Eduovisual
Special Senses & Otolaryngology
Uveitis: workup and management
— Anterior (iritis, iridocyclitis) — most common (~60%), inflammation in anterior chamber
— Intermediate (pars planitis) — vitreous is primary site
— Posterior — retina/choroid involvement
— Panuveitis — all segments inflamed
— Unilateral, deep aching pain (not surface burning)
— Photophobia is severe and reproducible with consensual light reflex (light in unaffected eye still hurts the inflamed one) — pathognomonic for anterior uveitis
— History of systemic disease: ankylosing spondylitis, IBD, psoriatic arthritis, reactive arthritis, sarcoidosis, Behçet, JIA, syphilis, TB, HSV/VZV, HIV
— Painless floaters + blurred vision → think intermediate or posterior
— Acute (<3 months, sudden onset) — typical of HLA-B27 anterior uveitis
— Recurrent — repeated episodes with >3-mo quiescence off therapy
— Chronic — relapse within 3 months of stopping therapy
Board pearl: A patient with low back pain stiffness and a painful red eye with photophobia → suspect HLA-B27-associated acute anterior uveitis (ankylosing spondylitis). The eye complaint may precede joint diagnosis by years — don't miss the systemic workup.
— Acute unilateral eye pain, redness, photophobia, tearing, blurred vision
— Miotic (small) pupil, sluggishly reactive
— Vision typically only mildly reduced unless hypopyon or synechiae present
— Discharge is absent (helps distinguish from conjunctivitis/bacterial keratitis)
— Painless floaters ("cobwebs," "snowballs") with progressive blurred vision
— Minimal redness; eye looks deceptively quiet externally
— MS association in adults — ask about prior optic neuritis, paresthesias, weakness
— Decreased visual acuity, scotomata, photopsias
— Often bilateral; painless unless panuveitis
— Higher likelihood of infectious or sight-threatening etiology
— Joints/back: morning stiffness, sacroiliitis (spondyloarthropathy)
— GI: bloody diarrhea, weight loss (IBD)
— Skin/GU: psoriasis, urethritis, oral/genital ulcers (Behçet, reactive arthritis)
— Pulmonary: cough, dyspnea, erythema nodosum (sarcoidosis)
— Sexual history & risk factors: syphilis, HIV (CD4 count if known)
— Travel/exposures: TB risk, undercooked meat or cat litter (toxoplasmosis)
— Pediatric: ANA-positive oligoarticular JIA → bilateral chronic asymptomatic anterior uveitis
— Trauma/surgery: recent intraocular surgery → endophthalmitis, sympathetic ophthalmia
— Medications: rifabutin, bisphosphonates, cidofovir, checkpoint inhibitors can cause uveitis
— Hypopyon (Behçet, HLA-B27, endophthalmitis)
— Severe vision loss
— Posthesion of any intraocular procedure within 6 weeks
Key distinction: Anterior uveitis = pain + photophobia + miotic pupil + clear discharge-free eye. Conjunctivitis = itching/burning + discharge + normal pupil + normal vision. The painful consensual photophobia (light in fellow eye triggers pain in affected eye) reliably localizes inflammation to the iris/ciliary body.
— Ciliary flush (circumcorneal injection)—deep violaceous ring at limbus; distinguishes anterior uveitis from conjunctivitis (which shows diffuse, peripheral injection)
— Pupil miosis with sluggish reactivity
— Visual acuity (always document Snellen both eyes)
— Cells and flare in anterior chamber—graded 0–4+ per SUN criteria; cells = inflammatory cells, flare = protein leakage
— Keratic precipitates (KPs): inflammatory cells on corneal endothelium
· Fine/stellate → viral (HSV, VZV), Fuchs
· Mutton-fat (large, greasy) → granulomatous (sarcoidosis, TB, syphilis, sympathetic ophthalmia, VKH)
— Hypopyon: layered WBCs in anterior chamber—Behçet, HLA-B27 acute anterior uveitis, endophthalmitis
— Posterior synechiae: iris adhesions to lens, cause irregular pupil and risk angle-closure glaucoma
— Busacca/Koeppe nodules: iris nodules in granulomatous disease
— Vitreous cells, snowballs (vitreous aggregates), snowbanking on pars plana
— Chorioretinal lesions, vasculitis (frosted-branch angiitis in CMV)
— Toxoplasmosis: focal retinitis adjacent to old pigmented scar ("headlight in fog")
— CMV retinitis: hemorrhagic "pizza pie" lesions in HIV with CD4 <50
— Optic disc edema in posterior/panuveitis
— Often low initially (ciliary body shutdown reduces aqueous production)
— May become elevated with trabeculitis or synechiae-induced angle closure
CCS pearl: Order "ophthalmology consult, same day" and "visual acuity" immediately. Tonometry and slit-lamp will be done by the consultant—but document VA yourself; it's the single most important triage number that flags emergent referral.
