SUN II Classification of Uveitides

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Article initiated by:
Evan Wotipka, MD, Talhah Zubair, Eesha Irfanullah, Karen Armbrust MD PhD
All contributors:
Eesha Irfanullah, Talhah Zubair, Karen Armbrust MD PhD, Evan Wotipka, MD
Assigned editor:
Review:
Assigned status Update Pending
.


Uveitis Overview

The uveitides are a collection of diseases characterized by uveal inflammation.[1] Together, these diseases constitute the fifth leading cause of blindness in the developed world.[2] Classes of uveitis are typically divided anatomically, based on where inflammation occurs (i.e., anterior uveitis, intermediate uveitis, posterior uveitis, and panuveitis).[3]  However, uveitis affecting a given anatomic location may have distinct etiologies, clinical courses, prognoses, and treatment indications. Although there are specified clinical diagnostic criteria for several of the uveitides, prior to 2021 there was no validated systemic approach for classification of individual uveitides. Agreement between experts on specific cases is moderate at best.[4] These incongruities have resulted in a lack of uniformity of reporting in the ophthalmic literature.

SUN II Classification Study Overview

In 2021, the Standardization of Uveitis Nomenclature (SUN) Working Group published a paper in the American Journal of Ophthalmology titled “Development of Classification Criteria for the Uveitides”, describing their work to develop classification criteria for the 25 most common uveitides. The objective of the study was to establish a uniform group of patients for inclusion in research studies and to maximize the probability that all study participants will be widely accepted as having the disease.[5] Notably, classification criteria are intended primarily for research and differ from diagnostic criteria in that classification criteria emphasize statistical specificity, whereas diagnostic criteria emphasize sensitivity. As such, patients in the clinic may be classified clinically as having a specific type of uveitis, even though they do not meet the SUN II criteria for that uveitis type. Additional papers in the same journal issue described the specific classification criteria for each uveitis entity listed in Table 1 below:

Table 1: Uveitic Diseases Addressed by the SUN Developing Classification Criteria for the Uveitides Project. Adapted from Standardization of Uveitis Nomenclature (SUN) Working Group 2021.[5]
Anatomic Class Infectious Systemic Disease Associated Eye-Limited
Anterior Cytomegalovirus anterior uveitis Juvenile idiopathic arthritis–associated anterior uveitis Fuchs uveitis syndrome
Herpes simplex virus anterior uveitis Spondyloarthritis/HLA-B27-associated anterior uveitis
Varicella zoster virus anterior uveitis Tubulointerstitial nephritis with uveitis
Syphilitic anterior uveitis Sarcoidosis-associated anterior uveitis
Intermediate Syphilitic intermediate uveitis Multiple sclerosis–associated intermediate uveitis Pars planitis
Sarcoidosis-associated intermediate uveitis Intermediate uveitis, non–pars planitis type
Posterior Acute retinal necrosis Sarcoidosis-associated posterior uveitits Acute posterior multifocal placoid pigment epitheliopathy
Cytomegalovirus retinitis Birdshot chorioretinitis
Syphilitic posterior uveitis Multiple evanescent white dot syndrome
Toxoplasmic retinitis Multifocal choroiditis with panuveitis
Tuberculous posterior uveitis Punctate inner choroiditis
Serpiginous choroiditis
Panuveitis Syphilitic panuveitis Behçet disease uveitis Sympathetic ophthalmia
Tuberculous panuveitis
Sarcoidosis-associated panuveitis
Vogt-Koyanagi-Harada disease (early-stage and late-stage)

Methods and Results

The study developed the classification criteria in 4 phases: 1) informatics; 2) case collection; 3) case selection; 4) machine learning. The informatics phase standardized language to describe each uveitis type and mapped terms to individual diseases. During the case collection phase, the team input 5,766 cases into a database, averaging 100-250 cases for each uveitis type. In the case selection phase, uveitis experts actively reviewed the cases, using formal consensus to determine if they indicated a specific identifiable disease. Cases with supermajority agreement (>75%) among experts were input into the final database, totaling 4,046 cases. Machine learning techniques (multinomial logistic regression with lasso regularization) were used to establish a set of distinguishing criteria among a subset of cases (the “training set” ~85% of cases) and validate these criteria with a second subset of cases (“the validation set” ~15% of cases).

The overall accuracy estimates for each uveitic class (e.g. anterior, intermediate, etc.) are depicted in Table 2. Tables 3 through 6 contain the classification and exclusion criteria, as well as the misclassification rate (the proportion of cases in the validation set classified incorrectly by the machine learning algorithm when compared to the consensus diagnosis) for the specific anterior, intermediate, posterior, and pan-uveitis entities.

