posterior uveitis

• uveal tissue

  • middle layer of the eye between sclera and retina
  • has ciliary body, iris, choroid and RPE
  • name derived from grape as looks like grape when dissected
  • each section has own function
  • contains complex immune cells as well as pigment containing melanocytes
  • classification of units - standardisation of uveitis nomenclature working group guidance, international study group, categorised based on anatomy, aetiology and time of clinical activity

  
• positions of uveitis

  • classified according to primary sight of inflammation

  anterior

  • anterior chamber

  intermediate

  • vitreous and pars plana

  posterior

  • retina and choroid

  pan uveitis

  • entire uveal tract

  
• causes of uveitis

  • infective - toxoplasmosis, TB and syphillus
  • non infectious - undifferentiated auto immune, reactive
  • masquerade - underlying disease such as malignancy
• timing of uveitis

  • onset is sudden or insidious
  • duration - limited to 3 months or less or persistent
  • clinical course
  • acute - sudden onset and limited duration
  • recurrent - repeated episodes separate by inactive periods
  • chronic - persistent duration with relapse in less than 3 months
  • remission - no acitivity for more than 3 months
• intermediate uveitis 


  • SUN classification
  • diagnosis is clinical 50% idiopathic, can be multiple sclerosis, sarcoidosis, Lyme disease, syphillis, TB
  • accounts for 15% of all uveitis
  • variable course
  • blurred vision, floaters, normally painless, no redness
  • VA dependent on severity and presence of CMO
  • have spill over inflammation in anteiror chamber, vitreous cells, haze and condensation
  • snow balls - white focal collections of inflammatory cells and exudates
  • snow banking - peripheral fibrovascular plaque
  • peripheral periphlebitis - mild peripheral vascular sheathing
  • CMO in 50%
  • long term ERM common, cataract caused by inflammation or steroids, secondary glaucoma

  
• maquarades

  • lymphoma
  • endophthalmosis
  • toxoplasmosis
  • investigate bloods - ESR, CRP, U&E, ACE
  • OCT - cystoid macular odema
  • OCT A - reduced blood flow
  • FA - peripheral shut down

  treatments

  • varies between centres
  • treat underlying causes
  • steroids - oral, intravitreal - ozurdex, iluvien
  • second line immunosupression - mycofenolate, mofetil, tarcolimus
  • cataract and glaucoma management

  can have peri ocular steroid injection, non steroidal anti inflammatory, peripheral cyrotherapy, retinal laser if siginificant shut down, vitrectomy

  
• posterior uveitis

  • loss of vision
  • painless
  • floaters
  • photopsia
  • blurring
  • mild vitritis
  • posterior pole lesions
  • ill defined patches - creamy, white, grey, depending on cause
  • peripheral vasculature shut down
  • neovascular changes
  • CNV
• choroiditis

  • inflammation of choroid
  • inflammatory foci at level of choroid and RPE
  • isolated choroiditis is rare

  
• retintis

  • inflammation of retina
  • conjunction with choroiditis and vasculitis
  • demarcated areas of infection
  • leave pigmented atrophy when no longer active
  • haemorrhage and exudates often apparent

  
• vasculitis

  • inflammation of retinal vasculature
  • unusual in isolation
  • peri vascular sheathing
  • exudates
  • cotton wool spots
  • occlusion
  • neovascular changes

  
• neuroretinitis

  • inflammation of optic nerve
  • peripapillary area and neural retina
  • lipid rich exudate may form macular star
  • can be clinically similar to hypertensive retinopathy

  
• referral for posterior uveitis

  • urgent
  • contact local centre
  • often local guidelines for time frames to be seen
  • needs specialist input
  • empirical investigation and treatment started on first visit
• causes of posterior uveitis

  • wide range
  • infectious
  • autoimune
  • secondary
• infectious causes - parasitic

  • toxoplasmosis - may be symptomatic , can be congenital from mother or aquired from soil, cat faces, undercooked meat
  • toxocara