— No mandatory workup—empirical treatment by ophthalmology is acceptable if classic and isolated
— Workup triggered by: bilateral, recurrent, granulomatous, posterior/intermediate, severe, or systemic symptoms
— CBC with differential — leukocytosis (infection), eosinophilia (parasitic), cytopenias (lymphoma masquerade)
— ESR, CRP — nonspecific inflammation
— Comprehensive metabolic panel — baseline before immunosuppression
— HLA-B27 — if anterior, acute, recurrent, unilateral, especially with back/joint sx
— ACE and lysozyme — sarcoidosis (low sensitivity; supportive, not diagnostic)
— RPR + treponemal test (FTA-ABS or TP-PA) — syphilis can cause any pattern; mandatory in any uveitis workup
— QuantiFERON-TB Gold or PPD — required before starting biologics/steroids long-term
— HIV — universal screening; opportunistic infections shift differential
— ANA — especially pediatric to assess JIA-associated uveitis risk
— Toxoplasma IgG/IgM, Bartonella, Lyme (endemic), Brucella, HSV/VZV PCR if atypical
— CXR or chest CT — bilateral hilar adenopathy (sarcoid), TB, malignancy
— Sacroiliac MRI or X-ray — if back pain and HLA-B27 positive
— MRI brain — intermediate uveitis to evaluate for MS (periventricular plaques)
— OCT — detect cystoid macular edema (CME), the chief cause of vision loss
— Fluorescein angiography — retinal vasculitis, leakage
— B-scan ultrasound — if media opacity prevents fundus view
Step 3 management: Always send RPR + treponemal + HIV + QuantiFERON in any non-trivial uveitis—syphilis is the "great masquerader" of uveitis and is curable; missing it is a board favorite. TB screening also gates safe initiation of systemic immunosuppression.
— Pursue tissue or fluid sampling guided by anatomy and suspected cause
— Send for PCR (HSV-1/2, VZV, CMV, Toxoplasma) and cytology
— Indication: suspected viral anterior uveitis with elevated IOP, refractory posterior uveitis, suspected intraocular lymphoma
— Goldmann–Witmer coefficient — intraocular vs serum antibody ratio for toxoplasmosis confirmation
— Suspected vitreoretinal lymphoma (older patient, bilateral, steroid-responsive but relapsing, CNS sx) — send for flow cytometry, IL-10:IL-6 ratio (>1 favors lymphoma), MYD88 mutation
— Atypical infectious uveitis after failed empiric therapy
— Conjunctival nodule, lacrimal gland, mediastinal/hilar node, skin lesion
— Non-caseating granulomas → sarcoidosis
— Caseating granulomas with AFB → TB
— Wide-field fundus photography and angiography — peripheral vasculitis (Behçet)
— Indocyanine green angiography — choroidal pathology (Vogt-Koyanagi-Harada, birdshot chorioretinopathy)
— Enhanced-depth OCT — choroidal thickness in VKH
— Vogt-Koyanagi-Harada (VKH): bilateral panuveitis + meningismus + vitiligo/poliosis + alopecia + dysacusis—LP shows lymphocytic pleocytosis
— Behçet: clinical diagnosis (oral + genital ulcers + uveitis + skin lesions + pathergy)
— Birdshot chorioretinopathy: HLA-A29 (>95% sensitive), middle-aged adults, cream-colored fundus lesions
Board pearl: A patient with bilateral granulomatous panuveitis + tinnitus + alopecia + hearing loss + vitiligo = VKH; treat aggressively with high-dose systemic steroids early to prevent sunset-glow fundus and permanent visual loss.