Table 2: Accuracy of Validation Set Classification Criteria for the Uveitides. Adapted from Standardization of Uveitis Nomenclature (SUN) Working Group 2021.[5]
Uveitic class Number of diseases Accuracy (%) 95% Confidence Interval
Anterior uveitides 9 96.7 92.4-98.6
Intermediate uveitides 5 99.3 96.1-99.9
Posterior uveitides 9 98.0 94.3-99.3
Panuveitides 7 94.0 89.0-96.8
Infectious posterior/panuveitides 5 93.3 89.1-96.3

SUN II Classification of Uveitides

Anterior Uveitides

Table 3: Classification criteria of anterior uveitides. Adapted from multiple tables from Standardization of Uveitis Nomenclature (SUN) Working Group 2021. [6][7][8][9][10][11][12][13][14][15]
Name Etiology Classification Criteria Exclusion Criteria Misclassification rate training set (%) Misclassification rate validation set (%)
Cytomegalovirus anterior uveitis[7] Infectious
  • Evidence of anterior uveitis
    1. anterior chamber cells
    2. if anterior vitreous cells are present, anterior chamber inflammation should be present
    3. no evidence of retinitis

AND

  • Evidence of cytomegalovirus infection in the eye
    1. positive PCR for cytomegalovirus on aqueous specimen
  1. Positive serology for syphilis using a treponemal test
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating non-caseating granulomata)
  3. Aqueous specimen PCR positive for herpes simplex virus or varicella zoster virus
 1.3 0.0
Herpes simplex virus anterior uveitis[6]
  • Evidence of anterior uveitis:
    1. anterior chamber cells
    2. if anterior vitreous cells are present, severity is less than anterior chamber inflammation
    3. no evidence of retinitis

AND

  1. Unilateral uveitis (unless there is a positive aqueous PCR* for herpes simplex virus)
  2. Evidence of herpes simplex infection in the eye
    1. aqueous humor PCR positive for herpes simplex virus OR
    2. sectoral iris atrophy in a patient ≤50 years of age OR
    3. herpes simplex keratitis
  1. Concomitant dermatomal/cutaneous varicella zoster virus (unless aqueous specimen PCR positive for herpes simplex virus)
  2. Positive serology for syphilis using a treponemal test
  3. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating non-caseating granulomata)
  4. Aqueous specimen PCR positive for cytomegalovirus or varicella zoster virus
 8.3% 17%
Varicella Zoster Virus Anterior Uveitis[8]
  • Evidence of anterior uveitis
    1. anterior chamber cells
    2. if anterior vitreous cells are present, severity is less than anterior chamber inflammation
    3. no evidence of retinitis

AND

  • Unilateral uveitis (unless there is a positive aqueous PCR* for varicella zoster virus)

AND

  • Evidence of varicella zoster virus infection in the eye:

a.   aqueous humor PCR positive for varicella zoster virus OR

b.  sectoral iris atrophy in a patient ≥ 60 years of age OR

c.   concurrent or recent dermatomal Herpes zoster

  1. Positive serology for syphilis using a treponemal test
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating non-caseating granulomata)
  3. Aqueous specimen PCR positive for cytomegalovirus or herpes simplex virus
 0.9 0
Syphilitic anterior uveitis[9]
  • Uveitis with a compatible uveitic presentation, including:
    1. Anterior uveitis OR
    2. Intermediate uveitis or anterior/intermediate uveitis OR
    3. Posterior uveitis or panuveitis with 1 of the following presentations:
      1. Placoid inflammation of the retinal pigment epithelium or
      2. Multifocal inflammation of the retina/retinal pigment epithelium or
      3. Necrotizing retinitis or
      4. Retinal vasculitis

AND

  • Evidence of infection with Treponema pallidum, either
    1. Positive treponemal test and nontreponemal test OR
    2. Positive treponemal test with 2 different treponemal tests
  1. History of adequate treatment for syphilitic uveitis[16]
 0 0
Juvenile idiopathic arthritis-associated chronic anterior uveitis[10] Systemic disease associated
  • Evidence of anterior uveitis:
    1. anterior chamber cells
    2. if anterior vitreous cells are present, severity is less than anterior chamber inflammation.

AND

  • Chronic anterior uveitis or, if at initial diagnosis, uveitis is of insidious onset and asymptomatic /minimally symptomatic

AND

  • Juvenile idiopathic arthritis of the following subtypes
    1. Oligoarthritis, persistent or extended, OR
    2. Rheumatoid-factor negative polyarthritis, OR
    3. Juvenile psoriatic arthritis, other than psoriatic spondylitis
  1. Diseases Enthesitis-related arthritis[11]
  2. Positive serologic test for syphilis using a treponemal test
  3. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating noncaseating granulomata) or other granulomatous disease (eg, familial juvenile systemic granulomatosis)
  4. Aqueous specimen PCR positive for cytomegalovirus, herpes simplex virus, or varicella zoster virus
 2.4 0
Spondyloarthritis/HLAB27-associated anterior uveitis[12]
  • Evidence of anterior uveitis
    1. Anterior chamber cells
    2. If anterior vitreous cells are present, severity is less than anterior chamber inflammation

AND either both #2 and #3 OR #4

  1. Characteristic uveitis course
    • Acute or recurrent acute, unilateral or unilateral alternating course OR
    • Chronic course with a history of a recurrent acute, unilateral or unilateral alternating course evolving into chronic course.
  2. ASAS-defined spondyloarthritis (axial or peripheral) and/or HLA-B27-positive

OR

3. Chronic uveitis with both ASAS-defined spondyloarthritis (axial or peripheral) AND HLA-B27-positive

  1. Positive serology for syphilis using a treponemal test
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating non-caseating granulomata)
  3. Aqueous specimen PCR positive for cytomegalovirus, herpes simplex virus or varicella zoster virus
 0 3.6
Tubulointerstitial nephritis with uveitis[13]
  • Evidence of anterior uveitis
    1. Anterior chamber cells
    2. If vitritis or choroiditis or retinal vascular changes are present, anterior chamber inflammation also should be present