  
• infectious causes - viral

  • acute retinal necrosis - herpes simplex and zoster
  • CMV retinitis - immunocompromised px
  • progressive retinal necrosis - PORN , herpes zoster

  
• other causes

  • fungal candida - endogenous
  • presumed ocular histoplasmosis syndrome POHS as seen in HIV
  • bacterial - TB, syphilis, lyme

  
• autoimmune causes

  • multiple sclerosis
  • sarcodosis
  • VKH
  • bechets
  • miscallaneous idiopathic chorioretinopathies
  • sympathetic opthalmia
• multiple sclerosis

  • neuroinflammatory condition causing unmylination of neurones
  • most common ocular association is optic neuritis
  • can be associated with IU and play plantis
  • MRI brain scan part of screening
• sarcoidosis

  • autoimmune systemic condition
  • collections of anti inflammatory cells produce granulomas
  • can exist throughout body
  • ocular complications include granulomatous anterior uveitis
  • Intermediate and posterior uveitis
  • chest x ray shows hilar lymphadenopathy
  • blood shows raised serum ACE
• VKH

  • vogt Koyangi Harada syndrome
  • multisystem autoimmune disease of melanocyte containing tissues
  • prodromal phases include neurological manifestations
  • acute uvietic stage includes dalen fuchs nodules, panuveitis leading to exudative detachment
• bechets disease

  • idiopathic multisystem disease
  • recurrent mouth ulcers, genital ulceration and uveitis
  • vasculitis always present – involves small medium and large veins and arteries
  • Central nervous system and cardiovascular complications are common and mortality is 5% at 5 years
  • Disease common in patients from countries along the silk route
  • Inflammation throughout the entire uveal tract
• white dot syndromes

  one subgroup

  • MEWDS - multiple evanescent white dot syndrome
  • AIBSE - acute idiopathic blind spot enlargement
  • APMPEE - acute posterior multifocal placoid pigment
  • serpigenious
  • acute macular neuroretinopathy
  • AZOOR - acute zonal occult outer retinopathy
  • PIC - punctuate inner retinopathy
  • MFCPU - multifocal choroiditis and pan uveitis
  • SFU - progressive sub retinal fibrosis and uveitis
  • includes bird shot retinopathy
  • others are rare
• MEWDS= multiple evanescent white dot syndrome

  • chorioretinopathy

  
• AMPEE - acute posterior multifocal placoid pigment epitheliopathy

  • chorioretinopathy

  
• serpingenous

  • chorioreitnopathies

  
• AZOOR - acute zonal occult outer retinopathy

  • chorioretinopathy

  
• PIC - punctuate inner chorioretinopathy 


  • see image

  
• SFU - progressive sub retinal fibrosis and uveitis 


  • see image

  
• MFU - multifocal choroiditis 


  see image

  
• bird shot chorioretinopathy 


  • see image

  
• secondary causes

  • latrogenic - due to medication such as chemotherapy
  • masquarade - lymphoma
  • paraneoplastic syndromes - cancer associated retinopathy, bilateral diffuse uveal melanocytotic proliferation BDUMP, melanoma associated retinopathy
• investigations into secondary causes

  • bloods
  • systemic x ray, CT, MRI brain
  • OCT
  • OCTA
  • ICG
  • FFA

  
• treatment

  • treat any underlying cause - anti biotics, anti MS meds, antivirals, anti fungals
  • steroids - need admission for high dosage to initiate treatment - exclude infection
  • ozurdex , illuvien
  • Anti VEGF for CNV
  • second line treatment - mycophenolate, methotrexate, tacrolimus
  • adulimumab, rituximab
• prognosis

  • extremely variable based on cause
  • if macula not involved then good prognosis
• summary

  • SUN and ISUG classify according to location, aetiology, timing of clinical activity
  • causes are infective, non infective or autoimmune, secondary or masqurade
  • treatment includes treating underlying cause, immunosuppression, steroids local or systemic, second line immunotherapy, biologic therapy
  • prognosis variable depending on cause and area
  • wide range of spectrum of disease presentation and activity
  • early referal as significant risk of irreversible sight loss
  • treatment in specialist centre needed