to report a case of frosted branch angiitis ( fba ) secondary to neuro - behet .
description , diagnosis , angiogram imaging and follow - up of a 28-year - old female with fba .
 frosted branch angiitis is a clinical term applied to three conditions : infiltration of vessels by malignant cells , and sheathing of vessels either secondary to an active disorder or subsequently to a previous inflammatory disease .
our patient 's history of two optic neuropathies and the lack of demyelinating signs in neuroimaging made us consider fba in the context of neuro - behet .
recognition of the category of fba from the clinical signs is essential to establish the correct diagnosis and prescribe the appropriate treatment .
frosted branch angiitis ( fba ) has been classically considered a disorder characterized by acute panuveitis with vasculitis .
fba onset is sudden and patients complain of painless vision loss , floaters and photopsias .
severe vascular sheathing , as seen on a fundus image , is characteristic of this disorder .
it is generally associated with retinal edema and deep retinal hemorrhages . since its first description in 1976 , the term  frosted branch angiitis has been indistinctly used in the literature to describe different conditions where fundus imaging discloses an unusual thick sheathing surrounding the retinal vessels .
a 28-year - old female presented to our department complaining of progressive visual loss in both eyes ( ou ) for 2 days .
she had a personal history of anterior ischemic optic neuropathy in the right eye ( od ) 5 years ago .
the ophthalmic examination disclosed decreased visual acuities ( va ) ou : 20/200 od and 20/120 in the left eye ( os ) .
the intraocular pressure ou was normal along the process . on slit - lamp examination ,
inflammation was seen in the anterior chamber ( cells 2 + ) and in the vitreous ( cells 2 + ) ou .
fundus examination showed severe sheathing of retinal vessels , retinal hemorrhages , roth 's spots and macular edema ou ( fig .
the intense and acute flare of vasculitis led us to consider a case of fba , and a set of proofs were requested .
serology for hepatitis b and c viruses , human immunodeficiency virus ( hiv ) , syphilis , cytomegalovirus ( cmv ) , epstein - barr virus ( ebv ) , toxoplasma and mantoux test were negative as well as anti - nuclear and anti - neutrophil cytoplasmic antibodies .
next , computerized tomography ( ct ) and magnetic resonance imaging ( mri ) were requested to rule out involvement of the central nervous system and demyelinating disease , respectively . neither did disclose any abnormality .
finally , the pathergy test was performed and was positive ( erythematous papules 3.5 mm 48 h after the prick ) .
the suspected diagnosis was fba secondary to behet 's disease and neuroimaging did not demonstrate demyelinating signs .
( weight : 65 kg , cyclosporine dose : 4.6 mg / kg / day ) , dexamethasone eye drops each 2 h and homatropine eye drops t.i.d .
only 24 h after starting steroids , the inflammation of the vessels improved considerably ( fig .
improvement was also seen on fluorescein angiography ( fa ) which disclosed macular edema ou , no vasculitis , and late peripapillary staining 1 day after the inflammation started .
seven days after the regimen started , the patient remained with the same va ou , but the inflammation enhanced both in the anterior chamber ( cells 0.5 + ) and in the vitreous ( cells 0.5 + ) .
moreover , funduscopy disclosed a macular star , temporal atrophy on the optic nerve and temporal peripheral hemorrhages associated with exhausted vessels at that level ou ( fig .
the patient was kept on that regimen until 3 weeks later when steroids were tapered slowly during 3 months .
four months after her problems started , the patient presented a significant va improvement ( od 20/20 and os 20/20 ) and no intraocular inflammation .
ocular fundus imaging disclosed some hard exudates where macular stars were and mottling of the retinal pigment epithelium at the fovea level .
fa only showed an ischemic zone in the temporal retina which produced a peripheral scotoma in the visual fields performed ( fig .