AND

  • Evidence of tubulointerstitial nephritis, either
    1. Positive renal biopsy OR
    2. Elevated urine β-microglobulin and either abnormal urine analysis or elevated serum creatinine
  1. Positive serology for syphilis using a treponemal test
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating noncaseating granulomata)
 1.2 0
Sarcoidosis-associated anterior uveitis[14]
  • Compatible uveitic picture, either
    1. Anterior uveitis OR
    2. Intermediate or anterior/intermediate uveitis OR
    3. Posterior uveitis with either choroiditis (paucifocal choroidal nodule[s] or multifocal choroiditis) OR
    4. Panuveitis with choroiditis or retinal vascular sheathing or retinal vascular occlusion

AND

  • Evidence of sarcoidosis, either
    1. Tissue biopsy demonstrating non-caseating granulomata or
    2. Bilateral hilar adenopathy on chest imaging
  1. Positive serology for syphilis using a treponemal test
  2. Evidence of infection with Mycobacterium tuberculosis, either
    1. Histologically- or microbiologically-confirmed infection with M. tuberculosis OR
    2. Positive interferon-Ɣ release assay (IGRA) OR
    3. Positive tuberculin skin test
 3.2 0
Fuchs uveitis syndrome[15] Eye-limited
  • Evidence of anterior uveitis
    1. anterior chamber cells
    2. if vitreous cells are present, anterior chamber inflammation also should be present
    3. no evidence of active retinitis

AND

  • Unilateral uveitis

AND

  • Evidence of Fuchs uveitis syndrome
    1. heterochromia OR
    2. unilateral diffuse iris atrophy AND stellate keratic precipitates

AND

  • Neither endotheliitis nor nodular, coin-shaped endothelial lesions
  1. Positive serology for syphilis using a treponemal test
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating non-caseating granulomata)
  3. Aqueous specimen PCR  positive for cytomegalovirus, herpes simplex virus or varicella zoster virus
 4.7 5.5

Intermediate Uveitides

Table 4: Classification criteria of intermediate uveitides. Adapted from multiple tables from Standardization of Uveitis Nomenclature (SUN) Working Group 2021.[9][17][14][18][19]
Name Etiology Classification Criteria Exclusion Criteria Misclassification rate training set (%) Misclassification rate validation set (%)
Syphilitic intermediate uveitis[9] Infectious
  • Uveitis with a compatible uveitic presentation, including
    1. Anterior uveitis OR
    2. Intermediate uveitis or anterior/intermediate uveitis OR
    3. Posterior uveitis or panuveitis with 1 of the following presentations:
      1. Placoid inflammation of the retinal pigment epithelium or
      2. Multifocal inflammation of the retina/retinal pigment epithelium or
      3. Necrotizing retinitis or
      4. Retinal vasculitis

AND

  • Evidence of infection with Treponema pallidum, either
    1. Positive treponemal test and nontreponemal test OR
    2. Positive treponemal test with 2 different treponemal tests
  1. History of adequate treatment for syphilitic uveitis[16]
 6.0 0
Multiple sclerosis–associated intermediate uveitis[17] Systemic disease associated
  • Evidence of intermediate uveitis
    1. Vitreous cells or vitreous haze or both;
    2. If anterior chamber cells are present, anterior chamber inflammation is less than that of vitreous;
    3. No evidence of retinitis or choroiditis;

AND

  • Evidence of multiple sclerosis using the revised McDonald diagnostic criteria[20]
  1. Serology positive for syphilis, using a treponemal test;
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating noncaseating granulomata);
  3. Serology positive for Lyme disease; either IgG or IgM (eg, positive ELISA and Western blot results, with the requisite number of bands for assay used.
 0 0
Sarcoidosis-associated intermediate uveitis[14]
  • Compatible uveitic picture, either
    1. Anterior uveitis OR
    2. Intermediate or anterior/intermediate uveitis OR
    3. Posterior uveitis with either choroiditis (paucifocal choroidal nodule[s] or multifocal choroiditis) OR
    4. Panuveitis with choroiditis or retinal vascular sheathing or retinal vascular occlusion

AND

  • Evidence of sarcoidosis, either
    1. Tissue biopsy demonstrating non-caseating granulomata OR
    2. Bilateral hilar adenopathy on chest imaging
  1. Positive serology for syphilis using a treponemal test
  2. Evidence of infection with Mycobacterium tuberculosis,either
    1. Histologically- or microbiologically-confirmed infection with M. tuberculosis OR
    2. Positive interferon-Ɣ release assay (IGRA) OR
    3. Positive tuberculin skin test
 2.6 0
Pars planitis[18] Eye-limited
  • Evidence of intermediate uveitis
    1. Vitreous cells AND/OR vitreous haze
    2. If anterior chamber cells are present, anterior chamber inflammation severity less than vitreous severity
    3. No evidence of retinitis or choroiditis
    4. No retinal vascular occlusion in posterior pole & mid-periphery

