uveitis
anamnesis
Subjective: Start of symptoms in the eyes? pain?
Systemic symptoms: back pain, joint pain? Night sweats? fever? cough? Gastrointestinal problems? skin problems? Stays abroad? Tick bite? Cat scratch, bite? Vaccinations? Previous investigations (laboratory, X-ray, etc.)?
Diseases: rheumatism? infectious diseases (e.g. HIV)?
Medication:?
Allergies:?
Family history:?
Visual aids: glasses/contact lenses?
Ophthalmic examination
- SL (first in miosis) with fundus copy (in mydriasis after initial examination then 1x/year), corneal sensitivity, eye pressure measurement, gonioscopy, vitreous examination
- Determine the center of inflammation
- anterior uveitis
intermedia - posterior
- Panuveitits
- retinal vasculitis
- Uveitis-related complications
- ligament keratopathy
- glaucoma
- posterior synechiae
- Cataracta secundaria
- CME
- CNV
- Phthysis bulbi
Grading according to SUN (uveitis nomenclature group)
cells in the anterior chamber (cells in 1x1 mm slit light at 16x magnification)
- < 1 cells = grade 0
- 1-5 cells = grade 0.5+
- 6-15 = grade 1+
- 16-25 = grade 2+
- 26-50 = grade 3+
- > 50 = grade 4+
Flare in the front chamber
- No flare = grade 0
- Fine = grade 1+
- Moderate = grade 2+
- Distinct (iris/lens appear blurry) = grade 3+
- Intensive = grade 4+ (fibrin)
cells in the vitreous body (intermed/posterior uveitis)
- None = grade 0
- Very few = grade 0.5+
- Mild = grade 1+
- Moderate = grade 2+
- Strong = grade 3+
- Very strong = grade 4+
Formulate the diagnosis
- acute/chronic (>3 months) /redcidifying (inflammation-free intervals) /persistent
- single-sided/two-sided
- non-granulomatous/granulomatous (mutton-fatt KP, Irisnoduli)
- Anterior/intermediate/posterior/panuveitis
- etiology (e.g. HLA-B27-associated, idiopathic)
- Laboratory (date): (e.g. ACE+)
- Chest X-ray: (e.g. sarcoid signs)->
- ... with cystoid macular oedema/secondary glaucoma/cataracta/synechia/retinal vasculitis
Clarification of etiology
No clarifications
- In the case of non-granulomatous anterior uveitis, only after the second relapse otherwise upon initial diagnosis
- for known systemic underlying diseases (e.g. sarcoidosis, tuberculosis)
- Drug side effects (e.g. rifabutin, bisphosphonates, cidofovir, etanercept, sulfonamides, fluoroquinolones, etc.) that match uveitis
Too many unnecessary investigations can lead to false diagnoses due to false positive results.
Basic diagnostics
For all bilateral, granulomatous, or recurrent uveitis
- differential blood count (infection, systemic diseases, hematologic diseases, side effect of immunosuppression?)
- CRP/ESR (Erythrocyte sedimation rate)
- creatinine (kidney involvement, e.g. granulomatosis/SLE, before systemic therapy with steroids, methotrexate, etc.)
- ASAT, ALAT (see creatinine)
- electrolytes (Na, K, Ca) (before systemic therapy)
- Lues serology (syphilis, TPPA+RPR/VDRL)
Advanced diagnostics
Anterior uveitis (adults)
- OCT macula (macular edema?)
- Unilateral findings, increased eye pressure, focal iris atrophy, fine to medium-sized surgeries, recurrent: Only in unclear cases chamber water puncture: HSV, VZV (less commonly CMV, EBV)
- small diffusely distributed, star-shaped endothelial fittings (“moth-eating” -like pigment defects of iris/fox uveitis: usually no further diagnosis
- unilateral, acute fibrinous (or non-granulomatous), painful: HLA-B27 (spondyloarthropathy, M. Bechterew, M. Reiter, M. Crohn, psoriasis, reactive arthritis?)
- back or joint pain (rheumatological assessment)
- Gastrointestinal complaints (gastroenterological evaluation)
- HLA-B27 negative on one side
- hypopyone, aphthae, genital ulcers -> Behcet muscle
- Regression -> no weather clarifications
- Recurrence -> Clarifications for anterior granulomatous uveitis
- erythema migrans/arthritis/neurological late manifestation: Borrelia serology
- Bilateral young patients
- Suspicion of juvenile idiopathic arthritis: ANA
- general symptoms
- urine beta2 microglobulin, creatinine, urine status (TINU=tubulointerstitial nephritis and uveitis syndrome)
- Granulomatous (or non-acute-fibrinous)
- sIL-2r (most sensitive) + ACE (lysozyme only when ACE or sIL-2r are not available) (sarcoidosis?)