— Untreated infectious uveitis (HSV keratouveitis, syphilis, toxoplasmosis, TB, endophthalmitis) worsens with steroids alone
— Step 3 sequence: ophthalmology evaluation → infectious workup → treat infection if present → add anti-inflammatory
— Step 1: Topical corticosteroids (prednisolone acetate 1% drops) ± cycloplegic
— Step 2: Periocular or intravitreal steroid injection (triamcinolone, dexamethasone implant) for unilateral refractory or CME
— Step 3: Systemic corticosteroids (oral prednisone 1 mg/kg/day) for bilateral, intermediate/posterior, or severe disease
— Step 4: Steroid-sparing immunomodulator if >7.5 mg prednisone needed beyond 3 months or steroid toxicity emerges
· Antimetabolites: methotrexate, mycophenolate, azathioprine
· T-cell inhibitors: cyclosporine, tacrolimus
— Step 5: Biologics — adalimumab (FDA-approved for non-infectious intermediate/posterior/panuveitis), infliximab; rituximab for refractory
— Anatomy (posterior > intermediate > anterior in vision risk)
— Bilaterality, granulomatous features, CME, vasculitis, optic neuropathy
— Underlying systemic disease severity
— Cycloplegic (cyclopentolate, homatropine, atropine) — anterior uveitis: relieves ciliary spasm pain AND prevents posterior synechiae by keeping pupil dilated
— IOP-lowering drops if secondary glaucoma; avoid prostaglandin analogs (may worsen inflammation/CME)
Step 3 management: Never start chronic systemic steroids without first checking glucose, BMD risk, PPD/QuantiFERON, hepatitis B/C serologies, and bone health plan (calcium/vitamin D ± bisphosphonate per ACR guidelines if anticipated >3 months at ≥7.5 mg/day).
— Prednisolone acetate 1% — start 1 drop every 1–2 hours while awake; taper over weeks based on cell count
— Difluprednate 0.05% — more potent, fewer drops needed; higher IOP risk
— Side effects: elevated IOP ("steroid responder"—up to 30% of patients), cataract (posterior subcapsular), corneal infection risk
— Cyclopentolate 1% TID or homatropine 5% BID for moderate inflammation
— Atropine 1% for severe inflammation with hypopyon
— Purpose: comfort + synechiae prevention
— Oral prednisone 1 mg/kg/day (max ~60–80 mg) for intermediate/posterior/panuveitis
— IV methylprednisolone 1 g/day × 3 days for sight-threatening disease (e.g., VKH, severe Behçet)
— Taper over 2–3 months; never abrupt withdrawal
— Add PJP prophylaxis (TMP-SMX) if ≥20 mg prednisone for >1 month, calcium/vitamin D, GI protection if NSAID coadministered
— Methotrexate 15–25 mg weekly + folate — first-line oral; monitor LFTs, CBC q4–8 weeks
— Mycophenolate mofetil 1–1.5 g BID — well tolerated; teratogenic, monitor CBC/LFTs
— Azathioprine 1–2.5 mg/kg/day — check TPMT activity first; risk of severe leukopenia
— Cyclosporine 2.5–5 mg/kg/day — monitor renal function and BP
— Adalimumab 40 mg SC q2 weeks — FDA-approved for non-infectious intermediate/posterior/panuveitis; screen TB and hepatitis B before initiation
— Infliximab — preferred for Behçet uveitis
— Toxoplasmosis: pyrimethamine + sulfadiazine + folinic acid (or TMP-SMX)
— HSV/VZV uveitis: oral acyclovir or valacyclovir + topical steroid (under cover)
— Syphilis: IV penicillin G × 14 days (neurosyphilis regimen) — ocular syphilis = neurosyphilis
— CMV retinitis: IV ganciclovir or oral valganciclovir + intravitreal injections
Board pearl: Ocular syphilis is treated as neurosyphilis: IV penicillin G 18–24 million units/day × 10–14 days, regardless of CSF findings. Do an LP and HIV test in every confirmed case.