AND

  • Evidence of pars planitis
    1. Vitreous snowballs OR
    2. Pars plana snowbanks
  1. Multiple sclerosis, defined by the McDonald criteria18
  2. Positive serology test result for syphilis using a treponemal test
  3. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating non-caseating granulomata)
  4. Positive serology for Lyme disease, either IgG or IgM (e.g. positive ELISA AND Western blot with requisite number of bands for assay used)
 0 1.7
Intermediate uveitis, non–pars planitis type[19]
  • Evidence of intermediate uveitis
    1. vitreous cells AND/OR vitreous haze
    2. if anterior chamber cells are present, anterior chamber inflammation less than vitreous humor
    3. no evidence of retinitis

AND

  • No evidence of pars planitis
    1. neither vitreous snowballs NOR
    2. pars plana snowbanks
  1. Multiple sclerosis, defined by the McDonald criteria[20]
  2. Positive serology for syphilis using a treponemal test
  3. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating noncaseating granulomata)
  4. Positive serology for Lyme disease, either IgG or IgM (eg, positive ELISA AND Western blot with requisite number of bands for assay used)
  5. Evidence of intraocular lymphoma on diagnostic vitrectomy
 0 0

Posterior Uveitides

Table 5: Classification criteria of posterior uveitides. Adapted from multiple tables from Standardization of Uveitis Nomenclature (SUN) Working Group 2021.[21][22][23][24][25][26][27][28][29][30]
Name Etiology Classification Criteria Exclusion Criteria Misclassification rate training set (%) Misclassification rate validation set (%)
Acute retinal necrosis[21] Infectious
  • Necrotizing retinitis involving the peripheral retina


AND (either #2 OR #3)


2. Evidence of infection with either HSV or VZV Positive PCR for either HSV or VZV from either an aqueous or vitreous specimen

3. Characteristic clinical picture:

  • Circumferential or confluent retinitis AND
  • Retinal vascular sheathing and/or occlusion AND
  • More than minimal vitritis
  1. Positive serology for syphilis using a treponemal test
  2. Intraocular specimen PCR-positive for cytomegalovirus or Toxoplasma gondii (unless there is immunocompromise, morphologic evidence for >1 infection, the characteristic clinical picture of acute retinal necrosis, and the intraocular fluid specimen has a positive PCR for either HSV or VZV)
 15 11.5
Cytomegalovirus retinitis[22]
  • Necrotizing retinitis with indistinct borders due to numerous small (<50 µm) satellites

AND

  • Immune compromise, either
    1. Systemic (eg, AIDS, organ transplant, chemotherapy) OR
    2. Ocular (eg, intraocular corticosteroids or chemotherapy)

AND (#A or #B)

A. Characteristic clinical picture ([1 or 2 or 3] and 4)

  1. Wedge-shaped area of retinitis OR
  2. Hemorrhagic appearance of the retinitis OR
  3. Granular appearance of the retinitis AND
  4. Absent to mild vitritis

OR

B. Evidence of intraocular infection with cytomegalovirus

  1. Positive PCR for cytomegalovirus from either the aqueous or vitreous specimen
  1. Positive serology for syphilis using a treponemal test
  2. Intraocular specimen PCR-positive for infection by herpes simplex virus, varicella zoster virus, or Toxoplasma gondii (unless there is immune compromise, morphologic evidence for >1 infection, the characteristic picture of cytomegalovirus retinitis, and the intraocular fluid specimen also has a positive PCR result for cytomegalovirus)
 6.9 6.3
Syphilitic posterior uveitis[9]
  • Uveitis with a compatible uveitic presentation, including
    1. Anterior uveitis OR
    2. Intermediate uveitis or anterior/intermediate uveitis OR
    3. Posterior uveitis or panuveitis with 1 of the following presentations:
      1. Placoid inflammation of the retinal pigment epithelium or
      2. Multifocal inflammation of the retina/retinal pigmentepithelium or
      3. Necrotizing retinitis or
      4. Retinal vasculitis

AND

  • Evidence of infection with Treponema pallidum, either
    1. Positive treponemal test and nontreponemal test
    2. Positive treponemal test with 2 different treponemal tests
  1. History of adequate treatment for syphilitic uveitis[16]
 0 0
Toxoplasmic retinitis[23]
  • Focal or paucifocal necrotizing retinitis


AND (#A or #B)


A. Evidence of infection with Toxoplasma gondii

  • Positive PCR for Toxoplasma gondii from either the aqueous or vitreous specimen

     OR

  • Positive serum IgM antibodies against Toxoplasma gondii


OR


B. Characteristic clinical ocular features

  1. Hyperpigmented and/or atrophic chorioretinal scar (“toxoplasmic scar”) AND (2. or 3.)
  2. Round or oval retinitis lesions OR
  3. Recurrent acute (episodic) course
  1. Both negative IgG AND IgM antibodies against Toxoplasma gondii (unless there is a positive PCR for Toxoplasma gondii from an aqueous or vitreous specimen)
  2. Positive serology for syphilis using a treponemal test
  3. Intraocular specimen PCR-positive for herpes simplex virus, varicella zoster virus or cytomegalovirus (unless there is immune compromise, morphologic evidence for >1 infection, the characteristic picture of toxoplasmic retinitis, and the intraocular fluid specimen also has a positive PCR for T. gondii)
 8.2 10
Tubercular posterior uveitis[24]
  • Evidence of a tubercular uveitis compatible uveitic syndrome
    1. anterior uveitis with iris nodules
    2. serpiginous-like tubercular choroiditis
    3. choroidal nodule (ie, tuberculoma)
    4. in individuals with active systemic tuberculosis, multifocal choroiditis
    5. occlusive retinal vasculitis

