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<a class="elsevierStyleCrossRefs" href="#fig0005">Figs&#46; 1 and 2</a>&#41;&#46; Autofluorescence revealed hypoautofluorescence with a hyperautofluorescent halo surrounding the detachments&#44; and fluorescein angiography showed the typical &#8220;starry sky&#8221; image with multiple leakage sites&#44; and diffusion in the late stages&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnostic criteria for Vogt&#8211;Koyanagi&#8211;Harada disease</span><p id="par0030" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">1&#46;</span><p id="par0035" class="elsevierStylePara elsevierViewall">No history of perforating trauma or prior surgeries&#46;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">2&#46;</span><p id="par0040" class="elsevierStylePara elsevierViewall">No evidence of previous eye disease&#46;</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">3&#46;</span><p id="par0045" class="elsevierStylePara elsevierViewall">Bilateral eye involvement &#40;A or B&#44; 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placoid areas of hyperfluorescence&#44; and optic nerve staining&#46;</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">&#8226;</span><p id="par0075" class="elsevierStylePara elsevierViewall">Diffuse thickening with no posterior scleritis &#40;ultrasound&#41;&#46;</p></li></ul></p><p id="par0080" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0020"><li class="elsevierStyleListItem" id="lsti0040"><span class="elsevierStyleLabel">B&#46;</span><p id="par0085" class="elsevierStylePara elsevierViewall">Late manifestations&#58;</p></li></ul></p><p id="par0090" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0025"><li class="elsevierStyleListItem" id="lsti0045"><span class="elsevierStyleLabel">&#8226;</span><p id="par0095" class="elsevierStylePara elsevierViewall">History of previous manifestations&#46;</p></li><li class="elsevierStyleListItem" id="lsti0050"><span class="elsevierStyleLabel">&#8226;</span><p id="par0100" class="elsevierStylePara elsevierViewall">Ocular depigmentation&#58; Sugiura sign or sunset glow fundus&#46;</p></li><li class="elsevierStyleListItem" id="lsti0055"><span class="elsevierStyleLabel">&#8226;</span><p id="par0105" class="elsevierStylePara elsevierViewall">Others such as numerous chorioretinal scars&#44; recurring or chronic anterior uveitis&#46;</p></li></ul></p><p id="par0110" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0030"><li class="elsevierStyleListItem" id="lsti0060"><span class="elsevierStyleLabel">4&#46;</span><p id="par0115" class="elsevierStylePara elsevierViewall">Neurological and or auditory findings&#58;</p></li></ul></p><p id="par0120" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0035"><li class="elsevierStyleListItem" id="lsti0065"><span class="elsevierStyleLabel">&#8226;</span><p id="par0125" class="elsevierStylePara elsevierViewall">Meningismus&#46;</p></li><li class="elsevierStyleListItem" id="lsti0070"><span class="elsevierStyleLabel">&#8226;</span><p id="par0130" class="elsevierStylePara elsevierViewall">Tinnitus and&#47;or hearing loss&#46;</p></li><li class="elsevierStyleListItem" id="lsti0075"><span class="elsevierStyleLabel">&#8226;</span><p id="par0135" class="elsevierStylePara elsevierViewall">Pleocytosis in cerebrospinal fluid&#46;</p></li></ul></p><p id="par0140" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0040"><li class="elsevierStyleListItem" id="lsti0080"><span class="elsevierStyleLabel">5&#46;</span><p id="par0145" class="elsevierStylePara elsevierViewall">Dermatological findings&#44; after uveitis&#58;</p></li></ul></p><p id="par0150" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0045"><li class="elsevierStyleListItem" id="lsti0085"><span class="elsevierStyleLabel">&#8226;</span><p id="par0155" class="elsevierStylePara elsevierViewall">Alopecia&#44; vitiligo&#44; poliosis&#46;</p></li></ul></p><p id="par0160" class="elsevierStylePara elsevierViewall">VKH syndrome is defined as&#58;</p><p id="par0165" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0050"><li class="elsevierStyleListItem" id="lsti0090"><span class="elsevierStyleLabel">&#8226;</span><p id="par0170" class="elsevierStylePara elsevierViewall">Complete&#44; when the patient meets criteria 1 to 5&#46;</p></li><li class="elsevierStyleListItem" id="lsti0095"><span class="elsevierStyleLabel">&#8226;</span><p id="par0175" class="elsevierStylePara elsevierViewall">Incomplete&#44; when the patient meets criteria 1 to 3&#44; plus the 4th or 5th criteria&#46;</p></li><li class="elsevierStyleListItem" id="lsti0100"><span class="elsevierStyleLabel">&#8226;</span><p id="par0180" class="elsevierStylePara elsevierViewall">Probable&#44; when only ocular criteria 1 to 3 are present&#46;</p></li></ul></p><p id="par0185" class="elsevierStylePara elsevierViewall">Therefore&#44; our patient fully met the clinical criteria established by the American Uveitis Society &#40;AUS&#41;&#44; published in 2001&#44; in sections 1&#44; 2&#44; 3A&#44; 4&#58; pleocytes in CSF&#44; and 5 alopecia&#46; Finally&#44; a diagnosis of complete acute uveitic stage VKH was made&#46;</p><p id="par0190" class="elsevierStylePara elsevierViewall">CSF showed the presence of pleocytosis&#46;</p><p id="par0195" class="elsevierStylePara elsevierViewall">Microbiological study discounted acute or latent tuberculosis infection&#46;</p><p id="par0200" class="elsevierStylePara elsevierViewall">QuantiFERON&#174;-TB