— Sub-Tenon triamcinolone (40 mg) — posterior depot, weeks of effect; risk of IOP rise, ptosis, globe perforation
— Intravitreal triamcinolone (2–4 mg) — rapid onset; cataract and IOP risks
— Dexamethasone intravitreal implant (Ozurdex) — biodegradable, ~3–6 months effect; FDA-approved for non-infectious posterior uveitis
— Fluocinolone acetonide implant (Retisert, Yutiq) — sustained release up to 3 years; nearly universal cataract and ~30% glaucoma rates
— Clears vitreous debris, allows sampling for PCR/cytology
— Indications: vitreous hemorrhage, suspected lymphoma, refractory CME, retinal detachment, endophthalmitis
— Cataract surgery — defer until ≥3 months of quiescence; perioperative steroid burst essential
— Glaucoma surgery — trabeculectomy with mitomycin C or glaucoma drainage device for uncontrolled steroid- or synechiae-related glaucoma
— Synechiolysis — mechanical separation of synechiae if pupillary block develops; consider laser peripheral iridotomy
— Retinal detachment repair — scleral buckle or vitrectomy
— Postoperative or endogenous; presents as severe pain, hypopyon, vitritis, vision loss
— Emergency: vitreous tap + intravitreal antibiotics (vancomycin + ceftazidime ± amphotericin if fungal); systemic antibiotics if endogenous
— Adjunct for choroidal neovascularization or refractory CME after inflammation controlled
— Peripheral iridotomy for pupillary block
— Photocoagulation for retinal neovascularization secondary to ischemic vasculitis
CCS pearl: For suspected postoperative endophthalmitis within 6 weeks of cataract surgery, order STAT ophthalmology consult, vitreous tap with intravitreal vancomycin + ceftazidime; do NOT wait for cultures. Delay >24 hours dramatically worsens visual prognosis.
— Higher pretest probability of masquerade syndromes: primary vitreoretinal lymphoma, leukemia, metastatic disease, paraneoplastic retinopathy
· Suspect when "uveitis" is steroid-responsive initially then relapses, especially bilateral with CNS symptoms
· Threshold for vitreous biopsy is lower in patients >60
— Drug-induced uveitis more common: bisphosphonates (especially zoledronate), rifabutin (with CYP inducers), checkpoint inhibitors, sulfonamides
— Comorbid diabetes and hypertension complicate steroid use—anticipate hyperglycemia, fluid retention; check HbA1c, blood pressure, weight at each visit
— Osteoporosis prevention mandatory: DEXA scan, calcium 1200 mg/d + vitamin D 800–1000 IU/d, bisphosphonate if prednisone ≥7.5 mg/d expected >3 months
— Avoid or dose-reduce cyclosporine and tacrolimus—nephrotoxic; monitor trough levels and creatinine
— Methotrexate contraindicated if CrCl <30; reduce dose at 30–60
— NSAIDs—often avoided to spare renal function and because they have minimal role in uveitis treatment
— TMP-SMX (PJP prophylaxis) — dose-adjust; watch hyperkalemia, creatinine bump
— Acyclovir/valacyclovir for HSV uveitis—renal dosing critical to avoid crystal nephropathy and neurotoxicity
— Methotrexate, azathioprine, leflunomide—hepatotoxic; check baseline LFTs and hepatitis B/C, monitor q4–8 weeks
— Avoid in active hepatitis or cirrhosis
— Mycophenolate generally safer; still monitor LFTs
— Reactivation risk for hepatitis B with biologics/rituximab—check HBsAg, anti-HBc; treat prophylactically with entecavir or tenofovir if positive
Step 3 management: Before starting chronic immunosuppression in elderly: baseline CBC, CMP, hepatitis B/C, HIV, QuantiFERON, CXR, DEXA, age-appropriate cancer screening (colon, breast, cervical, prostate, lung), and vaccinations (pneumococcal, recombinant zoster, annual influenza, hepatitis B if non-immune)—live vaccines should be given before immunosuppression.