AND

  • Evidence of infection with Mycobacterium tuberculosis, either
    1. histologically or microbiologically confirmed infection with M. tuberculosisa OR
    2. positive IGRA* OR
    3. positive tuberculin skin test
  1. Positive serology for syphilis using a treponemal test
  2. Positive biopsy for sarcoidosis (and therefore an absence of histologic or microbiologic confirmation of infection with M. tuberculosis)
  3. Uveitic syndrome compatible with either sarcoidosis-associated uveitis or tubercular uveitis and bilateral hilar adenopathy on chest imaging without histologic or microbiologic confirmation of the diagnosis of infection with M. tuberculosis
 3.4

*Overall misclassification rate for tubercular uveitis.

3.6

*Overall misclassification rate for tubercular uveitis.

Sarcoidosis-associated posterior uveitis[14] Systemic-disease associated
  • Compatible uveitic picture, either
    1. Anterior uveitis OR
    2. Intermediate or anterior/intermediate uveitis OR
    3. Posterior uveitis with either choroiditis (paucifocal choroidal nodule[s] or multifocal choroiditis) OR
    4. Panuveitis with choroiditis or retinal vascular sheathing or retinal vascular occlusion

AND

  • Evidence of sarcoidosis, either
    1. Tissue biopsy demonstrating non-caseating granulomata OR
    2. Bilateral hilar adenopathy on chest imaging
  1. Positive serology for syphilis using a treponemal test
  2. Evidence of infection with Mycobacterium tuberculosis[a] ,either
    1. Histologically- or microbiologically-confirmed infection with M. tuberculosis OR
    2. Positive interferon-Ɣ release assay (IGRA) OR
    3. Positive tuberculin skin test
 N/A N/A
Acute posterior multifocal placoid pigment epitheliopathy (APMPEE)[25] Eye-limited Paucifocal or multifocal choroidal lesions on clinical examination with:
  • Plaque-like or placoid appearance to the lesions

AND

  • Characteristic fluorescein angiogram in the acute phase of the disease (lesions are hypofluorescent early and diffusely hyperfluorescent late)
  1. Positive serologic test for syphilis using a treponemal test
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating noncaseating granulomata)
 5 0
Birdshot chorioretinitis[26] [#’s 1, 2, and 3] OR # 4


1. Characteristic bilateral multifocal choroiditis on ophthalmoscopy

  • Multifocal cream-colored or yellow-orange, oval or round choroidal lesions (“birdshot spots”)


AND


2. Absent to mild anterior chamber inflammation

  1. Absent to mild anterior chamber cells AND
  2. No keratic precipitates AND
  3. No posterior synechiae


AND


3. Absent to moderate vitritis


OR


4. Multifocal choroiditis with

  1. Positive HLA-A29 test AND either (2. or 3.)
  2. Characteristic “birdshot” spots (multifocal cream-colored or yellow-orange, oval or round choroidal lesions) on ophthalmoscopy OR
  3. Characteristic indocyanine green angiogram (multifocal hypofluorescent spots) without characteristic “birdshot” spots on ophthalmoscopy
  1. Positive serologic test for syphilis using a treponemal test
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating noncaseating granulomata)
  3. Evidence of intraocular lymphoma on diagnostic vitrectomy or tissue biopsy
 10 0
Multiple evanescent white dot syndrome (MEWDS)[27]
  • Multifocal chorioretinal gray-white spots with foveal granularity

Characteristic fluorescein angiogram or optical coherence tomogram (OCT)

  1. “Wreath-like” hyperfluorescent lesions on fluorescein angiogram OR
  2. Hyperreflective lesions on OCT extending from the retinal pigment epithelium, into and/or through the ellipsoid zone into the outer nuclear layer of the retina

Absent to mild anterior chamber and vitreous inflammation

  1. Positive serologic test for syphilis using a treponemal test
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating non-caseating granulomata)
  3. Bilateral simultaneous disease onset
 7 0
Multifocal choroiditis with panuveitis (MFCPU)[28]

*

  • Multifocal choroiditis with
    1. Oval or round lesions AND
    2. Predominant lesion size >125 µm

AND

  • Characteristic appearance
    1. “Punched-out atrophic” chorioretinal scars OR
    2. Active lesions with more than minimal vitreous inflammation

AND

  • Inflammatory lesions and/or characteristic scars involving the midperiphery or periphery with or without posterior pole involvement
  1. Positive serologic test for syphilis using a treponemal test
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating noncaseating granulomata)
  3. In tuberculosis-endemic regions or tuberculosis-exposed individuals, evidence of infection with Mycobacterium tuberculosis
    1. Histologically or microbiologically confirmed infection with M. tuberculosis OR
    2. Positive interferon-γ release assay OR
    3. Positive tuberculin skin test
 15 0
Punctate inner choroiditis (PIC)[29]

