a crystalline keratopathy, for which other conditions should be excluded before reassurance is provided (choice A). Genetic testing (choice B) could be useful in patients with Fabry disease or other metabolic causes of verticillata, but Fabry disease would be less likely in this context. There is no evidence of an infection or an infiltrate, so initiating corneal scraping and culture (choice C) would not be indicated.

Monoclonal gammopathy of unknown significance (MGUS) and multiple myeloma (MM) are dyscrasias of plasma cells that result in elevated levels of abnormal monoclonal antibodies in the blood (paraproteinemia). Patients with MGUS are often asymptomatic and may lack signs of pathology on physical examination. Often, the discovery of MGUS in a patient is incidental when a patient has had laboratory work done for another screening. Multiple myeloma and MGUS may appear similar, although the testing for MM usually reveals higher degrees of paraproteinemia and plasma-cell proliferation. Because of the rate of progression from MGUS to MM (approximately 1% per year), patients with MGUS must be monitored closely to detect conversion to MM.1

Survey Complete!

Sign in to take quiz and track your certificates

Buy This Activity

JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 CME Credit™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC

Article Information

Corresponding Author: Vishal Jhanji, MD, Department of Ophthalmology, University of Pittsburgh School of Medicine, 203 Lothrop St, Pittsburgh, PA 15213 (jhanjiv@pitt.edu).

Published Online: March 5, 2020. doi:10.1001/jamaophthalmol.2020.0128

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Therneau  TM , Kyle  RA , Melton  LJ  III ,  et al.  Incidence of monoclonal gammopathy of undetermined significance and estimation of duration before first clinical recognition.   Mayo Clin Proc. 2012;87(11):1071-1079. doi:10.1016/j.mayocp.2012.06.014PubMedGoogle ScholarCrossref
2.
Garibaldi  DC , Gottsch  J , de la Cruz  Z , Haas  M , Green  WR .  Immunotactoid keratopathy: a clinicopathologic case report and a review of reports of corneal involvement in systemic paraproteinemias.   Surv Ophthalmol. 2005;50(1):61-80. doi:10.1016/j.survophthal.2004.10.002PubMedGoogle ScholarCrossref
3.
Chierego  C , Merz  T , Fasolo  A , Lagali  N , Pedrotti  E .  In vivo confocal microscopy of verticillata-like paraproteinemic keratopathy in a patient with monoclonal gammopathy of uncertain significance evolving into smoldering multiple myeloma.   Am J Ophthalmol Case Rep. 2019;15:100505. doi:10.1016/j.ajoc.2019.100505PubMedGoogle Scholar
4.
Balderman  SR , Lichtman  MA .  Unusual manifestations of monoclonal gammopathy, I: ocular disease.   Rambam Maimonides Med J. 2015;6(3):e0026. doi:10.5041/RMMJ.10211PubMedGoogle Scholar
5.
Karakus  S , Gottsch  JD , Caturegli  P , Eghrari  AO .  Monoclonal gammopathy of “ocular” significance.   Am J Ophthalmol Case Rep. 2019;15:100471. doi:10.1016/j.ajoc.2019.100471PubMedGoogle Scholar
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_LoginSubscribe_Purchase
Close
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_LoginSubscribe_Purchase
Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Close

Name Your Search

Save Search
Close
With a personal account, you can:
  • Track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
jn-learning_Modal_SaveSearch_NoAccess_Purchase
Close

Lookup An Activity

or

Close

My Saved Searches

You currently have no searches saved.

Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Close