Fall 2017 Newsletter

Follow our latest updates in our Fall 2017 Newsletter

Fall 2017 Newsletter
7th Residency Announcement

Exciting Residency Announcement!

ACGME approves seventh resident complement; first increase in over 30 years!

Grand Rounds

Grand Rounds and Case Studies

Check out our weekly presentations

OTEP

OTEP

Ophthalmic Technician Education Program

LARGEST STUDY ON CHINESE AMERICANS PUBLISHED

LARGEST STUDY ON CHINESE
AMERICANS PUBLISHED

USC Ophthalmology Researchers Find More
Effective Treatments For Blinding Eye Diseases

EDUCATION

Case Study: Catch the Sombrero

YouningZhang Toy
Presenter: Youning Zhang, MD Discussant: Brian Toy, MD
 

History

  • 54 year-old female with h/o SLE with lupus nephritis, CKD, anemia, HTN here for routine eye exam
  • Patient reports gradual onset progressively worsening painless blurry vision OU for 1-2 years
  • Denies any other symptoms
  • Past medical history includes: SLE with nephritis, anemia, HTN
  • Medications include: mycophenolate, hydroxychloroquine, prednisone, benazepril, darbepoietin, ferrous sulfate, folic acid

Exam Findings

  • VA 20/70 ph 20/40 OD, 20/60 ph 20/40 OS. IOP 22 OD, 20 OS. Pupils round reactive, no RAPD. Extraocular movement intact bilaterally. VFFTC.
  • Slit lamp examination normal
  • Dilated fundus exam showed RPE mottling temporal to the fovea in both eyes and chorioretinal scar inferonasal to the left optic nerve

Differential Diagnosis

  • Hydroxychloroquine toxicity
  • Macular hole
  • Stargardt disease
  • Cone dystrophy
  • Age-related macular degeneration

Additional Investigations

Figure 1: Fundus autofluorescence (FAF) in 2014 shows parafoveal hyper-autofluorescence worse inferotemporally in both eyes.
Figure 1: Fundus autofluorescence (FAF) in 2014 shows parafoveal hyper-autofluorescence worse inferotemporally in both eyes.
Figure 2: FAF in 2017 demonstrates temporal parafoveal patch of hypo-autofluorescence with surrounding hyper-autofluorescence halo bilaterally.
Figure 2: FAF in 2017 demonstrates temporal parafoveal patch of hypo-autofluorescence with surrounding hyper-autofluorescence halo bilaterally.

Catch the Sombrero 3

Figure 3: OCT of macula demonstrated temporal > nasal parafoveal EZ/IZ disruption that appeared progressed in 2017 compared to 2014.
Figure 3: OCT of macula demonstrated temporal > nasal parafoveal EZ/IZ disruption that appeared progressed in 2017 compared to 2014.

 

  • HVF 10-2 in 2017 demonstrated nasal superior > inferior arcuate parafoveal depressions consistent with the pattern of the parafoveal EZ/IZ disruption seen on OCT which was worse temporally and inferiorly

Diagnosis

  • Hydroxychloroquine toxicity

Pathophysiology

  • Hydroxychloroquine affects the pH of intracellular vacuoles, modifying the function of lysosomes and antigen processing in antigen-presenting cells.
  • Hydroxychloroquine furthermore affects DNA/RNA synthesis in photoreceptors. Membranous cytoplasmic bodies also form in retinal ganglion cells, leading to cell death. RPE cells can bind the drug in an inactive form on melanin granules but once the binding capacity is exceeded, cell damage occurs.

Treatment

  • Immediate cessation of hydroxychloroquine when definitive toxicity is detected
  • Progression of maculopathy may progress for 1-3 years depending on the severity of maculopathy at the time of drug cessation
  • No effective treatment to reverse hydroxychloroquine toxicity

Screening Guidelines

  • The maximum recommended dose for hydroxychloroquine is 5mg/kg of actual body weight. This is a revised recommendation from the AAO in 2016, based on the work of Marmor and Melles in analyzing data from a large Northern California patient population.
  • Risk factors for toxicity include:
    • Dose > 5mg/kg actual body weight
    • Longer duration of use
    • Concurrent tamoxifen use
    • Renal disease
    • Presence of other retinal or macular disease
  • Minimum screening is at baseline (within one year of starting drug) and then annually starting at five years of use, sooner if any risk factors are present
  • Screening consists of:
    • HVF 10-2 (white stim) for all patients (add HVF 24-2 for Asian patients)
    • SD-OCT
    • Consider: fundus autofluorescence, multifocal ERG

Prognosis and Future Directions

  • The Ophthalmology and Rheumatology services at LAC+USC are collaborating to improve the screening process for patients taking hydroxychloroquine to eliminate the incidence of new toxicity in the LA County DHS patient population.

References

  • Hallberg A, Naeser P, Andersson A. Effects of long-term chloroquine exposure on the phospholipid metabolism in retina and pigment epithelium of the mouse. Acta Ophthalmol (Copenh). 1990;68:125-130.
  • Leung LS, Neal JW, Wakelee HA, et al. Rapid onset of retinal toxicity from ghigh-dose hydroxychloroquine given for cancer therapy. Am J Ophthalmol 2015; 160:799-805.
  • Lyons JS, Severns ML. Using multifocal ERG ring ratios to detect and follow plaquenil retinal toxicity: a review: review of mfERG ring ratios in plaquenil toxicity
  • Melles RB, Marmor MF. The risk of toxic retinopathy in patients on long-term hydroxychloroquine therapy. JAMA Ophthalmol 2014;132:1453-60.
  • Marmor MF, Kellner U, Lai TY, et al. Recommendations on Screening for Chloroquine and Hydroxychloroquine Retinopathy (2016 revision). American Academy of Ophthalmology
  • Perlman E, Friday R, Greenberg P, et al. Dosechecker: Solving the hydroxychloroquine dosing dilemma with a smart phone app. 2017 ACR/ARHP Annual Meeting. Abstract number 2635. Sep 18, 2017.
  • Rosenthal AR, Kolb H, Bergsma D, Huxsoll D, Hopkins JL. Chloroquine retinopathy in the rhesus monkey. Invest Ophthalmol Vis Sci. 1978;17:1158-1175.
  • Wolfe F, Marmor MF. Rates and predictors of hydroxychloroquine retinal toxicity in patients with rheumatoid arthritis and systemic lupus erythematosus. Arthritis Care Res (Hoboken). 2010;62:775-784.

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Produced by: Monica Chavez, John Daniel and Dr. Vivek Patel
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