Standard Diagnostic Criteria for various conditions
Diagnostic criteria for acute retinal necrosis: American Uveitis Society
Diagnostic Criteria for Behçet’s Disease
Diagnostic Criteria for Birdshot Chorioretinopathy for Research Purposes
ICHD-II Diagnostic Criteria for Cluster Headache
Diagnostic criteria for cryptophthalmos syndrome
Diagnostic criteria for Kawasaki disease
Criteria for Diagnosis of Lyme Disease
ICHD-II Diagnostic Criteria for Migraine
Diagnostic Criteria for Neurofibromatosis
American Rheumatism Association’s Revised Criteria for Rheumatoid Arthritis
International Criteria for the Diagnosis of Ocular Sarcoidosis
New Classification Criteria for Sjo¨gren’s Syndrome by American College of Rheumatology
AMERICAN COLLEGE of RHEUMATOLOGY CLASSIFICATION CRITERIA FOR SLE
The American Rheumatology Association’s Criteria for the Classification of Systemic Sclerosis
Diagnostic Criteria for Tension-type headache
Diagnostic Criteria for von Hippel–Lindau Disease
Revised Criteria for Diagnosis of Vogt–Koyanagi–Harada Disease (VKH)
Diagnostic criteria for acute retinal necrosis: American Uveitis Society
Holland GN. Executive committee of the American uveitis society. Am J Ophthalmol .1994; 117:6 63–667.
In 1994, the Executive Committee of the American Uveitis Society published some diagnostic criteria for acute retinal necrosis. These includes
1.One or more discrete foci of retinal necrosis in the periphery,
2.Rapid disease progression in the absence of therapy,
3.Circumferential spread of the lesions,
4.Occlusive arteriolar vasculopathy, and
5.A prominent vitreous or anterior chamber inflammatory reaction.
Other characteristics that support, but are not required for, a diagnosis of acute retinal necrosis include optic neuropathy, scleritis and pain.
Diagnostic Criteria for Behçet’s Disease
From Behçet’s Disease Research Committee” Clinical Research Section Recommendation. Jpn Ophthalmol 1974; 18:291
Major Criteria |
Minor Criteria |
Types of Behçet’s Disease
Complete:All four major symptoms appear in the clinical course
Incomplete:
Three of four major symptoms appear in the clinical course
Recurrent hypopyon–iritis or typical chorioretinitis and one other major symptom appear in the clinical course
Suspect:Two major criteria appear in the clinical course
Possible:One major criterion appears in the clinical course
Diagnostic Criteria for Behçet’s Disease (International Study Group for Behcet’s disease)
International Study Group for Behcet’s Disease. Criteria for diagnosis of Behcet’s Disease. Lancet 1990; 335:1078.
Recurrent oral ulceration: Minor aphtous,major aphtous,or herpetiform ulceration observed by physician or patient, which recurred at least three times in one-12 month period.
Plus 2 of:
Recurrent genital ulceration: Aphthous ulceration or scarring, observed by physician or patient.
Eye lesions: Anteror uveitis, posterior uveitis, or cells in vitreous on slit-lamp examination; or retinal vasculitis observed by ophthalmologist.
Skin lesions: Erythema nodosum observed by physician or patient, pseudofolliculitis, or papulopustular lesions; or acneiform nodules observed by physician in post adolescent patients not on corticosteroid treatment.
Positive pathergy test: Read by physician at 24–48 h.
Diagnostic Criteria for Birdshot Chorioretinopathy for Research Purposes
Levinson RD, Brezin A, Rothova A, et al: Research criteria for the diagnosis of birdshot chorioretinopathy: results of an international consensus conference. Am J Ophthalmol 2006; 141:185-187.