*

  • Multifocal choroidal inflammatory lesions
    1. Predominant lesion size of <250 µm and
    2. Punctate lesion appearance

AND

  • Lesion involvement of posterior pole with or without mid-periphery

AND

  • Absent to minimal anterior chamber and vitreous inflammation
  1. Positive serologic test result for syphilis using a treponemal test
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy results demonstrating non-caseating granulomata)
 15 9
Serpiginous choroiditis[30]
  • Paucifocal or multifoca choroiditis with an ameboid or serpentine shape, not necessarily contiguous with the optic disc

AND

  • Characteristic imaging
    1. Fluorescein angiogram with early diffuse hypofluorescent lesions and late hyperfluorescent lesion borders OR
    2. Fundus autofluorescence with hypo-autofluorescent lesions with hyper-autofluorescent borders

AND

  • Absent to minimal anterior chamber and vitreous inflammation
  1. Positive serologic test for syphilis using a treponemal test
  2. Evidence of sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating noncaseating granulomata)
  3. Evidence of infection with Mycobacterium tuberculosis, either
    1. Histologically or microbiologically confirmed infection with M. tuberculosis  OR
    2. Positive interferon-γ release assay OR
    3. Positive tuberculin skin test
 0 0
*Some researchers consider MFCPU and PIC as variants due to their rare co-occurrence, similar imaging appearance, and shared features, while others note distinctions in morphology and clinical course. A cluster analysis of the two diseases identified two distinct groups based on inflammation and lesion location: anterior chamber and virtuous inflammation are largely absent in PIC and lesion location tends to be posterior in PIC and peripheral in MFCPU.[31] Histologic evaluations suggest differences, but genetic studies indicate possible similarities in pathogenesis. Despite some overlap, the SUN Working Group chooses to define PIC and MFCPU separately, acknowledging occasional cases with mixed features.

Panuveitides

Table 6: Classification criteria of panuveitides. Adapted from multiple tables from Standardization of Uveitis Nomenclature (SUN) Working Group 2021.[9][14][24][32][33]
Name Etiology Classification Criteria Exclusion Criteria Misclassification rate training set (%) Misclassification rate validation set (%)
Syphilitic panuveitis[9]
  • Uveitis with a compatible uveitic presentation, including
    1. Anterior uveitis OR
    2. Intermediate uveitis or anterior/intermediate uveitis OR
    3. Posterior uveitis or panuveitis with 1 of the following presentations:
      1. Placoid inflammation of the retinal pigment epithelium or
      2. Multifocal inflammation of the retina/retinal pigmentepithelium or
      3. Necrotizing retinitis or
      4. Retinal vasculitis

AND

  • Evidence of infection with Treponema pallidum, either
    1. Positive treponemal test and nontreponemal test
    2. Positive treponemal test with 2 different treponemal tests
  1. History of adequate treatment for syphilitic uveitis[16]
 0 0
Tubercular panuveitis[24]
  • Evidence of a tubercular uveitis compatible uveitic syndrome
    1. anterior uveitis with iris nodules
    2. serpiginous-like tubercular choroiditis
    3. choroidal nodule (ie, tuberculoma)
    4. in individuals with active systemic tuberculosis, multifocal choroiditis
    5. occlusive retinal vasculitis

AND

  • Evidence of infection with Mycobacterium tuberculosis, either
    1. histologically or microbiologically confirmed infection with M. tuberculosisa OR
    2. positive IGRA* OR
    3. positive tuberculin skin test
  1. Positive serology for syphilis using a treponemal test
  2. Positive biopsy for sarcoidosis (and therefore an absence of histologic or microbiologic confirmation of infection with M. tuberculosis)
  3. Uveitic syndrome compatible with either sarcoidosis-associated uveitis or tubercular uveitis and bilateral hilar adenopathy on chest imaging without histologic or microbiologic confirmation of the diagnosis of infection with M. tuberculosis
 3.4

*Overall misclassification rate for tubercular uveitis.

3.6

*Overall misclassification rate for tubercular uveitis.

Behçet disease uveitis[34]
  • Compatible uveitic syndrome
    1. Anterior uveitis
    2. Anterior and intermediate uveitis
    3. Posterior uveitis with retinal vasculitis and/or focal retinal infiltrates
    4. Panuveitis with retinal vasculitis and/or focal retinal infiltrates

AND

  • A diagnosis of Behçet disease using International Study Group for Behçet Disease criteria[35] : Oral ulcers PLUS any two of the following
    1. Genital ulcers
    2. Uveitis (typical defined eye lesions)
    3. Typical defined skin lesions
    4. Positive pathergy test
  1. Positive serology for syphilis using a treponemal test
  2. Evidence for sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating noncaseating granulomata)
 0.6 0
Sarcoidosis-associated panuveitis[14]
  • Compatible uveitic picture, either
    1. Anterior uveitis OR
    2. Intermediate or anterior/intermediate uveitis OR
    3. Posterior uveitis with either choroiditis (paucifocal choroidal nodule[s] or multifocal choroiditis) OR
    4. Panuveitis with choroiditis or retinal vascular sheathing or retinal vascular occlusion

