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ophtho.uveitis.core.v1PRODUCTION
ophtho.uveitis.core.v1

Uveitis (anterior / intermediate / posterior / panuveitis)

general_internal_medicineacutesubacutechronicadultpediatric
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Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Frame as SUN-classified intraocular inflammation by anatomic site; state that vision-threatening look-alikes (angle closure, keratitis, scleritis, endophthalmitis, intraocular-lymphoma masquerade) and the systemic-disease engines are recognised and routed OUT by engine_id, not re-authored here. Establish the over-arching guardrail: treatable infectious causes are excluded before / alongside immunosuppression (AAO IMT guidance 2017/2018; Sharma Int J STD AIDS 2024).

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uveitis scope + SUN frame confirmed; not-this-engine concerns route-mapped by engine_id

Patient inputs (17)

Mutton-fat (granulomatous) keratic precipitates / iris nodules raise sarcoid/TB/VKH/sympathetic prior; fine non-granulomatous KPs favour HLA-B27/HSV — strongest single morphological pivot (SUN 2021; AAO uveitis guidance)

Mobile hypopyon → HLA-B27 acute anterior uveitis or Behçet; immobile/with retinitis → endophthalmitis route-out — drives infectious-vs-immune branch (SUN 2021 Behçet criteria)

Pain + photophobia + ciliary injection localises anterior; painless floaters localise intermediate/posterior — also the pivot vs conjunctivitis (no pain/photophobia/vision loss) (SUN Jabs 2005)

Back pain/enthesitis (SpA), cough/erythema nodosum/lymphadenopathy (sarcoid), oral/genital ulcers (Behçet), GI symptoms (IBD) targets the systemic-association screen (SUN 2021; AAO uveitis guidance)

TB/endemic exposure, syphilis/STI risk, prior HSV/VZV, toxoplasma/CMV risk — the treatable-infectious screen that MUST precede or accompany immunosuppression (Sharma Int J STD AIDS 2024; AAO IMT guidance 2017/2018)

Primary site of inflammation (anterior vs intermediate vs posterior vs pan) is the SUN-defined organising axis that selects the cause list, the work-up, and the treatment route (Jabs Am J Ophthalmol 2005; SUN 2021)

Unilateral-acute-recurrent → HLA-B27/HSV pattern; bilateral-chronic-insidious → sarcoid/TB/VKH/JIA pattern — laterality + chronicity reframe the pretest priors (SUN 2021 Classification Criteria)

Treponemal + non-treponemal serology in essentially all non-trivial/posterior/recurrent uveitis — syphilis is the great masquerader and is curable with pathogen-directed therapy (Sharma Int J STD AIDS 2024)

IGRA/TST for TB uveitis and as the mandatory pre-biologic / pre-immunosuppression latent-TB screen (AAO IMT guidance — anti-TNF needs TB screen; VISUAL trials)

Profound/sudden vision loss, fixed mid-dilated pupil + cloudy cornea (angle closure), corneal infiltrate (keratitis), boring pain + scleral tenderness (scleritis), post-procedure/endogenous endophthalmitis → route OUT by engine_id (SUN 2021; AAO)

High IOP → uveitic glaucoma / angle-closure differentiation; low IOP → ciliary shutdown/hypotony — both are management-altering and route-relevant (MUST IOP/glaucoma Friedman Ophthalmology 2013)

Serum ACE + chest X-ray/CT for sarcoid-associated granulomatous uveitis (bilateral granulomatous pattern raises this prior) (SUN 2021 sarcoid criteria)

HLA-B27 in unilateral acute non-granulomatous anterior uveitis — strong association with the spondyloarthropathy spectrum and recurrence risk; routes to rheum.spondyloarthropathy.core.v1 (SUN 2021 HLA-B27 AAU criteria)

OCT for cystoid macular oedema — the leading cause of uveitic visual loss and the monitored treatment target (POINT Thorne Ophthalmology 2018)

JIA-associated anterior uveitis is typically ASYMPTOMATIC and chronic — needs scheduled screening slit-lamp regardless of symptoms (Grassi J Rheumatol 2007; Ramanan NEJM 2017 SYCAMORE)

Methotrexate and mycophenolate are teratogenic and contraindicated in pregnancy — gates the steroid-sparing arm (FAST Rathinam JAMA 2019)

Baseline CBC/LFT/renal before and during methotrexate/mycophenolate (elevated LFTs the most common AE in FAST) and for biologic safety (FAST Rathinam JAMA 2019)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (7)