— Uveitis activity often decreases during pregnancy (immune modulation) and may flare postpartum
— Safe: topical corticosteroids and cycloplegics (minimal systemic absorption); short systemic prednisone if needed (preferred over fluorinated steroids—placenta inactivates prednisone)
— Avoid: methotrexate, mycophenolate, cyclophosphamide (all teratogenic—stop ≥3 months pre-conception)
— Acceptable in pregnancy: azathioprine, cyclosporine, tacrolimus; TNF inhibitors generally continued through 2nd trimester, individualized in 3rd
— Toxoplasmosis chorioretinitis: spiramycin in pregnancy; pyrimethamine+sulfadiazine after first trimester with folinic acid
— JIA-associated uveitis—classic stem: young girl with oligoarticular ANA-positive JIA, chronic bilateral asymptomatic anterior uveitis, often discovered on routine screening
· Screen slit-lamp every 3 months in high-risk children for 4 years after JIA diagnosis
· Treat with topical steroids + methotrexate first-line systemic; adalimumab if inadequate response (AAP/ACR recommendation)
· Band keratopathy and posterior synechiae are common silent complications
— TINU syndrome: tubulointerstitial nephritis + uveitis—check urinalysis, β2-microglobulin in adolescent with bilateral anterior uveitis
— Pediatric Behçet, sarcoidosis (Blau syndrome with NOD2 mutation) — granulomatous uveitis + arthritis + rash in child <5
— CMV retinitis if CD4 <50: hemorrhagic necrotizing retinitis; treat with valganciclovir + intravitreal ganciclovir; restart/optimize ART (watch immune recovery uveitis)
— Toxoplasma retinochoroiditis—more aggressive; longer treatment course
— Acute retinal necrosis (HSV/VZV) — peripheral necrotizing retinitis; IV acyclovir then oral valacyclovir + intravitreal foscarnet; prophylactic laser barrier
— Endogenous endophthalmitis — candidemia (line, TPN, IVDU), bacterial bacteremia
Board pearl: ANA-positive oligoarticular JIA in a young girl = highest risk for chronic anterior uveitis. It is asymptomatic—diagnosis depends on scheduled slit-lamp screening, not on symptoms reported by the child.
— Most common cause of vision loss in uveitis (especially intermediate/posterior)
— Diagnose on OCT (petaloid pattern on fluorescein angiography)
— Treat with periocular/intravitreal steroids, systemic anti-inflammatory escalation, acetazolamide; refractory cases: anti-VEGF, dexamethasone implant
— Iris adheres to anterior lens capsule → irregular pupil ("festooned pupil" on dilation)
— Risk: iris bombé and secondary angle-closure glaucoma when 360° synechiae block aqueous flow
— Treatment: aggressive cycloplegia, laser peripheral iridotomy if bombé
— Posterior subcapsular cataract from chronic inflammation + steroid use
— Defer surgery until 3 months of quiescence; perioperative steroid coverage
— Mechanisms: trabeculitis, synechiae, steroid response, neovascularization
— Avoid prostaglandin analogs (may worsen inflammation/CME); use beta-blockers, alpha-agonists, carbonic anhydrase inhibitors first
— Surgical: trabeculectomy with mitomycin C, glaucoma drainage device
— Vasculitis with capillary nonperfusion → neovascularization, vitreous hemorrhage
— Retinal detachment (exudative in VKH, tractional/rhegmatogenous in chronic uveitis)
— Choroidal neovascularization
— Epiretinal membrane
— Steroid: hyperglycemia, osteoporosis, weight gain, mood/psychosis, AVN of hip, infection
— Immunosuppressants: marrow suppression, hepatotoxicity, nephrotoxicity, malignancy risk (especially skin), opportunistic infection
— Biologics: TB/HBV reactivation, demyelination, infusion reactions, malignancy
Key distinction: Sudden painful red eye + dilated mid-position pupil + elevated IOP in a uveitis patient = acute angle-closure from iris bombé (synechiae-induced pupillary block), NOT primary angle-closure glaucoma. Treatment: IOP-lowering drops, then laser peripheral iridotomy.
— Any new red eye with photophobia, decreased vision, or suspicion of uveitis
— Hypopyon, severe pain, or vision worse than baseline by ≥2 lines
— Suspected endophthalmitis (postsurgical or endogenous) — vitreous tap + intravitreal antibiotics within hours
— Acute retinal necrosis — IV antivirals
— Sight-threatening Behçet with retinal vasculitis — IV methylprednisolone + infliximab
— VKH with neurologic symptoms — IV steroid pulse, neurology consult, LP
— Posterior scleritis with vision loss
— Sight-threatening disease requiring IV methylprednisolone (1 g/day × 3 days)
— Initiation of cytotoxic therapy with monitoring needs (cyclophosphamide)
— Endogenous endophthalmitis with sepsis evaluation
— Severe systemic vasculitis presentation
— Rheumatology — non-infectious uveitis with systemic features; manages immunomodulators
— Infectious disease — syphilis, TB, opportunistic infection, complex serologic workup
— Neurology — intermediate uveitis with MS workup, VKH, neurosarcoidosis
— Pulmonology — sarcoidosis with parenchymal disease
— Gastroenterology — IBD-associated uveitis
— Oncology/hematology — suspected lymphoma masquerade
— Refractory disease after first-line systemic therapy
— Need for intravitreal implants, complex surgery
— Pediatric uveitis (ideally co-managed with pediatric rheum)
— Worsening pain or redness
— Sudden flashes, floaters, curtain over vision (retinal detachment)
— Increasing photophobia despite drops
— Fever, systemic symptoms
CCS pearl: In CCS, the right answer for "painful red eye + hypopyon + recent intraocular surgery" is stat ophthalmology consult and admit—do NOT delay for outpatient referral. Move clock forward only after consult and intravitreal therapy are ordered.