gold&#58; negative&#46;</p><p id="par0205" class="elsevierStylePara elsevierViewall">CSF culture at 45 days&#58; culture negative for mycobacteria&#44; negative for bacteria&#44; and negative for mycosis&#46;</p><p id="par0210" class="elsevierStylePara elsevierViewall">Serology for hepatitis C virus&#44; hepatitis C&#44; HIV and syphilis was negative&#46;</p><p id="par0215" class="elsevierStylePara elsevierViewall">There is currently no consensus as to the therapeutic regimen for patients with VKH disease&#46;</p><p id="par0220" class="elsevierStylePara elsevierViewall">Treatment was started with 3 1<span class="elsevierStyleHsp" style=""></span>g boluses of methylprednisolone and improved VA was achieved&#58; &#46;7 in both eyes&#46;</p><p id="par0225" class="elsevierStylePara elsevierViewall">In relation to the pharmacological management of our patient&#44; it is important to highlight her history of an associated metabolic syndrome manifested by chronic hypertension&#44; type 2 diabetes mellitus treated with oral antidiabetic agents&#44; obesity and dyslipidaemia&#46; After induction treatment with intravenous boluses of corticosteroids&#44; the patient suffered metabolic decompensation complicated by sustained hyperglycaemia and hypertensive crisis&#44; therefore we decided to start early treatment with an oral immunosuppressant as a steroid saver&#44; which would also ensure induction initiated with corticosteroids to serve as bridge therapy until starting biological therapy&#46;</p><p id="par0230" class="elsevierStylePara elsevierViewall">We studied the different treatment options and straight away discounted methotrexate due to a fatty liver and hypertransaminasaemia&#44; and known prior intolerance&#46; We ruled out cyclosporine due to its potential nephrotoxic effect&#44; and the coexistence of kidney failure risk factors &#40;chronic hypertension and diabetes mellitus&#41;&#46; A major limitation to treatment in our case was the patient&#39;s obesity&#46; Therefore&#44; given our experience with the drug we decided to start mycophenolate mofetil as a steroid saver until we could establish biological therapy as the definitive treatment&#44; in this case adalimumab&#46;</p><p id="par0235" class="elsevierStylePara elsevierViewall">The patient was monitored using SD-OCT &#40;Heidelberg&#41; obtaining sequential images that showed a reduction in the serous detachments&#44; persistent impairment of the external retina&#44; and residual perimacular hyperautofluorescence&#46;</p></span></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Discussion</span><p id="par0240" class="elsevierStylePara elsevierViewall">Diagnosis of VKH disease is clinical&#46; The characteristic eye involvement is non-infectious granulomatous panuveitis&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> The presence of neurological manifestations makes an urgent differential diagnosis necessary given the possibility of infection of the CSF&#46; Therefore&#44; detailed medical history and a complete examination are compulsory&#46;</p><p id="par0245" class="elsevierStylePara elsevierViewall">RA associated with VKH disease is extremely rare&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> The most common eye involvement in RA is keratoconjunctivitis sicca&#44; which is present in 25&#37; of patients&#46; In contrast&#44; bilateral panuveitis is unusual&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a></p><p id="par0250" class="elsevierStylePara elsevierViewall">By means of CT and MRI we ruled out the presence of space-occupying&#44; vascular or inflammatory lesions&#46;</p><p id="par0255" class="elsevierStylePara elsevierViewall">By testing the CSF&#44; we found the presence of pleocytosis&#44; and the microbiological study discounted acute or latent tuberculosis infection&#46;</p><p id="par0260" class="elsevierStylePara elsevierViewall">Given the patient&#39;s history of RA&#44; ocular ultrasound ruled out posterior scleritis&#46;</p><p id="par0265" class="elsevierStylePara elsevierViewall">Eventually we made a diagnosis of complete VKH in the acute uveitic phase&#46; Our patient fully met the clinical criteria of the AUS&#44; published in 2001&#44; in sections 1&#44; 2&#44; 3A&#44; 4&#58; pleocytosis in CSF&#44; and 5&#58; alopecia&#46;</p><p id="par0270" class="elsevierStylePara elsevierViewall">Our patient responded favourably to systemic corticosteroids&#44; however&#44; due to her prior metabolic syndrome and to prevent complications associated with prolonged steroid use&#44; administration of immunosuppressant treatment was necessary from the start of the disease&#46; To achieve complete remission&#44; mycophenolate mofetil was prescribed as bridge therapy until starting a TNF inhibitor&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#8211;6</span></a></p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Conclusion</span><p id="par0275" class="elsevierStylePara elsevierViewall">Diagnosis of VKH disease is challenging&#44; and differential diagnosis is compulsory&#46; A multidisciplinary approach to autoimmune diseases enables diagnosis and prompt treatment&#44; improves the prognosis of these diseases&#44; and significantly reduces sequelae to the vital organs&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflict of interests</span><p id="par0280" class="elsevierStylePara elsevierViewall">The authors have no conflict of interests to declare&#46;</p></span></span>"
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