Vogt Koyanagi-Harada

1. Vogt Koyanagi-Harada Luca Cimino Ambulatorio di Immunologia Oculare Arcispedale S.Maria Nuova Reggio Emilia, Italy Luca Cappuccini Arcispedale S.Maria Nuova Reggio Emilia, Italy

2. 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.

3. 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).

4. 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 -1.75 sf in LE). • Before of this the patient never used glasses.

5. K.A. 21 YEAR OLD M Bilateral swelling optic disc

6. 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.).

7. 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)

8. K.A. 21 YEAR OLD M • But FAG showed only minimal alterations of RPE (it was substasially negative)

9. K.A. 21 YEAR OLD M

10. K.A. 21 YEAR OLD M OCT: confirmed Bilateral serous retinal detachment

11. 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 Behçet (HLA-B51+ oral aphtae).

12. Bilateral serous retinal detachment

13. K.A. 21 YEAR OLD M • To resolve this doubts the Ophthalmologist sent the patient to us… • What we do?

14. 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 -1.75 sf in LE • The fundus examination confirmed the swelling disc + showed a bilateral retinal serous detachment in both the eyes.

15. 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.

16. What I think • At this point one examination is crucial to confirm the diagnosis ?

17. Red free photos: RPE detachmentFAG: minimal alterations of RPE.

18. K.A. 3 Mar 2004 ICG 1.24 ICG 1.35

19. ICGA: irregularly shaped hypofluorescent zones at middle and late phases

20. IGCA: peripheral dahk areas

21. K.A. 21 YEAR OLD M • A lumbar puncture was performed and 178 cells /μL were noted, 100% were lymphocytes. • the diagnosis was reached within 10 days from the onset of symptoms.

22. VKH disease: definition • Bilateral granulomatous panuveitis + systemic manifestations. • Unknown etiology. • Auto-immune process against choroidal melanocytes probable.

23. VKH disease 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

24. 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 ¾ criteria = VKH (no prior ocular trauma or surgery)

25. Therapy • Initial intravenous methylprednisolone 1 g X 3 days, followed by oral prednisone 1.0 mg/kg tapered.

26. K.A. 21 YEAR OLD M • 31st March ’04: • VA was 1.0 in OU. • Resolution of serous retinal detachment

27. 31st March ’04

28. FAG showed RPE alterations 31st March ’04

29. ICGA: quasi complete resolution of signs 31st March ’04

30. ICGA: quasi complete resolution of signs

31. K.A. 19 Mag 2004

32. FAG showed increased RPE alterations

33. IGCA: complete resolution

34. K.A. 21 YEAR OLD M • He showed RPE changes • in maculararea. • VA 1.0 in both eyes.

36. 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).

37. 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.

38. 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.