— Herpetic (HSV, VZV) anterior uveitis — unilateral, elevated IOP, sectoral iris atrophy, decreased corneal sensation, history of dendritic keratitis
— Ocular syphilis — any pattern; check RPR + treponemal; treat as neurosyphilis
— Tuberculous uveitis — granulomatous; serpiginous-like choroiditis; QuantiFERON + chest imaging
— Toxoplasma chorioretinitis — focal retinitis adjacent to pigmented scar; treat with TMP-SMX or pyrimethamine/sulfadiazine
— CMV retinitis — CD4 <50; hemorrhagic necrosis ("pizza pie")
— Acute retinal necrosis — VZV/HSV in immunocompetent; peripheral retinal necrosis, occlusive arteritis
— Endophthalmitis — postoperative (Staph epidermidis, Strep) or endogenous (Candida, Staph aureus)
— Bartonella (cat-scratch) — neuroretinitis with macular star
— Lyme — endemic regions, multiple ocular manifestations
— HLA-B27 spondyloarthropathies (AS, reactive, psoriatic, IBD-associated)—acute, unilateral, recurrent, alternating anterior
— Sarcoidosis — bilateral granulomatous, mutton-fat KPs, hilar adenopathy
— Behçet — recurrent hypopyon, oral/genital ulcers, retinal vasculitis
— VKH — bilateral panuveitis with neurologic/cutaneous features
— JIA — pediatric chronic asymptomatic anterior
— Multiple sclerosis — associated intermediate uveitis (pars planitis)
— Sympathetic ophthalmia — bilateral granulomatous panuveitis after penetrating injury or surgery
— Birdshot chorioretinopathy — HLA-A29, cream lesions
— Rifabutin, bisphosphonates (especially IV), cidofovir, sulfonamides, checkpoint inhibitors (ipilimumab, nivolumab), BRAF inhibitors, prostaglandin analogs
Board pearl: A bilateral granulomatous panuveitis weeks to months after penetrating eye injury or intraocular surgery in the contralateral eye = sympathetic ophthalmia. Prevention: enucleate severely damaged blind eye within 2 weeks if no visual prognosis.
— Acute angle-closure glaucoma — severe pain, nausea/vomiting, mid-dilated fixed pupil, high IOP (>40), halos around lights; treat with topical timolol/pilocarpine/apraclonidine + oral/IV acetazolamide → laser peripheral iridotomy
— Bacterial conjunctivitis — purulent discharge, diffuse injection, no pain, no vision change, normal pupil
— Viral conjunctivitis — watery discharge, preauricular adenopathy, follicular reaction
— Allergic conjunctivitis — bilateral itching, papillae, history of atopy
— Bacterial keratitis — corneal infiltrate, contact lens use, severe pain, vision loss—fluorescein-staining ulcer
— HSV epithelial keratitis — dendritic ulcer staining with fluorescein
— Episcleritis — sectoral redness, mild discomfort, blanches with topical phenylephrine
— Scleritis — deep boring pain that wakes patient at night, bluish hue, does NOT blanch with phenylephrine; strong systemic association (RA, GPA, relapsing polychondritis)
— Subconjunctival hemorrhage — painless flat red patch, no vision change
— Dry eye / blepharitis — chronic, gritty, no anterior chamber reaction
— Primary vitreoretinal lymphoma — older patient, bilateral, "uveitis" responsive then refractory to steroids, CNS lesions; vitreous biopsy with IL-10:IL-6 >1, MYD88 mutation
— Leukemia — pseudohypopyon (malignant cells)
— Retinoblastoma in children — leukocoria, pseudohypopyon
— Intraocular foreign body / retained lens fragment — chronic inflammation post-trauma/surgery
— Retinal detachment with pigment cells in anterior chamber
— Pigment dispersion / pseudoexfoliation
— Juvenile xanthogranuloma — spontaneous hyphema in infant
— Melanoma — necrotic uveal melanoma can mimic posterior uveitis
Key distinction: Scleritis vs episcleritis — scleritis causes deep boring pain, does not blanch with phenylephrine, and demands systemic vasculitis workup (ANCA, RF, ANA, CRP). Episcleritis is benign, blanches with phenylephrine, and usually self-limited.