AND

  • Evidence of sarcoidosis, either
    1. Tissue biopsy demonstrating non-caseating granulomata OR
    2. Bilateral hilar adenopathy on chest imaging
  1. Positive serology for syphilis using a treponemal test
  2. Evidence of infection with Mycobacterium tuberculosis ,either
    1. Histologically- or microbiologically-confirmed infection with M. tuberculosis OR
    2. Positive interferon-Ɣ release assay (IGRA) OR
    3. Positive tuberculin skin test
 1.2 0
Early-Stage Vogt-Koyanagi-Harada Disease[32]
Diagnosis requires #1 or #2 AND #3


1. Evidence of Harada disease

  1. Serous (exudative) retinal detachment AND (2. and/or 3)
  2. Multiloculated appearance on fluorescein angiogram OR
  3. Septae on optical coherence tomogram


OR


2. Panuveitis with ≥2 of the following neurologic symptoms or signs:

  1. Headache
  2. Tinnitus
  3. Dysacusis
  4. Meningismus
  5. Cerebrospinal fluid pleocytosis


AND


3. No history of penetrating ocular trauma or vitreoretinal surgery prior to disease onset

  1. Positive serology for syphilis using a treponemal test
  2. Evidence for sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating noncaseating granulomata)
 8.0 7.7
Late-Stage Vogt-Koyanagi-Harada Disease[32]
  • History of early-stage Vogt-Koyanagi-Harada disease


AND

  • Sunset glow fundus


OR

  • Uveitis AND ≥1 of the following cutaneous findings

a. Vitiligo

b. Poliosis

c. Alopecia

  1. Positive serology for syphilis using a treponemal test
  2. Evidence for sarcoidosis (either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating noncaseating granulomata)
 1.0 12
Sympathetic ophthalmia[33] Eye-limited
  • History of unilateral ocular trauma or surgery


AND

  • Ocular inflammation, either
  1. Bilateral OR
  2. If there is no view in the inciting eye (e.g., enucleated, phthistic, opaque cornea), then inflammation is detectable in the sympathizing eye
 1.   Serology test positive for syphilis, using a treponemal test

2.   Evidence for sarcoidosis, either bilateral hilar adenopathy on chest imaging or tissue biopsy demonstrating non-caseating granulomata