Required Findings
• Bilateral disease
• Presence of at least three ‘birdshot lesions’ inferior or nasal to the optic disk in one eye
• Low-grade anterior segment inflammation (≤1+ cells in the anterior chamber)
• Low-grade vitreous inflammatory reaction (≤ 2+ vitreous haze)
Supportive Findings
• HLA-A29 positive
• Retinal vasculitis
• CME
Exclusion Criteria
• Keratic precipitates
• Posterior synechiae
• Presence of infectious, neoplastic, or other inflammatory disease that can cause multifocal choroidal lesions
ICHD-II Diagnostic Criteria for Cluster Headache
A. At least 5 attacks fulfilling criteria B-D
B. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 minutes if untreated
C. Headache is accompanied by at least 1 of the following:
Ipsilateral conjunctival injection and/or lacrimation
Ipsilateral nasal congestion and/or rhinorrhoea
Ipsilateral eyelid oedema
Ipsilateral forehead and facial sweating
Ipsilateral miosis and/or ptosis
A sense of restlessness or agitation
D. Attacks have a frequency from 1 every other day to 8/day
E. Not attributed to another disorder
Diagnostic criteria for cryptophthalmos syndrome
For diagnosis of cryptophthalmos syndrome, patients must have at least two major criteria and one minor criterion, or they may have one major criterion and four minor criteria.
Major criteria |
Minor criteria |
1. Cryptophthalmos |
1.Congenital malformation of the nose |
2. Syndactyly |
2.Congenital malformation of the ears |
3. Abnormal genitalia |
3.Congenital malformation of the larynx |
4.Sibling with cryptophthalmos syndrome |
4. Cleft lip and/or palate |
5. Skeletal defects |
|
6. Umbilical hernia |
|
7. Renal agenesis |
|
8. Mental retardation |
Diagnostic criteria for Kawasaki disease
Melish ME, Hicks RV: Kawasaki syndrome: clinical features. Pathophysiology, etiology and therapy. J Rheumatol 1990; 17(suppl 24):2-10.
A patient who fulfills five of the following six criteria should be considered to have Kawasaki disease
- Fever persisting for more than 5 days
- Bilateral conjunctival injection
- Changes in the mouth consisting of
- Erythema and fissuring of the lips
- Diffuse oropharyngeal erythema
- Strawberry tongue
- Changes in the peripheral extremities consisting of
- Induration of the hands and feet
- Erythema of the palms and soles
- Desquamation of finger and toe tips approximately 2 weeks after onset
- Transverse grooves across fingernails, 2 to 3 months after onset
- Erythematous rash of torso
- Enlarged lymph node mass greater than 1.5 cm in diameter
Criteria for Diagnosis of Lyme Disease
Morb Mortal Wkly Rep. 1997:46(RR-10):1–55 and Recommendations for public health surveillance. Morb Mortal Wkly Rep. 1995;44:590–1
Presence of any one of the following criteria satisfies the diagnosis of Lyme disease:
1.Development of erythema migrans (EM) within 30 days of exposure in an endemic area; size of lesion should be at least 5 cm
2.In the absence of EM, history of exposure to endemic area, with signs involving one organ system and positive laboratory test
3.No history of exposure to endemic area, but with EM as well as involvement of two organ systems
4.No history of exposure to endemic area, but with EM and a positive serology
ICHD-II Diagnostic Criteria for Migraine
Migraine without aura
A. At least 5 attacks fulfilling criteria B-D
B. Headache attacks lasting 4-72 hours (untreated or unsuccessfully treated)
C. Headache has at least 2 of the following characteristics:
Unilateral location
Pulsating quality
Moderate or severe pain intensity
Aggravation by or causing avoidance of routine physical activity (eg, walking or climbing stairs)
D. During headache at least 1 of the following:
Nausea and/or vomiting
Photophobia and phonophobia
E. Not attributed to another disorder
Migraine with aura
A. At least 2 attacks fulfilling criterion B
B. Migraine aura fulfilling criteria B-C for one of the subforms (typical aura with migraine headache, typical aura with non-migraine headache, typical aura without headache, familial hemiplegic migraine, sporadic hemiplegic migraine, or basilar-type migraine)
C. Not attributed to another disorder
Typical aura with migraine headache
A. At least 2 attacks fulfilling criteria B-D
B. Aura consisting of at least 1 of the following, but no motor weakness:
Fully reversible visual symptoms including positive features (eg, flickering lights, spots or lines) and/or negative features (ie, loss of vision)
Fully reversible sensory symptoms including positive features (ie, pins and needles) and/or negative features (ie, numbness)
Fully reversible dysphasic speech disturbance
C. At least two of the following:
Homonymous visual symptoms and/or unilateral sensory symptoms
At least one aura symptom develops gradually over >/=5 minutes and/or different aura symptoms occur in succession over >/=5 minutes
Each symptom lasts >/=5 and
D. Headache fulfilling criteria B-D for “Migraine without aura” begins during the aura or follows aura within 60 minutes
E. Not attributed to another disorder
Diagnostic Criteria for Neurofibromatosis
Diagnostic Criteria for Neurofibromatosis Type 1
National Institute of Health Consensus Development Conference. Arch Neurol 1988; 45:575–578.
