Keys Flashcards
What syndromes are associated with Sarcoidosis?
(1) Löfgren’s Syndrome - Acute presentation – arthritis; erythema nodosum; bilateral hilar LN
(2) Mikulicz’s Syndrome - bilat lacrimal & salivary gland swelling 2o lymphoid neoplasia / sarcoidosis etc.
(3) Heerfordt’s Syndrome - uveo-parotid fever – chronic fever; parotid swelling; LMN VII; anterior uveitis
What are the ocular manifestations of Sarcoidosis?
SYSTEMIC: 90% manifest with bilateral hilar lymphadenopathy
OCULAR: 25-80% get ocular signs
(1) AAU – bilat, mutton fat KPs, Ant/Post Synaechia; nodules (not Berlin)
(2) Posterior Uveitis – vitritis, chorioretinitis, sarcoid spots, CNVM, h’ge
(3) Granuloma – conj, eyelid, orbit, ON, lacrimal drainage system
(4) Vascular – peripheral retinal periphlebitis, exudates (candle wax drippings)
(5) ON – swelling, granuloma, atrophy CNVM
What are the causes of scleritis?
RA; Wegener’s; relapsing polychond; PAN; sarcoidosis; SLE; HLAB27; SINS; infect (HZO, TB; Syphilis)
What are the ocular manifestations of Wegener’s granulomatosis.
Ocular involvement in 30-50%
(1) orbital inflammation
(2) episcleritis
(3) PUK
(4) AION
(5) CRAO
What is the classic clinical triad of Wegener’s granulomatosis?
(1) Small vessel vasculitis (cANCA Sn 91%, Sp 99% in active disease; Sn 60% in inactive disease)
(2) Necrotizing granulomatous inflammation of upper (95% ENT saddle nose) + lower respiratory tract (45% cavitation Air-Fluid levels)
(3) Necrotizing glomerulonephritis (18% leads to ARF - death)
What are the diagnostic features of Vogt-Koyanagi-Harada syndrome?
DEFINITION: Systemic idiopathic granulomatous condition with bilateral panuveitis; Autoimmune reaction against melanocytes, melanin & RPE
FEATURES:
(1) Vogt-Koyanagi: Skin (alopecia, poliosis, vitiligo); AAU
(2) Harada: Neuro (headache, encephalopathy, CSF pleocytosis); Auditory (tinnitus, deafness, vertigo); Post uveitis
POSTERIOR UVEITIS PHASES:
(1) Prodromal – flu-like illness, meningism - photophobia, conjunctivits, CSF pleocytosis
(2) Uveitic (few weeks) – bilat uveitis – Disc swelling, exudative RD, Dalen-Fuch’s nodules
(3) Chronic (3/12 after onset) – fundus dePigmentation (sunset glow), PPAtrophy, subretinal/OD CNVM, Poliosis
What are the clinical classification of syphilis?
(1) PRIMARY: Painless chancre (eyelid or conj) with regional LN [10-90/7 post exposure (for 4-6/52)]
(2) SECONDARY: Symmetrical maculopapular rash on palms & sole (condyloma latum); fever malaise; 6-8/52. Rare features: iridocyclitis, neuroretinitis, chorioretinitis (Natural history: 1/3 cured; 1/3 latent; 1/3 tertiary)
(3) LATENT: Asymptomatic - early vs late; punctuate retinitis rare but pathognomonic
(4) TERTIARY: Cardiovascular (10% AscAA/AR); gummas (16%; of skin & bone); neurosyphilis (6%) 1-10yrs
What are the ocular manifestations of syphilis?
(1) Cornea: IK with ghost vessels
(2) Uvea: granulomatous or non-granulomatous
(3) Iris: roseola (visible dilated capillaries); patchy hyperaemia with pink nodules
(4) Retina: vasculitis with exudation (uni/bil/multifoc); oedema (CWS); chorioretinitis (pig retinopat)
(5) Optic: Disc oedema with h’ge and exudate; ?late optic atrophy
(6) Pupils: Argyll Robinson pupil
What are the manifestations of neurosyphilis?
(1) Asymptomatic neurosyphilis: Reactive RPR /VDRL
(2) Meningovascular: MCA stroke
(3) General paresis: Frontotemporal meningoencephalitis (personality/affect/refexes/ eye/sensorium/intellect/speech)
(4) Tabes dosages: Demyelination of post columns – loss of pain/reflexes/position; ataxia; Argyll-Robertson pupil
What serological tests are available for Syphilis?
(1) VDRL or RPR: Response to Rx; current disease; used for screening clinical activity of disease
(2) FTA-ABS or TPHA: detect ever infected; used to confirm diagnosis
What are the common ocular HLA associations?
(1) HLA-A29: Birdshot choroidopathy
(2) HLA-B27: AAU (Ankylosing spondylitis 95%; Crohn’s (AAU:2%) & UC (AAU:5-12%); Reiter’s 80%; Psoriatic arthritis(AAU:7%))
(3) HLA-B51: Behçets Disease (new: IL10, IL23)
(4) HLA-DR3: Primary Sjögren’s; SLE; Myasthenia gravis
(5) HLA-DR4B1: Prolonged VKH
List causes of a hypertensive uveitis.
Viral (HSV/HZVsectorial iris atropy); toxoplasmosis; FHIC; Posner-Schlossman (CMV); Phacolytic; Steroid;
What proportion of patients with Behçet’s Disease manifest each symptom?
98-100% - Painful oral ulcers - 3x in 12/12 (last 10/7 heal without scars)
80-90% - Painful genital ulcers
80% - Erythema nodosum; Papulopustular lesion; pseudo-folliculitis; acneiform nodules
50% - Ocular signs – AAU, Post uveitis,vasculitis
50% - GI ulceration
15% - Arthritis/Joint Effusions
5-10% - CNS involvement (obliterative vasculitis, meningoencephalitis)
5-10% - Positive Pathergy Test - 20G oblique needle
What are the ocular manifestations of Behçet’s Disease?
(1) Bilat non-granulomatous no fibrinous AAU (80% bilat 20% unilat) +/- hypopyon (25%)
(2) Posterior: peri-arteritis/phlebitis (occlusive BRVO/BRAO), vitritis, retinitis, choroiditis, optic neuritis/papilloedema, CMO
What drugs can cause an anterior uveitis?
Bisphosphonates, rifabutin, cidofovir, metlpropranolol, sulphonamides