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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)

The American Rheumatology Association criteria for the classification and diagnosis of Wegener's Granulomatosis (WG)

 


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
Oral aphthous ulceration
Skin lesions
Erythema nodosum-like eruptions
Subcutaneous thrombophlebitis
Cutaneous hypersensitivity
Ocular lesions
Recurrent hypopyon iritis or iridocyclitis
Chorioretinitis
Genital aphthous ulcerations

Minor Criteria
Arthritic symptoms and signs (arthralgia, swelling, redness in large joints)
Gastrointestinal lesions (appendicitis-like pains, melena, diarrhea,  and so on)
Epididymitis
Vascular lesions (obliterative vasculitis, occlusions, aneurysms)
Central nervous system involvement
Brain stem syndrome
Meningoencephalomyelitic syndrome
Psychiatric symptoms

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

  1. Fever persisting for more than 5 days
  2. Bilateral conjunctival injection
  3. Changes in the mouth consisting of  
    1. Erythema and fissuring of the lips
    2. Diffuse oropharyngeal erythema
    3. Strawberry tongue
  4. Changes in the peripheral extremities consisting of 
    1. Induration of the hands and feet
    2. Erythema of the palms and soles
    3. Desquamation of finger and toe tips approximately 2 weeks after onset
    4. Transverse grooves across fingernails, 2 to 3 months after onset
  5. Erythematous rash of torso
  6. 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 </=60 minutes

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
>15 mm diameter in postpubertal individuals

Neurofibroma

Any type: 2 or more or
Plexiform: 1 or more

Axillary and inguinal freckles

 

Optic nerve glioma

1 or more

Lisch nodules

2 or more

A distinctive osseous lesion

Sphenoid wing dysplasia or
Congenital bowing or thinning of long bone cortex, with
or without pseudoarthrosis

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
<30 year

First-degree relative with NF 2

+

Any two of the following:
meningioma, glioma,
schwannoma, juvenile posterior
subcapsular lenticular opacities / juvenile cortical cataract


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
MTP joints.

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
by a physician.

Serum rheumatoid factor

Demonstration of abnormal amounts of serum rheumatoid factor by any method for which the result has
been positive in less than 5% of normal control subjects.

Radiographic changes

Radiographic changes typical of RA on posteroanterior hand and wrist radiographs. These must include
erosions or unequivocal bony decalcification localized in, or most marked adjacent to, the involved joints (osteoarthritic changes alone do not qualify)

 


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

  1. Mutton-fat/granulomatous keratic precipitates (KPs) and/or iris nodules (Koeppe/Busacca)
  2. Trabecular meshwork (TM) nodules and/or tent-shaped peripheral anterior synechiae (PAS)
  3. Snowballs/string of pearls vitreous opacities
  4. Multiple chorioretinal peripheral lesions (active and/or atrophic)
  5. Nodular and/or segmental periphlebitis (±candlewax drippings) and/or retinal macroaneurysm in an inflamed eye
  6. Optic disc nodule(s)/granuloma(s) and/or solitary choroidal nodule
  7. Bilaterality

Laboratory Investigations or Investigational Procedures

  1. Negative tuberculin test in a BCG-vaccinated patient or in a patient with a previously positive tuberculin skin test.
  2. Elevated serum angiotensin converting enzyme (ACE) and/or elevated serum lysozyme
  3. Chest x-ray; look for bilateral hilar lymphadenopathy(BHL)
  4. Abnormal liver enzyme tests (any two of alkaline phosphatase, ASAT. ALAT, LDH orγ-GT)
  5. 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 Classification Criteria for Sjo¨gren’s Syndrome by American College of Rheumatology

The classification 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:


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 our mnemonic 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 fulfil the major criterion or two of the three minor criteria. (97 % sensitive and 98 % specific for systemic sclerosis)

Major criterion:

Minor criteria:


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
Any one of the following

 

Positive

One or more retinal capillary hemangioma
One or more CNS hemangioma
One or more visceral lesion*

 

Negative

Two or more retinal capillary hemangioma
One retinal hemangioma with a visceral lesion

Two or more
CNS hemangioma
One CNS hemangioma with a
visceral lesion

 


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%.

  1. Nasal or oral inflammation: Development of painful or painless oral ulcers or purulent or bloody nasal discharge
  2. Abnormal chest radiograph: Chest radiograph showing the presence of nodules, fixed infiltrates, or cavities
  3. Urinary sediment: Microhematuria (>5 red blood cells per high power field) or red cell casts in urine sediment
  4. 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).