— Identify and treat underlying systemic disease—the strongest predictor of long-term ocular outcome
· Spondyloarthritis → NSAIDs + TNF inhibitor; recurrent uveitis often improves with adalimumab
· IBD → optimize gut disease control (anti-TNF, vedolimab note: vedolizumab does NOT cover uveitis)
· Sarcoidosis → methotrexate ± biologic
· Behçet → infliximab, azathioprine
— Goal: prednisone ≤7.5 mg/day for chronic maintenance; use immunomodulator to spare steroids
— Annual influenza (inactivated)
— Pneumococcal: PCV20 (or PCV15 + PPSV23)
— Hepatitis B if non-immune
— Recombinant zoster (Shingrix) — safe in immunocompromised, recommended ≥19 if on immunosuppression
— COVID-19 boosters
— HPV per age
— Avoid live vaccines while on biologics or ≥20 mg prednisone for >2 weeks
— PJP prophylaxis (TMP-SMX) if prednisone ≥20 mg/day for >1 month combined with another immunosuppressant
— Latent TB: isoniazid 9 months or rifampin 4 months before/with biologic initiation
— Latent HBV: entecavir or tenofovir with rituximab or anti-TNF
— Calcium 1000–1200 mg/d, vitamin D 800–1000 IU/d
— DEXA baseline if ≥2.5 mg prednisone/day expected >3 months
— Bisphosphonate (alendronate) if prednisone ≥7.5 mg/day expected >3 months or T-score ≤–1.0 with risk factors
— Sun protection (steroids → skin cancer risk, methotrexate photosensitivity)
— Annual dermatology screen on long-term immunosuppression
— Smoking cessation (worsens uveitis severity and TNF response)
— Pregnancy planning—stop teratogens preconception
Step 3 management: For a patient starting chronic methotrexate, schedule baseline CBC, CMP, hepatitis B/C, QuantiFERON, CXR, then CBC + LFTs every 4–8 weeks for 3 months, then every 8–12 weeks. Counsel on alcohol limitation, contraception (teratogenic), folic acid 1 mg/day.
— Acute anterior uveitis: weekly until inflammation resolves, then taper visits with drops
— Intermediate/posterior/panuveitis: every 1–4 weeks during active disease, every 2–3 months stable
— Pediatric JIA: routine slit-lamp screening every 3 months in high-risk patients (ANA+, oligoarticular, age <7)
— Prednisone: BP, glucose/HbA1c, weight at each visit; DEXA at baseline and yearly
— Methotrexate: CBC, LFTs, creatinine q4–8 wk × 3 mo then q8–12 wk
— Mycophenolate: CBC, LFTs q1–3 mo
— Azathioprine: TPMT at baseline, then CBC, LFTs q1–3 mo
— Cyclosporine/tacrolimus: BP, creatinine, drug levels q1–3 mo
— Biologics: annual TB screening, hepatitis serology, malignancy surveillance; CBC, CMP q3–6 mo
— Visual acuity, IOP, slit-lamp at each visit
— OCT for CME at intervals (q1–3 mo if recent macular involvement)
— Annual fundus photography for posterior disease
— Visual fields and OCT-RNFL if uveitic glaucoma
— Communicate medication list, monitoring plan, and red-flag symptoms to PCP at every visit
— Coordinate dose changes between rheumatology and ophthalmology to avoid duplicated or missed labs
— Adherence to drops on prescribed taper—stopping early triggers rebound flare
— Side effect awareness: cataract, glaucoma, infection
— Sun and eye protection (UV exposure may flare some posterior uveitides)
— Driving safety with monocular vision impairment—state-specific reporting
— Importance of carrying medication list (steroid alert wallet card for stress-dose needs)
— Pregnancy planning; contraception with teratogens
Board pearl: Premature discontinuation of topical prednisolone or oral prednisone is the #1 modifiable cause of uveitis recurrence. Always document a slow taper schedule and confirm patient understanding at every transition.