7 need judgement
  • informationallife_threateningsight_threatening_posterior_or_panuveitis
    Posterior uveitis / panuveitis with macular, optic-nerve, or vasculitic involvement, or profound/sudden vision loss (SUN 2021 Classification Criteria)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereinfectious_cause_must_exclude_before_steroid
    Positive syphilis serology / positive IGRA-TST / herpetic or toxoplasma features, or posterior/granulomatous/immunocompromised pattern before planned immunosuppression (Sharma Int J STD AIDS 2024; AAO IMT guidance 2017/2018)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverehypopyon_behcet_pattern
    Mobile hypopyon ± retinal vasculitis with oral/genital ulceration — Behçet uveitis pattern (SUN 2021 Behçet criteria)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereuveitic_glaucoma_or_steroid_iop_rise
    IOP elevation from uveitis, angle compromise, or corticosteroid response (implant ~4-fold IOP-elevation, ~3-fold glaucoma in MUST) (Friedman Ophthalmology 2013; MUST 2015)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveremasquerade_intraocular_lymphoma
    Elderly patient, chronic vitritis/sub-RPE infiltrates, steroid-refractory or steroid-dependent "uveitis" — primary vitreoretinal lymphoma masquerade (SUN 2021)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatepediatric_jia_asymptomatic_uveitis_screening
    At-risk child (ANA-positive oligoarticular JIA, young age at onset) requiring scheduled screening slit-lamp despite no symptoms (Grassi J Rheumatol 2007 — 20% prevalence, 88.7% within 4 y, often asymptomatic)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatecorticosteroid_dependent_chronic_noninfectious
    Cannot taper below ~7.5 mg prednisone/day without flare in non-infectious uveitis (FAST endpoint definition; Rathinam JAMA 2019)
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

Run this disease's risk and dosing calculators inline.

RISK_STRATIFICATIONoptionalDrives screening
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Recommended regimen

Uveitis — infection-gated tiered anti-inflammatory & steroid-sparing ladder
axis: uveitis_infection_gated_ladderstep 1 - Step 1 — Exclude / treat infectious cause BEFORE or WITH immunosuppression
Selected step "Step 1 — Exclude / treat infectious cause BEFORE or WITH immunosuppression" — Any uveitis before corticosteroid/immunomodulation; mandatory in posterior/pan, recurrent, granulomatous, or immunocompromised disease
  • infectious_screen_syphilis_tb_then_pathogen_directed_gate
    first line
    decision_gate
    triggers: posterior_or_panuveitis, recurrent_or_granulomatous, immunocompromised, before_immunosuppression
    AAO IMT guidance 2017/2018 + Sharma Int J STD AIDS 2024 — syphilis is the great masquerader; treatable infection must be excluded/treated before or alongside immunosuppression; anti-TNF requires TB screen. Route id.syphilis.core.v1 / id.tuberculosis.core.v1 by engine_id.
  • valaciclovir
    first line
    antiviral_nucleoside_analogue
    1 g • PO • TID
    triggers: hsv_or_vzv_anterior_uveitis, acute_retinal_necrosis_oral_step_down
    Pathogen-directed therapy for HSV/VZV anterior uveitis and ARN; corticosteroid only added under antiviral cover (SUN 2021 herpetic criteria; AAO uveitis guidance)
    rxcui 73645
  • aciclovir
    first line
    antiviral_nucleoside_analogue
    10 mg/kg • IV • q8h
    triggers: acute_retinal_necrosis_induction, severe_herpetic_posterior
    IV aciclovir induction for acute retinal necrosis before oral step-down (SUN 2021; AAO) — renal-dose by eGFR
    rxcui 281

outpatient playbook — drug actions (4)

  1. 1. prednisolone acetate 1% ophthalmic (non-infectious anterior)
    rxcui 8638
    1 drop • topical • q1-2h then taper
    trigger: Confirmed non-infectious anterior uveitis, infection excluded (AAO uveitis guidance)
    Topical corticosteroid to suppress AC inflammation on SUN grading
  2. 2. cyclopentolate 1% ophthalmic
    rxcui 3001
    1 drop • topical • BID-TID
    trigger: Ciliary spasm / posterior-synechiae prevention (AAO uveitis guidance)
    Cycloplegia for pain relief and synechiae prevention
  3. 3. valaciclovir (HSV/VZV anterior uveitis)
    rxcui 73645
    1 g • PO • TID
    trigger: Herpetic anterior uveitis (sectoral iris atrophy + raised IOP) (SUN 2021)
    Pathogen-directed antiviral; steroid only under cover
  4. 4. methotrexate (chronic steroid-dependent non-infectious)
    rxcui 6851
    15-25 mg • PO • weekly
    trigger: Corticosteroid-dependent non-infectious uveitis, TB/syphilis screened, not pregnant (FAST JAMA 2019)
    Steroid-sparing antimetabolite — favoured for posterior/pan in FAST