- Chest X-ray or CT (tuberculosis or sarcoidosis?)
- Tuberculosis (Quantiferon) Test (IGRA) (Tuberculosis?)
intermedia uveitis
- Typical: Snowballs, Vitritis, Younger Patients
- sIL-2r and ACE (sarcoidosis?)
- OCT (CME?) , fluorescence angiography (vasculitis, ischemia?)
- chest X-ray (sarcoidosis/tuberculosis?)
- Suspected Intraocular lymphoma (from 45 LJ): possibly diagnostic PPV
- Suspected M.S./CNS lymphoma (from 45 years): neurological ablation, cMRI
- erythema migrans/arthritis/neurological late manifestation: Borrelia serology
Posterior uveitis, panuveitis, retinal vasculitis
- sIL-2r and ACE (sarcoidosis?) , tuberculosis test (IGRA)
- OCT (CME?) , fluorescence angiography (vasculitis, ischemia?)
- Vd.A. Chorioiditis: with ICG, autofluorescence image
- chest X-ray (sarcoidosis/tuberculosis?)
- Suspected Retinitis
- VK sample/PPV (PCR on HSV, VZV, CMV, EBV)
- Suspected Papillitis, neuritis: neurological evaluation
- Suspected Vasculitis/focal retinitis with oral/genital canker sores: HLA-B51 (M. Behcet?)
- Suspected System vasculitis/collagenosis (particularly in retinal vasculitis and scleritis): autoantibodies (ANA, ANCA, dsDNA)
- Suspected Neuroretinitis: Bartonella Serology
- Suspected Endophthalmitis (chorioretinal infiltrates in case of suspected septic endophthalmitis: blood cultures, aqueous chamber sample, PPV, possibly chorioretinal biopsy)
- Skin changes: dermatology
- erythema migrans/arthritis/neurological late manifestation: Borrelia serology
- Suspected Birdshot chorioretinopathy: HLA-A29
Uveitis treatment
anterior uveitis
- Prednisolone 1% eye drops (e.g. Pred Forte®) and fading depending on inflammatory activity
- Prednisolone eye ointment (e.g. Ultracortenol®) at night for 1 week
- Scopolamine 0.25% 1-2x/day, cyclopentolate 2-3x/day
intermedia uveitis
- Therapy is required only in the presence of secondary complications that threaten vision (macular edema, papillitis, etc.)
- If necessary, systemic steroids after excluding the causes of infection
Posterior uveitis
- Systemic steroids after excluding infections
- The most common is toxoplasmosis
scleritis (diffuse/nodular/necrotizing)
- RF, anti-CCP (rheumatoid arthritis?)
- ANA (lupus erythematosus?)
- Anca (c-ANCA, p-ANCA) (vasculitides (e.g. GPA, MPA)
- anti-dsDNA, ENA (autoimmune screening)
- erythema migrans/neuroretinitis: Borrelia serology
- hepatitis B/C (before systemic immunosuppression)
- rheumatological assessment
-> Risk of sclera perforation
Peripheral ulcerative keratitis (PUK)
differential diagnoses
Moorens's ulcer: very painful, nasal or temporal without sclera involvement (possibly associated with hepatitis C virus)
marginal keratitis, furrow degeneration, senile peripheral corneal degeneration/furrows -> usually no therapy required
Dellen: painless -> intensified humidification
Staphylococcal hypersensitivity reaction/marginal keratitis -> typical steroids and antibiotics
Basic diagnostics
- RF, anti-CCP (rheumatoid arthritis?)
- ANA (lupus erythematosus?)
- Anca (c-ANCA, p-ANCA) (vasculitides (e.g. GPA, MPA)
- anti-dsDNA, ENA (autoimmune screening)
- hepatitis B/C (before systemic immunosuppression)
- rheumatological assessment
-> Risk of corneal perforation
therapy
- Intensive moistening or, if necessary, antibiotic-containing eye ointment
- doxycycline (tetracycline) 100 mg 2x/day (MMP inhibitor)
- Prednisolone 1mg/kg body weight
- proton pump inhibitor (e.g. Pantoprazole® 40 mg 1x/day)
- rheumatological evaluation and start with steroid-sparing immunomodulators (e.g. methotrexate, etc.)
Sjögren's syndrome
Sjögren-specific antibodies
- Anti-SSA/RO (Anti-Ro)
- Anti-SSB/LA (Anti-La)
- IgG game gel (specific for primary Sjögren's syndrome)
Non-specific antibodies
- ANA, RF, complement factors C3 and C4
- Inflammatory markers (CRP, ESR)
CAVE Emergency referral to a tertiary clinic in case of acute situations or in case of doubt
sources:
- www.eyewiki.org
- Will's Eye Manual 8th edition