2 ) . the patient remained relapse - free for over 1 year , then immunomodulatory therapies ( imts ) were slowly tapered .
however , after 9 months when she was on 100 mg a day of that regimen , decreased vision and scotomas od were noted . after a complete examination and imaging tests , she was diagnosed with ischemic posterior optic neuropathy .
the patient started again with steroids , and cyclosporine was increased until 300 mg a day , which totally restored va in 7 days .
thus , our patient is currently diagnosed with and followed - up like a case of neuro - behet .
first described this disorder in 1976 in a 6-year - old boy with severe bilateral white sheathing of all retinal vessels , which inspired the term  frosted branch angiitis. just around 60 cases worldwide   have been published up to date .
the prevalence of this disorder depends on the country : in japan affected patients are usually aged 616 years . on the other hand
, the reports on cases published from other countries , mostly caucasian individuals , described a late onset of this disorder ( usually in the third decade ) .
there are several agents and disorders which have been postulated to be causal agents or triggering factors of this entity ( table 1 ) .
the term  frosted branch angiitis became confusing over time because it was used to denote an specific disorder as well as just a kind of funduscopic image .
the term remained confusing until 1997 when kleiner   postulated a new differentiation and classification .
the first one ,  frosted branch appearance , denotes a funduscopic image in entities such as leukemia and lymphoma . in these disorders ,
this appearance is due to infiltration of the vessel walls by malignant cells and not by an inflammatory process .
the second category of fba is called  frosted branch response , in which a real vasculitis secondary to active infections or autoimmune diseases is observed ( table 1 ) .
the third category is  acute idiopathic frosted branch angiitis , which affects healthy young individuals who have had a infectious precedent which is held capable of causing a subsequent vascular inflammation .
this precedent responsible for triggering that secondary vasculitis usually is a flu - like illness , but hepatitis b vaccination , mumps , swine flu vaccination , and bacterial infection can also be responsible for initiating the inflammatory process .
therefore , a hypersensitivity to microbial antigens is the hypothesis most accepted in this subtype of fba .
1 ) . if a specific causal agent is found , it will have to be treated at the same time . on the other hand ,
if an autoimmune disease is the cause of the fba , imts will be the first choice . according to kleiner 's classification
skin folliculitis , recurrent aphthous ulcers , positive pathergy and hla b51 suggest the possibility of an underlying behet 's disease .
furthermore , the patient met the requirements according to the international criteria for the diagnosis of behet 's disease   and cases secondary to this disease have been published .
in fact , the two cases of optic neuropathy support the theory of neuro - behet
. the second flare of neuritis when cyclosporine was tapered would also support the diagnosis .
moreover , there have already been cases of optic neuritis in neuro - behet [ 6 , 7 ] . on the other hand , the typical neurologic manifestations and neuroimaging signs of neuro - behet  
therefore , our patient will be monitored closely and biologic therapies are , up to the present , stopped .
the patient is currently on 300 mg of cyclosporine a day ( weight : 65 kg , dose : 4.6 mg / kg / day ) and is asymptomatic again .
two other cases of fba secondary to behet 's disease have already been published [ 9 , 10 ] .
one case was treated with oral prednisolone and colchicine , which proved effective to control the inflammation .
the other case was a pregnant patient in whom it was not possible to use any immunomodulatory therapy and where the disease spontaneously resolved . in our case
we could have used other imts but we decided to use cyclosporine because we had had good results with it in behet cases . in summary ,
recognition of the correct category of fba from the clinical signs is essential to prescribe appropriate treatment . that is , in frosted branch appearance cases
, therapeutic procedures to treat the malignant disorder should be the main goal . in frosted branch response cases , finding and treating
for acute idiopathic fba cases , treating the ocular finding in order to avoid later complications should be our main aim .
thus , we have to keep in mind the characteristic fundus images for each category in order to choose the proper treatment .
 purposeto report a case of frosted branch angiitis ( fba ) secondary to neuro-behet.methodsdescription , diagnosis , angiogram imaging and follow - up of a 28-year - old female with fba.resultsfrosted branch angiitis is a clinical term applied to three conditions : infiltration of vessels by malignant cells , and sheathing of vessels either secondary to an active disorder or subsequently to a previous inflammatory disease . 
 our patient 's history of two optic neuropathies and the lack of demyelinating signs in neuroimaging made us consider fba in the context of neuro-behet.conclusionrecognition of the category of fba from the clinical signs is essential to establish the correct diagnosis and prescribe the appropriate treatment . 
Purpose
Methods
Results
Conclusion
Introduction
Clinical Case
Discussion
Disclosure Statement