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Painful, photophobic red eye with blurred vision, circumcorneal (ciliary) injection ± constricted/irregular pupil — acute anterior uveitis/iritis, the commonest presentation (SUN Jabs Am J Ophthalmol 2005); Floaters and painless visual blurring with little/no external injection — intermediate (vitritis/snowballs) or posterior uveitis (chorioretinitis) until proven otherwise (SUN 2021 Classification Criteria); Hypopyon or recurrent acute non-granulomatous anterior uveitis — HLA-B27 spondyloarthropathy spectrum / Behçet pattern (SUN 2021; AAO uveitis guidance).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Uveitis (anterior / intermediate / posterior / panuveitis)** (ophtho.uveitis.core.v1).
Phenotype framing: Terminal differential with named pivots: HLA-B27 acute anterior uveitis (unilateral, recurrent, fibrinous, hypopyon, +HLA-B27 pivot) vs HSV/VZV anterior (sectoral iris atrophy + raised IOP + corneal scar pivot) vs sarcoid (bilateral granulomatous mutton-fat KP + hilar adenopathy pivot) vs Behçet (hypopyon + retinal vasculitis + oral/genital ulcers pivot) vs JIA (asymptomatic chronic paediatric anterior, band keratopathy pivot) vs toxoplasma (focal retinochoroiditis adjacent to old scar pivot) vs syphilis/TB (any pattern — serology/IGRA pivot) vs intraocular lymphoma (elderly, vitreous cells, steroid-refractory — vitreous biopsy pivot). Distinguish from conjunctivitis / angle closure / keratitis / scleritis / endophthalmitis (SUN 2021 Classification Criteria).
Scope: Frame as SUN-classified intraocular inflammation by anatomic site; state that vision-threatening look-alikes (angle closure, keratitis, scleritis, endophthalmitis, intraocular-lymphoma masquerade) and the systemic-disease engines are recognised and routed OUT by engine_id, not re-authored here. Establish the over-arching guardrail: treatable infectious causes are excluded before / alongside immunosuppression (AAO IMT guidance 2017/2018; Sharma Int J STD AIDS 2024).

No severity triggers fired against current inputs.

Plan

Regimen axis: **Uveitis — infection-gated tiered anti-inflammatory & steroid-sparing ladder** — step "Step 1 — Exclude / treat infectious cause BEFORE or WITH immunosuppression".
1. infectious_screen_syphilis_tb_then_pathogen_directed_gate (decision_gate, first line) — AAO IMT guidance 2017/2018 + Sharma Int J STD AIDS 2024 — syphilis is the great masquerader; treatable infection must be excluded/treated before or alongside immunosuppression; anti-TNF requires TB screen. Route id.syphilis.core.v1 / id.tuberculosis.core.v1 by engine_id.
2. valaciclovir 1 g PO TID (antiviral_nucleoside_analogue, first line) — Pathogen-directed therapy for HSV/VZV anterior uveitis and ARN; corticosteroid only added under antiviral cover (SUN 2021 herpetic criteria; AAO uveitis guidance)
3. aciclovir 10 mg/kg IV q8h (antiviral_nucleoside_analogue, first line) — IV aciclovir induction for acute retinal necrosis before oral step-down (SUN 2021; AAO) — renal-dose by eGFR

Setting playbook (outpatient) — Classify by SUN site/laterality/chronicity, exclude treatable infection (syphilis/TB), start site-appropriate anti-inflammatory therapy (topical prednisolone + cycloplegic for anterior), and book ophthalmology follow-up with SUN-grading + IOP review; set up steroid-sparing pathway if chronic non-infectious (Jabs Am J Ophthalmol 2005; FAST JAMA 2019; AAO IMT guidance)
4. prednisolone acetate 1% ophthalmic (non-infectious anterior) 1 drop topical q1-2h then taper — Confirmed non-infectious anterior uveitis, infection excluded (AAO uveitis guidance) (Topical corticosteroid to suppress AC inflammation on SUN grading)
5. cyclopentolate 1% ophthalmic 1 drop topical BID-TID — Ciliary spasm / posterior-synechiae prevention (AAO uveitis guidance) (Cycloplegia for pain relief and synechiae prevention)
6. valaciclovir (HSV/VZV anterior uveitis) 1 g PO TID — Herpetic anterior uveitis (sectoral iris atrophy + raised IOP) (SUN 2021) (Pathogen-directed antiviral; steroid only under cover)
7. methotrexate (chronic steroid-dependent non-infectious) 15-25 mg PO weekly — Corticosteroid-dependent non-infectious uveitis, TB/syphilis screened, not pregnant (FAST JAMA 2019) (Steroid-sparing antimetabolite — favoured for posterior/pan in FAST)

