Author: Satabdi Nanda (India)
Co-authors: Kalpana Babu Murthy, Dhwani Shah, Rutul Shah
Purpose
To describe an unusual association of Probable Vogt-Kayanagi-Harada syndrome in a patient of Multiple Sclerosis. VKH spectrum is a multisystemic disorder autoimmune in nature, targeting melanocytes by a Th1 lymphocyte mediated action. It involves all pigmented structures like eye, inner ear, skin, meninges and hair. Bilaterality and absence of any history of trauma or intraocular surgery are diagnostic. Multiple Sclerosis is a chronic, inflammatory disorder involving the CNS, also autoimmune in pathogenesis , targeting myelin in axonal nerve sheaths. Association of uveitis with MS is commonly seen, with intermediate uveitis being the most common, followed by anterior uveitis, retinal vasculitis and panuveitis.
Setting/Venue
Clinical case report at a tertiary eye care centre in South India. A 38 year old female with demyelinating disease was referred to us for an uveitis opinion from a higher neuromedicine centre. She complained of progressive blurring of vision in both eyes since three months, with pain, redness, photophobia in both eyes since ten days. Since three years, she had numbness and tingling along with recurrent headaches with past history of panuveitis one year back. Neuroimaging showed bilateral frontoparietal white matter changes s/o demyelination. VEP and CSF studies were normal. She had been administered three loading doses of IVMP.
Methods
CBC, CRP, urinanalysis, ACE, serum Creatinine and CXR were normal. Erythrocyte sedimentation rate was raised (42 mm/hr). Anti- HIV 1 and 2, TPHA, Mantoux test were negative. Serum prolactin, thyroid profile were normal. Repeat MRI scan showed multiple T2/FLAIR hyperintense lesions in white matter and SC lesions.VFT showed bilateral generalized depression with centrocaecal scotoma.Repeat VEP showed prolonged P100 latency(Right Eye:115.70 ms,Left Eye:140.40ms). Serum ANA, ANCA, RA, NMO-MOG antibodies were negative. Repeat CSF analysis showed Oligoclonal bands.CSF ACE,NMO-MOG antibodies were absent. The diagnosis was established as MS as per revised McDonald Criteria. At presentation,BCVA was 6/18 OD and CFat1metre OS, shallow irregular AC, posterior synechiae, granulomatous KPs, cells and flare, hyperemic and edematous discs in both eyes. IOP was 12mm and 26mm of Hg in OD and OS.UBM showed malrotation of the inflamed and boggy ciliary body, with shallow AC on AS-OCT. USG B- scan showed choroidal thickening with disc edema.OCT showed vitreous cells, disc edema, multiple areas of shallow exudative retinal detachment over the posterior pole, choroidal folds, RPE undulations, Intraretinal fluid and hyperreflective dots in both eyes.The complete picture was suggestive of a Probable VKH presentation. FFA was not an option owing to the non-dilating pupil and cataractous lens.
Results
She was administered 4 loading doses of IV Methyl Prednisolone 1gm following which the vision in left eye dramatically improved to 6/24 along with resolution of inflammation with decrease in pain, photophobia and congestion in both eyes.Topical antiglaucoma medications (Brinzotim) was started. Repeat UBM scans showed resolution of malrotation of the ciliary body , along with opening up of the angles and reduction in IOP. Affordability being a factor, she was started on azathioprine 50 mg twice daily after discussion with the treating neurologist. Systemic corticosteroids were also started (40 mg per day). Over 3 weeks of immunosuppression coupled with oral steroids, the exudative detachments had resolved and choroidal folds had largely reduced in both eyes. The patient is doing very well with a visual acuity of 6/9 and 6/9P in right and left eye respectively at one month review. We plan to keep her on prolonged immunosuppression with very slow tapering of oral steroids.
Conlusions
This presentation of a probable VKH with MS is quite atypical and has only been documented twice in literature. MS commonly presents as intermediate uveitis, anterior uveitis or retinal vasculitis. In 2007, Montero et al. documented a similar case in a 34year female with MS who resented with complete VKH and was treated effectively with Interferon b1A and sytemic corticosteroids. Alberto et al. in 2020 published about a 35year female who presented with VKH and was later diagnosed as MS. she was treated with IVMP, Azathioprine with meticulous tapering. Options of treatment include biologicals, rituximab or azathioprine. the cost being as important factor in our case, azathioprine was chosen. HLA typing can also be considered in such cases. Careful choice of immunosupressants and long term follow up are of important value in such cases.
Financial Disclosure
NIL
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