Spotlight Case: A Numbing Case of Retinal Vasculitis With Multiple Differential Diagnoses

  • author profile pictureAshvini K. Reddy, MD
  • author profile pictureDiba Osmanzada, BS
  • author profile pictureSusan Osmanzada, BS
   

Case history

Figure 1: Fundus photographs show hyperemic optic nerves with bilateral occlusive retinal phlebitis with scattered intraretinal hemorrhages.

A 43-year-old Caucasian man is referred for evaluation of increased floaters and decreased vision in both eyes for 6 weeks. He is afebrile with no unusual rashes, enlargement of the lacrimal glands, or lymphadenopathy. Review of systems is remarkable for fatigue, frequent headaches, and generalized weakness. He has had intermittent numbness of his lower extremities for more than 10 years. Family history is remarkable for breast cancer, lung cancer, colon cancer, and skin cancer.

Best-corrected visual acuity (BCVA) is 20/25 in the right eye and 20/20 in the left eye. His pupil reaction is slightly sluggish in both eyes without an afferent pupillary defect (APD). Visual fields, intraocular pressure, and extraocular movement are within normal limits. Anterior segment examination is remarkable for quiet conjunctiva with a mixed population of keratic precipitates (fine and granulomatous) in both eyes. He has trace cell and 1+ cell in the anterior chamber of the right and left eyes, respectively. There is 2+ vitritis in both eyes.

Fundus photographs show hyperemic optic nerves with bilateral occlusive retinal phlebitis with scattered intraretinal hemorrhages (Figure 1). OCT of the macula demonstrates vitreous opacities without cystic retinal changes in both eyes (Figure 2). Fluorescein angiography (FA) demonstrates bilateral disc leakage with central and peripheral venous leakage with areas of peripheral nonperfusion (Figure 3).

Figure 2: OCT of macula demonstrates vitreous opacities without cystic retinal changes in both eyes.

His laboratory testing is normal for:

  • HIV
  • Tuberculosis
  • Syphilis

    Figure 3: FA demonstrates bilateral disc leakage with central and peripheral venous leakage with areas of peripheral nonperfusion.

  • Antineutrophil cytoplasmic antibodies (ANCA)
  • Antinuclear antibodies (ANA)
  • Rheumatoid factor (RF)
  • Toxoplasmosis
  • Angiotensin converting enzyme (ACE)
  • Bartonella serologies
  • Lyme disease

He has an elevated white blood cell count of 21 k/mcL.
   

What’s your diagnosis?

This patient’s diffuse retinal phlebitis and history of weakness and numbness are concerning for multiple sclerosis (MS). Demyelinating lesions on MRI confirm the diagnosis. A lumbar puncture was subsequently performed and was positive for oligoclonal bands with gamma restriction. He was started on high-dose steroids and showed limited improvement. He is actively managed by the neurology service.
  

Discussion

Figure 4: MRI of the brain with demyelinating lesions

The differential for retinal vasculitis is broad and includes infectious etiologies such as syphilis, toxoplasmosis, tuberculosis, and Lyme disease as well as inflammatory conditions such as Wegener’s granulomatosis, lupus, Behçet’s disease, and MS. Patient history and review of systems are important aspects of the approach to the management of patients with retinal vasculitis.

The patient had severe, occlusive retinal phlebitis with associated hemorrhage and vitritis. His complaints of weakness and numbness were highly suggestive of MS.

Ocular manifestations of MS include optic neuritis, pars planitis, and retinal vasculitis. MS-associated uveitis disproportionately affects young Caucasians and is more common in women. Ocular involvement is almost always bilateral and can be severe. Any history of neurologic disturbances such as weakness, numbness, or tingling should prompt an MRI to evaluate for demyelinating lesions, which are classically seen in MS. If concerning, the patient should be referred to a neurologist for lumbar puncture, cerebrospinal fluid analysis (which may show oligoclonal bands), and coordination of care. The human leukocyte antigen (HLA) HLA-DR15 haplotype has been associated with MS-related uveitis, and HLA typing may be indicated in select cases.

The mainstay of treatment in MS-related retinal vasculitis is oral or intravenous corticosteroids. Treatment with interferon and other agents may also be necessary for neurologic disease. The prognosis is generally good, especially when treatment is initiated early in the disease course.  

Take-home points

  1. MS disproportionately affects young, Caucasian women.
  2. The HLA-DR15 allele has been associated with MS-related uveitis.
  3. Ocular manifestations of MS include optic neuritis, intermediate uveitis, and retinal vasculitis.
  4. The mainstay of treatment for retinal vasculitis associated with MS is systemic steroids.
  5. Coordination with a neurologist is recommended.

References

1. Abu El-Asrar, A M, Herbort C P, Tabbara K F. Retinal vasculitis. Ocul Immunol Inflamm.  2005;13(6)415-433. doi:10.1080/09273940591003828.

2. Herbort C P, Cimino L, Abu El Asrar A M. Ocular vasculitis: a multidisciplinary approach. Curr Opin Rheumatol. 2205;17(1)25-33.

3. Schmidt H, Williamson D, Ashley-Koch A. HLA-DR15 haplotype and multiple sclerosis: a HuGE review [published online February 28, 2007]. Am J Epidemiol. 2007;165(10)1097-1109. doi:10.1093/aje/kwk118.

4. Zein G, Berta A, Foster S. Multiple sclerosis-associated uveitis. Ocul Immunol Inflamm. 2004;12(2):137-142. doi:10.1080/09273940490895344.

Financial disclosures

Dr. Reddy - None.

Ms. D. Osmanzada - None.

Ms. S. Osmanzada - None.

Dr. Hau - SEQUENOM: Speaker, Honoraria; THROMBOGENICS, INC: Other, Honoraria.

Dr. Choudhry 
- ALLERGAN, INC: Advisory Board, Speaker, Honoraria; BAUSCH + LOMB, INC: Advisory Board, Honoraria; BAYER HEALTHCARE: Consultant, Speaker, Honoraria; NOVARTIS: Advisory Board, Speaker, Grants, Honoraria; OPTOS PLC: Speaker, Honoraria.

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