Non-pharmacologic actions:
- Treponemal + non-treponemal syphilis serology and IGRA/TST before any immunosuppression (Sharma 2024; AAO IMT guidance)
- SUN grading + IOP + OCT macula documented; photograph if available (Jabs 2005; POINT 2018)
- Counsel gradual corticosteroid taper to avoid rebound (AAO uveitis guidance)
- Refer/route systemic association by engine_id (rheum.spondyloarthropathy / sarcoid / id.syphilis / id.tuberculosis)

AVOID / contraindication checks:
- Exclude or treat infection before immunosuppression (AAO IMT guidance 2017/2018; Sharma Int J STD AIDS 2024 — syphilis/TB/herpetic/toxoplasma must be screened; corticosteroid alone can worsen infectious uveitis)
- Methotrexate and mycophenolate contraindicated pregnancy (FAST Rathinam JAMA 2019 — teratogenic; defer/stop and use corticosteroid bridging in pregnancy)
- Tb hbv screen mandatory before anti tnf adalimumab (VISUAL Lancet 2016; AAO IMT guidance — anti TNF increases TB/invasive infection risk)
- Live vaccines contraindicated on immunosuppression and biologic (AAO IMT guidance)
- Monitor iop on any corticosteroid steroid responder and uveitic glaucoma (MUST Friedman Ophthalmology 2013 — implant ~4 fold IOP elevation, ~3 fold glaucoma)
- Renal dose valaciclovir aciclovir methotrexate cotrimoxazole (CKD EPI 2021 eGFR guided)

Monitoring

Regimen monitoring:
- SUN anterior chamber cell flare and vitreous haze grading each visit to inactive (Jabs Am J Ophthalmol 2005)
- IOP every visit steroid response and uveitic glaucoma (MUST Friedman 2013)
- OCT macula for cystoid macular oedema response (POINT Thorne Ophthalmology 2018)
- CBC LFT renal on methotrexate or mycophenolate (FAST Rathinam JAMA 2019 — LFT elevation commonest AE)
- biologic infection surveillance and periodic TB reassessment (AAO IMT guidance)
- pediatric JIA scheduled screening slit lamp by ANA age subtype (Grassi J Rheumatol 2007)

Setting (outpatient) monitoring:
- SUN AC cell/flare + vitreous haze re-grading at 1 week then per response (Jabs 2005)
- IOP every visit for steroid response and uveitic glaucoma (MUST Friedman 2013)
- OCT for CME resolution; CBC/LFT/renal if on antimetabolite (POINT 2018; FAST JAMA 2019)

Follow-up plan: Chronic-disease arc (the deep tail of this engine): structured corticosteroid-sparing taper to ≤7.5 mg prednisone with sustained inactivity (FAST endpoint); long-term antimetabolite/biologic continuation with periodic CBC/LFT/renal + biologic infection surveillance; recurrence counselling and rapid-access pathway for HLA-B27 recurrent AAU; complication surveillance and management routing — uveitic glaucoma (→ ophtho.acute-angle-closure-glaucoma.core.v1 for secondary glaucoma), cataract, band keratopathy, persistent CME, hypotony; PAEDIATRIC JIA asymptomatic-uveitis screening slit-lamp schedule by ANA/age/subtype with early methotrexate/adalimumab (Grassi J Rheumatol 2007; Wennink Acta Ophthalmol 2022 — earlier IMT improved outcomes; Ramanan NEJM 2017). Co-manage the systemic association via its engine_id.
- Close-out criterion: steroid-sparing maintenance + complication-surveillance + paediatric screening plan documented; systemic association co-managed by engine_id

Monitoring phase: Active-inflammation monitoring on the SUN scale: serial anterior-chamber cell/flare and vitreous-haze grading to "inactive"; IOP at every visit (steroid-response AND uveitic glaucoma — implant carries ~4-fold IOP risk and ~3-fold glaucoma in MUST, Friedman 2013 / MUST 2015); OCT for CME response (POINT). Steroid-sparing safety: CBC/LFT/renal on antimetabolites (FAST — LFT elevation the commonest AE); biologic infection vigilance. Counsel that corticosteroid taper is gradual to avoid rebound.