Presence of any two or more of the following Is diagnostic :
Café-au-laitspots (6 or more) |
>5 mm in diameter in prepubertal individuals |
Neurofibroma |
Any type: 2 or more or |
Axillary and inguinal freckles |
|
Optic nerve glioma |
1 or more |
Lisch nodules |
2 or more |
A distinctive osseous lesion |
Sphenoid wing dysplasia or |
First-degree relative with NF 1 |
Diagnostic Criteria for Neurofibromatosis Type 2
National Institute of Health Consensus Development Conference. Arch Neurol 1988; 45:575–578.
Presence of any ONE of the following
Bilateral vestibular Schwannoma |
||
First-degree relative with NF 2 |
+ |
Unilateral vestibular schwannoma |
First-degree relative with NF 2 |
+ |
Any two of the following: |
American Rheumatism Association’s Revised Criteria for Rheumatoid Arthritis
A patient shall be said to have RA if he or she has satisfied at least four of these criteria. Criteria 1 through 4 must have been present for atleast 6 weeks. Patients with two clinical diagnoses are not excluded. Designation as classic, definite, or probable RA is not made.
Morning stiffness |
Morning stiffness in and around joints, lasting at least 1 h before maximal improvemen |
Arthritis of three or more joints |
At least three joint areas simultaneously have had soft- tissue swelling or fluid (not bone overgrowth alone) observed by a physician. The 14 possible areas are right or left PIP, MCP, wrist, elbow, knee, ankle, and |
Symmetric arthritis |
Simultaneous involvement of the same joint areas (as defined earlier) on both sides of the body (bilateral involvement of PIPs, MCPs, or MTPs is acceptable without absolute symmetry). |
Rheumatoid nodules |
Subcutaneous nodules, over bone prominences or extensor surfaces, or in juxtaarticular regions, observed |
Serum rheumatoid factor |
Demonstration of abnormal amounts of serum rheumatoid factor by any method for which the result has |
Radiographic changes |
Radiographic changes typical of RA on posteroanterior hand and wrist radiographs. These must include |
International Criteria for the Diagnosis of Ocular Sarcoidosis
Herbort, Carl P., Rao, Narsing A., Mochizuki, Manabu and the members of the Scientific Committee of the First International Workshop on Ocular Sarcoidosis (IWOS) (2009) International Criteria for the Diagnosis of Ocular Sarcoidosis: Results of the First International Workshop on Ocular Sarcoidosis (IWOS)',Ocular Immunology & Inflammation,17:3,160 — 169
Clinical signs suggestive of ocular sarcoidosis
- Mutton-fat/granulomatous keratic precipitates (KPs) and/or iris nodules (Koeppe/Busacca)
- Trabecular meshwork (TM) nodules and/or tent-shaped peripheral anterior synechiae (PAS)
- Snowballs/string of pearls vitreous opacities
- Multiple chorioretinal peripheral lesions (active and/or atrophic)
- Nodular and/or segmental periphlebitis (±candlewax drippings) and/or retinal macroaneurysm in an inflamed eye
- Optic disc nodule(s)/granuloma(s) and/or solitary choroidal nodule
- Bilaterality
Laboratory Investigations or Investigational Procedures
- Negative tuberculin test in a BCG-vaccinated patient or in a patient with a previously positive tuberculin skin test.