4.2 6.7

References

  1. ↑ Jabs DA, Busingye J. Approach to the diagnosis of the uveitides. Am J Ophthalmol. Aug 2013;156(2):228-36. doi:10.1016/j.ajo.2013.03.027
  2. ↑ Nussenblatt RB. The natural history of uveitis. Int Ophthalmol. Oct 1990;14(5-6):303-8. doi:10.1007/BF00163549
  3. ↑ Jabs DA, Nussenblatt RB, Rosenbaum JT, Group SoUNSW. Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol. Sep 2005;140(3):509-16. doi:10.1016/j.ajo.2005.03.057
  4. ↑ Jabs DA, Dick A, Doucette JT, et al. Interobserver Agreement Among Uveitis Experts on Uveitic Diagnoses: The Standardization of Uveitis Nomenclature Experience. Am J Ophthalmol. Feb 2018;186:19-24. doi:10.1016/j.ajo.2017.10.028
  5. ↑ 5.0 5.1 5.2 Group SoUNSW. Development of Classification Criteria for the Uveitides. Am J Ophthalmol. Aug 2021;228:96-105. doi:10.1016/j.ajo.2021.03.061
  6. ↑ 6.0 6.1 Group SoUNSW. Classification Criteria for Herpes Simplex Virus Anterior Uveitis. Am J Ophthalmol. Aug 2021;228:231-236. doi:10.1016/j.ajo.2021.03.053
  7. ↑ 7.0 7.1 Group SoUNSW. Classification Criteria for Cytomegalovirus Anterior Uveitis. Am J Ophthalmol. Aug 2021;228:89-95. doi:10.1016/j.ajo.2021.03.060
  8. ↑ 8.0 8.1 Group SoUNSW. Classification Criteria for Varicella Zoster Virus Anterior Uveitis. Am J Ophthalmol. Aug 2021;228:165-173. doi:10.1016/j.ajo.2021.03.037
  9. ↑ 9.0 9.1 9.2 9.3 9.4 9.5 9.6 Group SoUNSW. Classification Criteria for Syphilitic Uveitis. Am J Ophthalmol. Aug 2021;228:182-191. doi:10.1016/j.ajo.2021.03.039
  10. ↑ 10.0 10.1 Group SoUNSW. Classification Criteria for Juvenile Idiopathic Arthritis-Associated Chronic Anterior Uveitis. Am J Ophthalmol. Aug 2021;228:192-197. doi:10.1016/j.ajo.2021.03.055
  11. ↑ 11.0 11.1 Aggarwal A, Misra DP. Enthesitis-related arthritis. Clin Rheumatol. Nov 2015;34(11):1839-46. doi:10.1007/s10067-015-3029-4
  12. ↑ 12.0 12.1 Group SoUNSW. Classification Criteria for Spondyloarthritis/HLA-B27-Associated Anterior Uveitis. Am J Ophthalmol. Aug 2021;228:117-125. doi:10.1016/j.ajo.2021.03.049
  13. ↑ 13.0 13.1 Group SoUNSW. Classification Criteria for Tubulointerstitial Nephritis With Uveitis Syndrome. Am J Ophthalmol. Aug 2021;228:255-261. doi:10.1016/j.ajo.2021.03.041
  14. ↑ 14.0 14.1 14.2 14.3 14.4 14.5 14.6 Group SoUNSW. Classification Criteria for Sarcoidosis-Associated Uveitis. Am J Ophthalmol. Aug 2021;228:220-230. doi:10.1016/j.ajo.2021.03.047
  15. ↑ 15.0 15.1 Group SoUNSW. Classification Criteria for Fuchs Uveitis Syndrome. Am J Ophthalmol. Aug 2021;228:262-267. doi:10.1016/j.ajo.2021.03.052
  16. ↑ 16.0 16.1 16.2 16.3 Prevention CDCa. Syphilis. 2015 Sexually Transmitted Diseases Treatment Guidelines. Available at cdc gov Accessed April. 2019;11
  17. ↑ 17.0 17.1 Group SoUNSW. Classification Criteria for Multiple Sclerosis-Associated Intermediate Uveitis. Am J Ophthalmol. Aug 2021;228:72-79. doi:10.1016/j.ajo.2021.03.044
  18. ↑ 18.0 18.1 Group SoUNSW. Classification Criteria For Pars Planitis. Am J Ophthalmol. Aug 2021;228:268-274. doi:10.1016/j.ajo.2021.03.045
  19. ↑ 19.0 19.1 Group SoUNSW. Classification Criteria for Intermediate Uveitis, Non-Pars Planitis Type. Am J Ophthalmol. Aug 2021;228:159-164. doi:10.1016/j.ajo.2021.03.054
  20. ↑ 20.0 20.1 Thompson AJ, Banwell BL, Barkhof F, et al. Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. The Lancet Neurology. 2018;17(2):162-173.
  21. ↑ 21.0 21.1 Group SoUNSW. Classification Criteria for Acute Retinal Necrosis Syndrome. Am J Ophthalmol. Aug 2021;228:237-244. doi:10.1016/j.ajo.2021.03.057
  22. ↑ 22.0 22.1 Group SoUNSW. Classification Criteria for Cytomegalovirus Retinitis. Am J Ophthalmol. Aug 2021;228:245-254. doi:10.1016/j.ajo.2021.03.051
  23. ↑ 23.0 23.1 Group SoUNSW. Classification Criteria for Toxoplasmic Retinitis. Am J Ophthalmol. Aug 2021;228:134-141. doi:10.1016/j.ajo.2021.03.042
  24. ↑ 24.0 24.1 24.2 24.3 Group SoUNSW. Classification Criteria for Tubercular Uveitis. Am J Ophthalmol. Aug 2021;228:142-151. doi:10.1016/j.ajo.2021.03.040
  25. ↑ 25.0 25.1 Group SoUNSW. Classification Criteria for Acute Posterior Multifocal Placoid Pigment Epitheliopathy. Am J Ophthalmol. Aug 2021;228:174-181. doi:10.1016/j.ajo.2021.03.056
  26. ↑ 26.0 26.1 Group SoUNSW. Classification Criteria for Birdshot Chorioretinitis. Am J Ophthalmol. Aug 2021;228:65-71. doi:10.1016/j.ajo.2021.03.059
  27. ↑ 27.0 27.1 Group SoUNSW. Classification Criteria For Multiple Evanescent White Dot Syndrome. Am J Ophthalmol. Aug 2021;228:198-204. doi:10.1016/j.ajo.2021.03.050
  28. ↑ 28.0 28.1 Group SoUNSW. Classification Criteria for Multifocal Choroiditis With Panuveitis. Am J Ophthalmol. Aug 2021;228:152-158. doi:10.1016/j.ajo.2021.03.043
  29. ↑ 29.0 29.1 Group SoUNSW. Classification Criteria for Punctate Inner Choroiditis. Am J Ophthalmol. Aug 2021;228:275-280. doi:10.1016/j.ajo.2021.03.046
  30. ↑ 30.0 30.1 Group SoUNSW. Classification Criteria for Serpiginous Choroiditis. Am J Ophthalmol. Aug 2021;228:126-133. doi:10.1016/j.ajo.2021.03.038
  31. ↑ Gilbert RM, Niederer RL, Kramer M, et al. Differentiating Multifocal Choroiditis and Punctate Inner Choroidopathy: A Cluster Analysis Approach. Am J Ophthalmol. May 2020;213:244-251. doi:10.1016/j.ajo.2020.01.031
  32. ↑ 32.0 32.1 32.2 Group SoUNSW. Classification Criteria for Vogt-Koyanagi-Harada Disease. Am J Ophthalmol. Aug 2021;228:205-211. doi:10.1016/j.ajo.2021.03.036
  33. ↑ 33.0 33.1 Group SoUNSW. Classification Criteria for Sympathetic Ophthalmia. Am J Ophthalmol. Aug 2021;228:212-219. doi:10.1016/j.ajo.2021.03.048
  34. ↑ Group SoUNSW. Classification Criteria for Behçet Disease Uveitis. Am J Ophthalmol. Aug 2021;228:80-88. doi:10.1016/j.ajo.2021.03.058
  35. ↑ Disease ISGfBs. Criteria for diagnosis of Behçet's disease. Lancet. 1990;335(8697):1078-1080.
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