Disposition

Current setting: outpatient — Classify by SUN site/laterality/chronicity, exclude treatable infection (syphilis/TB), start site-appropriate anti-inflammatory therapy (topical prednisolone + cycloplegic for anterior), and book ophthalmology follow-up with SUN-grading + IOP review; set up steroid-sparing pathway if chronic non-infectious (Jabs Am J Ophthalmol 2005; FAST JAMA 2019; AAO IMT guidance)

Disposition criteria:
- Mild non-granulomatous anterior uveitis → continue topical + cycloplegic, 1-week ophthalmology review (AAO uveitis guidance)
- Chronic non-infectious steroid-dependent → steroid-sparing pathway + uveitis-specialist co-management (FAST JAMA 2019)
- Sight-threatening / infectious posterior / paediatric severe JIA → urgent referral ± admission (SUN 2021; Ramanan NEJM 2017)

Escalation triggers (move to higher acuity):
- Sight-threatening posterior/pan or vision loss → urgent uveitis-specialist referral / route to ophtho.acute-vision-loss workup (SUN 2021)
- Fixed mid-dilated pupil + cloudy cornea + high IOP → route to ophtho.acute-angle-closure-glaucoma.core.v1 (AAO)
- Hypopyon with retinitis / post-procedure → suspected endophthalmitis, same-day route OUT (SUN 2021)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Posterior uveitis / panuveitis with macular, optic-nerve, or vasculitic involvement, or profound/sudden vision loss (SUN 2021 Classification Criteria)
- [SEVERE] Positive syphilis serology / positive IGRA-TST / herpetic or toxoplasma features, or posterior/granulomatous/immunocompromised pattern before planned immunosuppression (Sharma Int J STD AIDS 2024; AAO IMT guidance 2017/2018)
- [SEVERE] Mobile hypopyon ± retinal vasculitis with oral/genital ulceration — Behçet uveitis pattern (SUN 2021 Behçet criteria)

Citations

- SUN (Standardization of Uveitis Nomenclature) Working Group anatomic classification & grading (Jabs et al, Am J Ophthalmol 2005) + SUN Classification Criteria for the Uveitides (Am J Ophthalmol 2021) + AAO / Ocular Immunology guidance on noncorticosteroid systemic immunomodulatory therapy in non-infectious uveitis (2017/2018, currency re-checked 2026-05-17) + FAST uveitis trial (Rathinam, JAMA 2019) + VISUAL I/II adalimumab (Nguyen/Jaffe, Lancet 2016) + MUST trial (Ophthalmology 2015) + POINT trial (Thorne, Ophthalmology 2018) + SYCAMORE (Ramanan, NEJM 2017) [PMID:16196117](https://pubmed.ncbi.nlm.nih.gov/16196117/)
- Cited evidence (PMID 33848532) [PMID:33848532](https://pubmed.ncbi.nlm.nih.gov/33848532/)
- Cited evidence (PMID 34459962) [PMID:34459962](https://pubmed.ncbi.nlm.nih.gov/34459962/)
- Cited evidence (PMID 31503307) [PMID:31503307](https://pubmed.ncbi.nlm.nih.gov/31503307/)
- Cited evidence (PMID 24917273) [PMID:24917273](https://pubmed.ncbi.nlm.nih.gov/24917273/)

Last reconciled with current guidelines: 2026-05-17.
References
  • SUN (Standardization of Uveitis Nomenclature) Working Group anatomic classification & grading (Jabs et al, Am J Ophthalmol 2005) + SUN Classification Criteria for the Uveitides (Am J Ophthalmol 2021) + AAO / Ocular Immunology guidance on noncorticosteroid systemic immunomodulatory therapy in non-infectious uveitis (2017/2018, currency re-checked 2026-05-17) + FAST uveitis trial (Rathinam, JAMA 2019) + VISUAL I/II adalimumab (Nguyen/Jaffe, Lancet 2016) + MUST trial (Ophthalmology 2015) + POINT trial (Thorne, Ophthalmology 2018) + SYCAMORE (Ramanan, NEJM 2017) — PMID:16196117
  • Cited evidence (PMID 33848532) — PMID:33848532
  • Cited evidence (PMID 34459962) — PMID:34459962
  • Cited evidence (PMID 31503307) — PMID:31503307
  • Cited evidence (PMID 24917273) — PMID:24917273