systemic diseases: what is this?

11/8/2021

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Systemic Diseases and Their

Impact on the Retina

Michael W. Stewart, MD

Professor and Chairman

Department of Ophthalmology

Mayo Clinic Florida

Allergan: Research Support

Alkahest: Consultant

Bayer: Consultant

Biogen: Consultant

Regeneron: Research Support

Santen: Research Support

DisclosureCase

• 42 year old ♀ sent by primary physician for

decreased vision OS for 2 weeks. Also c/o strange

flashing lights.

• No significant medical history.

• Had complete physical 1 month ago.

• 25 pack year history of smoking.

• Review of systems:

– Mild non-productive cough for 2 months.

– Lower back pain for 6 weeks.

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Overview

• Systemic diseases may frequently involve the eyes and orbit

cause visual symptoms through secondary effects.

• Accurate diagnoses can frequently be made with a careful

ocular and systemic history. In medicine:

– 80% diagnoses made by history

– 10% by examination

– 10% by ancillary testing.

• Each component of the ocular examination – including

visual field and pupillary testing – can contribute critical

information to making the diagnosis.

Ocular Metastases

• Outnumber primary ocular malignancies 9:1

• Up to 18% of autopsied eyes of cancer patients

have choroidal metastases.

• Most common ophthalmic sites are choroid and

orbit.

• Typical complaints: blurred vision, visual field

defect, photopsias, pain, proptosis, diplopia.

• Most common primaries: breast, lung, colon.

Choroidal Metastases

• Creamy color

• 25% multiple

• Retinal Detachment

• Primary tumor is

usually known

• 64 year old lady with pain OD

• Hx of lung and cervical Ca

• Lung nodule followed 1 yr.

• 60 y.o. ♀ with treated non-metastatic breast cancer

now in remission.

• Found to have this lesion on routine eye exam.

• Given diagnosis of metastatic breast cancer!

• Recommended to have radiation and chemotherapy.

6 weeks later

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Systemic Diseases & Eye Findings

• Neoplasia

– Metastatic cancer

– Cancer-associated

retinopathy

• Vascular Diseases

– Hypertension and

retinopathy

– Diabetic retinopathy

• Infectious Diseases

– HIV/AIDS

• Inflammatory Diseases

– Rheumatoid scleritis

• Medications

– Plaquenil

Case

• 58 y.o. ♀ c/o decreased vision OU for 2

years

– Seen by a retina specialist 2 years ago but no

diagnosis.

– Developed a retinal detachment OS 1 year ago

fixed with vitrectomy – no improvement in

vision

– Now with 20/400 OU

– Medical hx:

• Breast CA in remission last evaluated 6 months ago

• No hx of abdominal surgery

Extramacular thinning with outer retinal loss

Summary

• Poor vision, thin retinas, hx of cancer

• Testing

– Normal vitamin concentrations, heavy metals,

FTA

– Anti-retinal antibody testing: antibodies

detected to 5 of 8 antigens tested

• Diagnosis of Autoimmune retinopathy

(idiopathic vs. cancer-associated)

Work-up

• Refer to Oncology to look for active breast

cancer

– Pet MRI showed

• Hilar and cervical lymph nodes

• Vertebral

– Positive biopsy of lymph node for breast Ca.

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• Neoplasia



retinopathy



retinopathy



– HIV/AIDS



• Medications

– Plaquenil

CASE

• 51 y.o. slim white female c/o blurred VA.

• Had LASIK 6 months ago.

• Seen 1.5 months ago with dry eyes.

• Recently stopped her anti-hypertensive

medication due to worsening dry eyes.

• Visual acuity

– OD: 20/25-

– OS: 20/20-

Both retinal and choroidal vascular

involvement Blood Pressure

225/150

Treatment: immediate transfer to ER.

Patients usually admitted for BP

control. Often have co-existing

kidney damage.

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Case

• 64 y.o. healthy ♂ c/o 4 weeks of new onset

headaches, mild GI distress, fatigue & malaise.

• Completely healthy except for history of BPH

(benign prostatic hypertrophy) with urinary

frequency that had been worsening recently.

• Noted sudden onset of monocular scotoma OS.

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What test should should be

done next?

BP: 180/110

What is the cause of this?

What other testing should be done?

Workup of Grade 3 Hypertensive

Retinopathy

• Urgent medical consult– Some would argue for evaluation in the Emergency

Department.

• Medical Exam– Otherwise unremarkable exam.

– Electrocardiogram: Normal

– Labs:

• Hb/HCT – 11.8/33.6

• Creatinine – 3.11 (normal <1.35)

– Residual bladder volume: 1000 ml (normal: 50-100)

Work-up continued…

• Renal Ultrasound shows obstructive

uropathy with dilated ureters and

hydronephrosis

Treatment

• Hospitalization– Urinary catheter

– Control of blood pressure

– Elective TURP trans-urethral resection of the

prostate

• 4 weeks later– BP: 125/77

– Hb/Hct: 13.6/39.1

– Creatinine: 1.72

– Post-void residual: 23 ml

9/20

10/12

Hypertensive Retinopathy

• Scheie Classification

• Normal

• Grade 1: mild arteriolar narrowing

• Grade 2: localized irregularity and

constriction

• Grade 3: CWSs and hemorrhages

• Grade 4: papilledema

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• Neoplasia



retinopathy



retinopathy



– HIV/AIDS



• Medications

– Plaquenil

2nd opinion for DME s/p IVTriamcinolone x 1

What treatment would you recommend for the right eye?

