Vogt Koyanagi-Harada Luca Cimino Luca Cappuccini
Ambulatorio di Immunologia Oculare Arcispedale S.Maria Nuova Reggio
Emilia, Italy LucaCappuccini K.A. 21 YEAR OLD M January 2004:
headache, flu-like illness, vertigo.
He thought about his work (welder). He followed therapy with non
steroidal drugs. K.A. 21 YEAR OLD M Unfortunately 5 days later his
vision decreased and
He went to Ophthalmic Emergency Service of a city University near
our Hospital ( was a holiday!). The patient was visited and because
of his history (headache before visual loss) Brain NMR was
performed (also because he showed bilateral swelling optic disc).
K.A. 21 YEAR OLD M But brain NRM was negative for tumor and/or
multiple sclerosis signs and/or CNS vasculitis. Natural VA was 0.4
in both eyes ( 0.6 with -2 sf in RE and 0.7 with sf in LE). Before
of this the patient never used glasses. K.A. 21 YEAR OLD M
Bilateral swelling optic disc K.A. 21 YEAR OLD M They decided to
perform a generous diagnostic sierologic work-up for uveitis (and
everything) with negative results except a positive HLA-B51 (well
related also at oral aphthae showed from the pt.). K.A. 21 YEAR OLD
M For a better evaluation the patients underwent a Fluorangiography
(also to have time to think about a possible diagnosis) K.A. 21
YEAR OLD M But FAG showed only minimal alterations of RPE (it was
substasially negative) K.A. 21 YEAR OLD M K.A. 21 YEAR OLD M OCT:
confirmed Bilateral serous retinal detachment K.A. 21 YEAR OLD M
What about the clinical diagnosis?
bilateral central serous chorioretinopathy (may be due to stress of
saudade: the pt live abrod far of his land). Primary inflammatory
choriocapillaropathy (a flu-like episode before visual loss +
myopic eyes). Abortive form of Behet (HLA-B51+ oral aphtae). serous
retinal detachment
Bilateral serous retinal detachment K.A. 21 YEAR OLD M To resolve
this doubts the Ophthalmologist sent the patient to us What we do?
K.A. 21 YEAR OLD M The anterior segment was uninflammed in both the
eyes. VA was 0.6 with -2 sf in RE and 0.7 with sf in LE The fundus
examination confirmed the swelling disc + showed a bilateral
retinal serous detachment in both the eyes. What I think 1.
Posterior exudative uveitis,bilateral.
2. Neurol. signs (hedache + vertigo ). 3. Cutaneous signs
(alopecia). 4. No history of penetrating eye trauma or surgery. 5.
Negative brain NRM. What I think At this point one examination is
crucial to confirm the diagnosis ? Red free photos: RPE detachment
FAG: minimal alterations of RPE. K.A. 3 Mar 2004 ICG 1.24 ICG 1.35
ICGA: irregularly shaped hypofluorescent zones at middle and late
phases IGCA: peripheral dahk areas K.A. 21 YEAR OLD M A lumbar
puncture was performed and
178 cells /L were noted, 100% were lymphocytes. the diagnosis was
reached within 10 days from the onset of symptoms. VKH disease:
definition
Bilateral granulomatous panuveitis + systemic manifestations.
Unknown etiology. Auto-immune process against choroidal melanocytes
probable. VKH disease Prodromal stage: headache, flu-like illness,
vertigo
Clinical stages : Prodromal stage: headache, flu-like illness,
vertigo Ocular exudative phase Chronic relapsing phase: chronic
irido-cyclitis and recurrences 4. Convalescent stage : sunset glow
fundus: choroidal depigmentation, RPE alterations, scars of
Dalen-Fuchs nodules, cutaneous signs VKH disease: diagnostic
criteria
AUS criteria ( despite new criteria in 2001, still very practical)
: 1. Iridocyclitis, bilateral 2. Posterior exudative uveitis,
bilateral 3. Inflammatory neurol. signs (meningismus, CSF
pleiocytosis, tinnitus) 4. Cutaneous signs (vitiligo, alopecia,
poliosis) Presence of criteria = VKH (no prior ocular trauma or
surgery) Therapy Initial intravenous methylprednisolone 1 g X 3
days, followed by oral prednisone 1.0 mg/kg tapered. Resolution of
serous retinal detachment
K.A. 21 YEAR OLD M 31st March 04: VA was 1.0 in OU. Resolution of
serous retinal detachment 31st March 04 FAG showed RPE
alterations
31st March 04 ICGA: quasi complete resolution of signs
31st March 04 ICGA: quasi complete resolution of signs K.A. 19 Mag
2004 FAG showed increased RPE alterations IGCA: complete resolution
K.A. 21 YEAR OLD M He showed RPE changes in macular area.
VA 1.0 in both eyes. Follow-up After 2 year the patient showed no
recurrenries (VA is full in both eyes). He showed minimal
alterations of RPE. He followed steroid therapy with 5 mg/die of
oral prednisone. He showed no recurrencies of systemic symptoms
(headache and hearing loss). Conclusion Early treatment of VKH may
prevent its progression into the chronic stage of disease. Goto H.,
Rao N. : Int Ophthalmol Clin 1990 The exact time interval from the
onset of the symptoms to the initiation of adequate treatment in
order to prevent chronic evolution is not known. Conclusion The
proportion of cases for which early and adequate therapy can
prevent chronic evolution is also not known. Unfortunately, a delay
of days, weeks or months cannot be avoided either because of
delayed consultation of the patient or delayed diagnosis of the
disease because of incomplete clinical picture at onset.