— Disclose risks: serious infection, malignancy (skin, lymphoma with thiopurines + anti-TNF), reactivation of latent TB/HBV, infusion reactions, teratogenicity
— Adolescents: assent + parental consent; discuss fertility implications of cyclophosphamide
— Syphilis, TB, HIV — reportable to local public health authority; partner notification for syphilis
— Document reporting in chart
— Determine state-specific reporting laws for visual impairment (many states require physician notification of DMV when corrected acuity falls below threshold or visual field is constricted)
— Counsel about driving limitations during periods of dilated pupils (mydriatic effect lasts up to 24 hours with atropine)
— Patient discharged from ED with "red eye" without ophthalmology follow-up may suffer irreversible vision loss—always confirm scheduled same-day or next-day ophtho appointment before discharge and document
— When transferring from inpatient to outpatient: ensure prednisone taper, prophylaxis, lab schedule, and rheum/ophthalmology coordination are communicated; written discharge instructions reduce litigation risk
— Verify that patient understands difference between prednisolone acetate (steroid drop) and prednisone (oral)—name confusion can cause severe inflammation or systemic effects if reversed
— High-alert medications: methotrexate is weekly, not daily—daily dosing has caused fatal pancytopenia; use Tall Man lettering and explicit "once weekly" labels
— Pediatric JIA screening adherence; involve school nurse and pediatrician
— Homeless or uninsured patients on immunosuppression: connect to medication assistance programs to ensure continuity
— Patient refusing systemic therapy for sight-threatening disease: assess capacity, document discussion, offer alternatives, respect autonomy after informed refusal
Step 3 management: A once-weekly methotrexate prescription must be written with explicit instructions ("take 4 tablets of 2.5 mg by mouth ONCE WEEKLY on Mondays"). Daily dosing errors are a board-tested patient-safety event and a top National Patient Safety Goal target.
Board pearl: "Recurrent hypopyon uveitis in a young man of Turkish/Mediterranean/Middle Eastern descent with oral ulcers" = Behçet disease. First-line systemic therapy for posterior involvement is infliximab or high-dose steroids + azathioprine.
— Diagnosis: HLA-B27 acute anterior uveitis with ankylosing spondylitis
— Workup: HLA-B27, sacroiliac imaging, RPR, HIV
— Treatment: prednisolone acetate drops + cyclopentolate; refer rheumatology
— Diagnosis: JIA-associated chronic anterior uveitis
— Treatment: topical steroid + methotrexate; adalimumab if refractory
— Diagnosis: VKH
— Treatment: high-dose IV methylprednisolone × 3 days then oral taper + steroid-sparing agent
— Diagnosis: Sarcoid uveitis
— Treatment: oral prednisone + methotrexate
— Diagnosis: Behçet
— Treatment: infliximab or azathioprine + prednisone
— Diagnosis: CMV retinitis
— Treatment: valganciclovir + intravitreal ganciclovir, optimize ART
— Diagnosis: Toxoplasmosis chorioretinitis
— Treatment: spiramycin in 1st trimester; pyrimethamine/sulfadiazine/folinic acid after
— Diagnosis: Postoperative endophthalmitis
— Treatment: STAT vitreous tap + intravitreal vancomycin + ceftazidime
— Diagnosis: Primary vitreoretinal lymphoma
— Diagnostic: vitrectomy with flow cytometry, IL-10:IL-6, MYD88
— Diagnosis: Rifabutin-induced uveitis
— Treatment: reduce rifabutin dose or stop; topical steroid
Key distinction: Ankylosing spondylitis uveitis is unilateral, acute, alternating; sarcoid/VKH/JIA are bilateral. This anatomy pattern alone often points to the systemic diagnosis on the boards.
Uveitis is intraocular inflammation that demands same-day ophthalmology evaluation, infection exclusion (especially syphilis, TB, HIV, HSV) before corticosteroids, and a stepwise treatment ladder from topical drops to systemic steroids to steroid-sparing immunomodulators (methotrexate, mycophenolate) to FDA-approved adalimumab, with workup tailored to anatomic class, bilaterality, and granulomatous features to identify underlying spondyloarthropathy, sarcoidosis, Behçet, VKH, JIA, or infectious cause.
Board pearl: When in doubt on Step 3, "send RPR, HIV, QuantiFERON, and refer to ophthalmology same day"—those four moves catch the highest-yield missable causes and prevent the most preventable vision loss.