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  • 1.  Acute VKH with sudden vision drop to HM and steroids not working

    Posted 2 hours ago

    Hi Dear All，

    I have a patient for whom I would appreciate your opinions. 

    A previously healthy 32-year-old female with acute VKH is on oral prednisone 50 mg daily for two weeks. FFA and OCT findings are typical for VKH, with a few cells in the anterior chamber. Repeat OCT shows significant improvement, with only subfoveal fluid. However, the patient reports worsening vision, and progress within a few days, her visual acuity drops from 20/50 to HM in both eyes, without having received any other immunosuppressive agents. Orbital and brain MRI w/o contrast are unremarkable. CBC and basic metabolic panel show no abnormalities. Serum AQP4 and MOG antibodies are negative. Syphilis, HIV, ACE, and ANA are also negative. Chest x-ray is normal. The uveitis specialist added mycophenolate later. 

    When I saw the patient, visual acuity was HM in both eyes, both pupils were 4 mm with no reaction to light, the anterior chamber was quiet, and the fundus appeared normal. A full-field ERG showed nearly undetectable waveforms. I ordered anti-retinal antibodies but the results are still pending. I am considering IVMP 1 g, but I'm not sure whether steroid pulse therapy will be effective in her case. Should PLEX or IVIG be prioritized instead? Is a PET-CT also indicated? 

    Any input is welcome.

    Thanks!



    ------------------------------
    Yan
    ------------------------------


  • 2.  RE: Acute VKH with sudden vision drop to HM and steroids not working

    Posted an hour ago
    Hi Yin,

    What a tough case. This process doesn't make sense. Was the Prednisone dose lowered and then the patient got worse?

    If the full field ERG is reliable and there truly is non-detectable waveforms, then there has to be widespread retinal photoreceptor dysfunction which should not occur acutely in the setting of a normal fundus. If the patient is worse on steroids, you need to reevaluate the diagnostic process. I would repeat the fluorescein angiogram along with an ICG angiogram and fundus autofluorescence as well as macular OCT. 

    Getting worse on high dose oral steroids, especially if initially responding would not be consistent with VKH, I would be highly concerned for an infectious process and you may need to do anterior chamber and or vitreous biopsy for viral PCRs and consider lumbar puncture for the same.  TB and sympathetic ophthalmia / sarcoid can mimic VKH as well syphllilis and lymphoma, although sympathetic ophthalmia and sarcoid should not worsen on steroids.

    Mycophenolate will take 2-4 weeks or more to start working and it does not sound like this patient has weeks to wait. If infection is ruled out, the next steps would be IV steroids and consideration of cyclophosphamide given the rapid decline but it helps if you know what you are treating.


    Best of luck to you and your patient,

    Drew



    Original Message


  • 3.  RE: Acute VKH with sudden vision drop to HM and steroids not working

    Posted an hour ago

    Hi Drew，

    Thanks for the opinion. It occurred while maintaining a dose of 50mg of prednisone, without any dose lowering. The fundus examination showed significant improvement, which means the ophthalmic findings clearly indicate a response to steroids. There were multifocal neurosensory retinal detachments and thickened, edematous choroid before using steroids. And now there is only subfoveal fluid, basically normal fundus. The anterior chamber cells have also resolved. She has no history of any ocular surgery or trauma. Currently, there is a symptom-sign dissociation.

    Best,

    Yan





    Original Message


  • 4.  RE: Acute VKH with sudden vision drop to HM and steroids not working

    Posted an hour ago
    Hi Yan,

    If there is photoreceptor dysfunction (as evidenced by the ERG l) and the retina looks okay, you have to look in the choroid (which provides oxygen / nutrients to photoreceptors) which means ICGA / FFA.

    Have to wonder about choroidal ischemia / vasculitis / infection.




    Original Message


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