1. Macular laser

2. Bevacizumab

3. Ranibizumab

4. Aflibercept

5. Ozurdex

6. Vitrectomy

OD: 20/50 OS: 20/150

Aflibercept injected monthly

20/50

20/40

20/40

20/30

Baseline

2 months

5 months

19 monthss/p Aflibercept x 14

Aflibercept

monthly x 2

Aflibercept

monthly x 3

Aflibercept

q2month x 8

What treatment would you recommend for the left eye?

1. Macular laser

2. Bevacizumab

3. Ranibizumab

4. Aflibercept

5. Ozurdex

6. Vitrectomy

20/15020/50

Patient underwent vitrectomy with

membrane stripping OS

Baseline

Vitrectomy/MS

1 month

5 months

36 months

CE/IOL

No Injections

20/150

20/25

20/70

20/100

OD OS

ODOS1 month later

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Diabetic Retinopathy

• Background diabetic retinopathy (BDR)

– microaneurysms, hemorrhages, cotton wool

spots, hard exudates

• Proliferative diabetic retinopathy (PDR)

– neovascularization of retina or disc

• Pre-proliferative diabetic retinopathy

(PPDR)

– IRMA, venous beading

Diabetes Mellitus

• 9.5% of U.S. population is afflicted.

• 90% are type 2 (generally adult).

• Diet and exercise are crucial to control.

• Patients are treated with insulin and/or

oral hypoglycemic drugs.

• Number 7 cause of death.

Fluorescein Angiogram Systemic Diseases & Eye Findings

• Neoplasia



retinopathy



retinopathy



– HIV/AIDS



• Medications

– Plaquenil

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Holland et al. AJO 1982

7 patients with CMV retinitis, retinal

periphlebitis and Kaposi’s sarcoma

Kaposi’s SarcomaPost Infectious scarring

Active retinitis

Unaffected retina

CMV Retinitis

AIDS retinopathy

HIV Retinopathy

Herpes Zoster

• Progressive Outer Retinal Necrosis (PORN)

• Rx. with acyclovir + foscavir or intravit ganciclovir

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Ocular Toxoplasmosis

• frequently associated with CNS disease

• may be multifocal

Pneumocystis choroidal infiltrates

AIDS Eye Disease

• Epidemic through early 1990s (CMV retinitis in 20-40% of patients).

• Incidence of retinitis increases with low CD4+ counts. CMV generally occurs with CD4+ < 50.

• Other problems range from keratitis, orbital tumors, optic neuropathies, intracranial tumors to infections.

• Incidence of CMV retinitis decreased up to 80% following introduction of protease inhibitors in 1995. This treatment regimen was known as HAART (Highly Active Anti-Retroviral Therapy).


• Neoplasia



retinopathy



retinopathy



– HIV/AIDS



• Medications

– Plaquenil

Case

• 40 y.o. African American female

developed severe headaches 2 wk.

previously.

• Mostly left sided: orbit to occiput

• Transient visual changes OS with

headache.

• Diagnosed with new-onset migraine:

given trial of Imitrex.

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Case … cont’d

• Now 2 day history of constant blurred VA

OS.

• Ophthalmic Hx: “retinal detachment”

OD 15 yrs. ago.

• Current exam

– VA: OD - 20/20; OS - 20/400

– Normal anterior segments

– No APD

Case … cont’d

• B-scan OS:

– 1. Subretinal fluid inferiorly.

– 2. Choroidal and scleral thickening.

Case … cont’d

• DX: Posterior scleritis

• Evaluation:

– ESR, ANA, ANCA negative

– Rheumatoid factor positive

• Treatment: Prednisone 60 mg QD

• Rheumatoid work-up to evaluate for arthritis.

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Four weeks later

VA = 20/20


• Neoplasia



retinopathy



retinopathy



– HIV/AIDS



• Medications

– Plaquenil

Case

• 62 y.o ♀ with 20+ year history of

rheumatoid arthritis and Sjogren’s.

• Takes Plaquenil 600 mg (3 pills) per day

for 20 year.

• Referred for possible plaquenil toxicity

based on appearance of OCT.

• VA is 20/25 OU

• Takes serum drops for severe dry eyes.

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Plaquenil toxicity

• Risk of toxicity

– Dependent on dose and length of treatment

– 1% at 5 years

– 2% at 10 years

– 20% at 20 years1% of patients receiving

hydroxychloroquine

• Recommended dose is <5 mg/kg ideal

body weight.

Plaquenil toxicity

• Annual screening recommended at 5

years

– Recommended HVF and SD-OCT

– Additional tests include FAF and mERG

• Screening should detect damage before it

becomes ophthalmoscopically visible.

• No treatment available; damage can occur

for 4 months after d/c of drug.

Thank you

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systemic diseases: what is this?

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