- Elevated serum angiotensin converting enzyme (ACE) and/or elevated serum lysozyme
- Chest x-ray; look for bilateral hilar lymphadenopathy(BHL)
- Abnormal liver enzyme tests (any two of alkaline phosphatase, ASAT. ALAT, LDH orγ-GT)
- Chest CT scan in patients with negative chest x-ray
Biopsy supported diagnosis with a compatible uveitis |
Definite oculara sarcoidosis |
Biopsy not done; presence of bilateral hilar lymphadenopathy (BHL) with a compatible uveitis |
Presumed ocular sarcoidosis |
Biopsy not done and BHL negative; presence of three of the suggestive intraocular signs and two positive investigational tests |
Probable ocular sarcoidosis |
Biopsy negative, four of the suggestive intraocular signsand two of the investigations are positive |
Possible ocular sarcoidosis |
New ClassificationCriteria for Sjo¨gren’s Syndrome by American College of Rheumatology
The classi?cation of SS, which applies to individuals with signs/symptoms that may be suggestive of SS, will be met in patients who have at least 2 of the following 3 objective features:
1. Positive serum anti-SSA/Ro and/or anti-SSB/La or (positive rheumatoid factor and ANA titer 1:320)
2. Labial salivary gland biopsy exhibiting focal lymphocytic sialadenitis with a focus score>1 focus/4 mm2
3. Keratoconjunctivitis sicca with ocular staining score> 3 (assuming that individual is not currently using daily eye drops for glaucoma and has not had corneal surgery or cosmetic eyelid surgery in the last 5 years)
Prior diagnosis of any of the following conditions would exclude participation in SS studies or therapeutic trials because of overlapping clinical features or interference with criteria tests:
- History of head and neck radiation treatment
- Hepatitis C infection
- Acquired immunode?ciency syndrome
- Sarcoidosis
- Amyloidosis
- Graft versus host disease
AMERICAN COLLEGE of RHEUMATOLOGY CLASSIFICATION CRITERIA FOR SLE
Serositis - Pleurisy, pericarditis on examination or diagnostic electrocardiogram (ECG) or imaging
Oral ulcers - Oral or nasopharyngeal, usually painless; palate is most specific
Arthritis - Nonerosive, two or more peripheral joints with tenderness or swelling
Photosensitivity - Unusual skin reaction to light exposure
Blood disorders - Leukopenia (< 4 × 103 cells/µL on more than one occasion), lymphopenia (< 1500 cells/µL on more than one occasion), thrombocytopenia (< 100 × 103 cells/µL in the absence of offending medications), hemolytic anemia
Renal involvement - Proteinuria (>0.5 g/d or 3+ positive on dipstick testing) or cellular casts
Antinuclear antibodies (ANAs) - Higher titers generally more specific (>1:160); must be in the absence of medications associated with drug-induced lupus
Immunologic phenomena - dsDNA; anti-Smith (Sm) antibodies; antiphospholipid antibodies (anticardiolipin immunoglobulin G [IgG] or immunoglobulin M [IgM] or lupus anticoagulant); biologic false-positive serologic test results for syphilis, lupus erythematosus (LE) cells (omitted in 1997 revised criteria)
Neurologic disorder - Seizures or psychosis in the absence of other causes
Malar rash - Fixed erythema over the cheeks and nasal bridge, flat or raised
Discoid rash - Erythematous raised-rimmed lesions with keratotic scaling and follicular plugging, often scarring
These criterias can be remembered with "SOAP BRAIN MD" mnemonic(Visit ourmnemonic section for other interesting collections of mnemonics in Ophthalmology.Click Here)
The American Rheumatology Association’s Criteria for the Classification of Systemic Sclerosis
Imboden J. B., Hellmann D. B., Stone J. H. : Current Rheumatology Diagnosis and Treatment, Second Edition, McGraw-Hill Companies Inc,2007
The patient should fulfill the major criterion or two of the three minor criteria. (97 % sensitive and 98 % specific for systemic sclerosis)
Major criterion:
- Proximal diffuse (truncal) sclerosis (skin tightness, thickening, non-pitting indurations)
Minor criteria:
- Sclerodactyly (only fingers and/or toes)
- Digital pitting scars or loss of substance of the digital finger pads (pulp loss)
- Bilateral basilar pulmonary fibrosis
Diagnostic Criteria for Tension-type headache
Infrequent episodic tension-type headache
A. At least 10 episodes occurring on <1 day/month on average (<12 days/year) and fulfilling criteria B-D
B. Headache lasting from 30 minutes to 7 days
C. Headache has at least 2 of the following characteristics:
Bilateral location
Pressing/tightening (non-pulsating) quality
Mild or moderate intensity
Not aggravated by routine physical activity such as walking or climbing stairs
D. Both of the following:
No nausea or vomiting (anorexia may occur)
No more than one of photophobia or phonophobia
E. Not attributed to another disorder
Diagnostic Criteria for von Hippel–Lindau Disease
Family history |
Required feature |
|
Positive |
One or more retinal capillary hemangioma |
|
Negative |
Two or more retinal capillary hemangioma |
Two or more |
Revised Criteria for Diagnosis of Vogt–Koyanagi–Harada Disease (VKH)
Read RW, Holland GN, Rao NA, et al: Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: report of an international committee on nomenclature. Am J Ophthalmol 2001; 131:647–652.
1.No history of penetrating ocular trauma or surgery preceding the initial onset of uveitis
2.No clinical or laboratory evidence suggestive of other ocular disease entities
3.Bilateral ocular involvement (a or b must be met, depending on the stage of disease when the patient is examined)
A.Early manifestations of disease (i) evidence of diffuse choroiditis (with or without anterior uveitis, vitreous inflammatory reaction, or optic disk hyperemia) which may manifest as (a) focal areas of subretinal fluid, or (b) bullous serous retinal detachments
B.late manifestations of disease
(i)History suggestive of prior presence of early findings noted in 3a and either (i) or (iii) below, or multiple signs from 3
(ii)Ocular depigmentation: either (a) sunset glow fundus or (b) Sugiura's sign
(iii)Other ocular signs including (a) nummular chorioretinal depigmented scars, or (b) retinal pigment epithelium clumping and/or migration, or © recurrent or chronic anterior uveitis
4.Neurological/auditory findings (may resolve by time of evaluation)
a.Meningismus (malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back, or a combination of these factors); note that headache alone is not sufficient to meet the definition of meningismus
b.Tinnitus
c.Cerebrospinal fluid pleocytosis
5.Integumentary finding (not preceding onset of central nervous system or ocular disease)
a.Alopecia, or
b.Poliosis, or
c.Vitiligo
Complete VKH:Criteria 1–5 must be present.
Incomplete VKH:Criteria 1–3 and either 4 or 5 must be present.
Probable VKH (isolated ocular disease):Criteria 1–3 must be present.
The American Rheumatology Association criteria for the classification and diagnosis of Wegener's Granulomatosis (WG)
Leavitt R.Y., Fauci A.S., Bloch D.A., Michel B.A., Hunder G.G., Arend W.P., et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33:1101- 07.
A patient shall be said to have Wegener's granulomatosis if at least 2 of these 4 features are present. The presence of any 2 or more features yields a sensitivity of 88.2% and a specificity of 92.0%.
- Nasal or oral inflammation: Development of painful or painless oral ulcers or purulent or bloody nasal discharge
- Abnormal chest radiograph: Chest radiograph showing the presence of nodules, fixed infiltrates, or cavities
- Urinary sediment: Microhematuria (>5 red blood cells per high power field) or red cell casts in urine sediment
- Granulomatous inflammation on biopsy: Histologic changes showing granulomatous inflammation within the wall of an artery or in the perivascular or extravascular area (artery or arteriole).