Criteria

AOSD classification criteria ¹²³⁴

Cush et al (1987)	Yamaguchi et al (1992)	Fautrel et al (2002)
2 points each:	Major criteria:	Major criteria:
Quotidian fever >39°C	Fever >39°C, intermittent, ≥1 week	Spiking fever ≥39°C
Evanescent rash	Arthralgia ≥2 weeks	Arthralgia
WBC count >12,000/mL and ESR >40 mm/1 hr	Characteristic rash	Transient erythema
Negative ANA and RF	WBC count >10,000/mL (>80% granulocytes)	Pharyngitis
Carpal ankylosis		PMN leukocytes ≥80%
		Glycosylated ferritin ≤20%
1 point each:	Minor criteria:	Minor criteria:
Onset age >35 yr	Sore throat	Maculopapular rash
Arthritis	Lymphadenopathy or splenomegaly	WBC count >10,000/Ml
Sore throat	LFT abnormal
RES involvement or LFT abnormal	Negative ANA/RF
Serositis
Cervical or tarsal ankylosis
	Exclusion criteria:
	Infections
	Malignancies
	Rheumatic diseases
Classification:	Classification:	Classification:
Probable AOSD: 10 pts during 12-week observation	5 criteria: at least 2 major and 3 minor	4 major criteria or 3 major + 2 minor
Definite AOSD: 10 pts with 6-months observation

Revised Antiphospholipid Syndrome ⁵

Antiphospholipid syndrome is present if at least one of the clinical criteria and one of the laboratory criteria that follow are met*
Clinical criteria
1. Vascular thrombosis¶
One or more clinical episodesΔ of arterial, venous, or small vessel thrombosis◊, in any tissue or organ. Thrombosis must be confirmed by objective validated criteria (ie, unequivocal findings of appropriate imaging studies or histopathology). For histopathologic confirmation, thrombosis should be present without significant evidence of inflammation in the vessel wall
2. Pregnancy morbidity
a. One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation, with normal fetal morphology documented by ultrasound or by direct examination of the fetus; or
b. One or more premature births of a morphologically normal neonate before the 34th week of gestation because of: (i) eclampsia or severe preeclampsia defined according to standard definitions, or (ii) recognized features of placental insufficiency§; or
c. Three or more unexplained consecutive spontaneous abortions before the 10th week of gestation, with maternal anatomic or hormonal abnormalities and paternal and maternal chromosomal causes excluded.
In studies of populations of patients who have more than one type of pregnancy morbidity, investigators are strongly encouraged to stratify groups of subjects according to a, b, or c above.
Laboratory criteria¥
1. LA present in plasma, on two or more occasions at least 12 weeks apart, detected according to the guidelines of the International Society on Thrombosis and Haemostasis (Scientific Subcommittee on LAs/phospholipid-dependent antibodies).
2. aCL of IgG and/or IgM isotype in serum or plasma, present in medium or high titer (ie, >40 GPL or MPL, or >the 99th percentile), on two or more occasions, at least 12 weeks apart, measured by a standardized ELISA.
3. Anti-beta-2 glycoprotein-I antibody of IgG and/or IgM isotype in serum or plasma (in titer >the 99th percentile), present on two or more occasions, at least 12 weeks apart, measured by a standardized ELISA, according to recommended procedures.
* Classification of APS should be avoided if less than 12 weeks or more than five years separate the positive aPL test and the clinical manifestation.
¶ Coexisting inherited or acquired factors for thrombosis are not reasons for excluding patients from APS trials. However, two subgroups of APS patients should be recognized, according to: (a) the presence; and (b) the absence of additional risk factors for thrombosis. Indicative (but not exhaustive) cases include: age (>55 in men and >65 in women) and the presence of any of the established risk factors for cardiovascular disease (hypertension, diabetes mellitus, elevated LDL or low HDL cholesterol, cigarette smoking, family history of premature cardiovascular disease, body mass index ≥30 kg m–2, microalbuminuria, estimated GFR <60 mL minute–1), inherited thrombophilias, oral contraceptives, nephrotic syndrome, malignancy, immobilization, and surgery. Thus, patients who fulfill criteria should be stratified according to contributing causes of thrombosis.
Δ A thrombotic episode in the past could be considered as a clinical criterion, provided that thrombosis is proved by appropriate diagnostic means and that no alternative diagnosis or cause of thrombosis is found.
◊ Superficial venous thrombosis is not included in the clinical criteria.
§ Generally accepted features of placental insufficiency include: (i) abnormal or non-reassuring fetal surveillance test(s), eg, a non-reactive non-stress test, suggestive of fetal hypoxemia; (ii) abnormal Doppler flow velocimetry waveform analysis suggestive of fetal hypoxemia, eg, absent end-diastolic flow in the umbilical artery; (iii) oligohydramnios, eg, an amniotic fluid index of 5 cm or less; or (iv) a postnatal birth weight less than the 10th percentile for the gestational age.
¥ Investigators are strongly advised to classify APS patients in studies into one of the following categories: I, more than one laboratory criteria present (any combination); IIa, LA present alone; IIb, aCL antibody present alone; IIc, anti-beta-2 glycoprotein-I antibody present alone.

Rheumatoid arthritis

2010 ACR/EULAR classification criteria

Target population (Who should be tested?): Patients who
1. Have at least one joint with definite clinical synovitis (swelling)*
2. With the synovitis not better explained by another disease†
Classification criteria for RA (score-based algorithm: add score of categories A–D; a score of ≥6 of 10 is needed for classification of a patient as having definite RA)‡	Score
A. Joint involvement§
1 large joint∥	0
2–10 large joints	1
1–3 small joints (with or without involvement of large joints)¶	2
4–10 small joints (with or without involvement of large joints)	3
>10 joints (at least 1 small joint)**	5
B. Serology (at least 1 test result is needed for classification)††
Negative RF and negative ACPA	0
Low-positive RF or low-positive ACPA	2
High-positive RF or high-positive ACPA	3
C. Acute-phase reactants (at least 1 test result is needed for classification)‡‡
Normal CRP and normal ESR	0
Abnormal CRP or abnormal ESR	1
D. Duration of symptoms§§
<6 weeks	0
≥6 weeks	1
*The criteria are aimed at classification of newly presenting patients. In addition, patients with erosive disease typical of rheumatoid arthritis (RA) with a history compatible with prior fulfillment of the 2010 criteria should be classified as having RA. Patients with longstanding disease, including those whose disease is inactive (with or without treatment) who, based on retrospectively available data, have previously fulfilled the 2010 criteria should be classified as having RA.
†Differential diagnoses vary among patients with different presentations but may include conditions such as systemic lupus erythematosus, psoriatic arthritis, and gout. If it is unclear about the relevant differential diagnoses to consider, an expert rheumatologist should be consulted.
‡Although patients with a score of 6 of 10 are not classifiable as having RA, their status can be reassessed, and the criteria might be fulfilled cumulatively over time.
§Joint involvement refers to any swollen or tender joint on examination, which may be confirmed by imaging evidence of synovitis. Distal interphalangeal joints, first carpometacarpal joints, and first metatarsophalangeal joints are excluded from assessment. Categories of joint distribution are classified according to the location and number of involved joints, with placement into the highest category possible based on the pattern of joint involvement.
∥“Large joints” refers to shoulders, elbows, hips, knees, and ankles.
¶“Small joints” refers to the metacarpophalangeal joints, proximal interphalangeal joints, second through fifth metatarsophalangeal joints, thumb interphalangeal joints, and wrists.
**In this category, at least one of the involved joints must be a small joint; the other joints can include any combination of large and additional small joints, as well as other joints not specifically listed elsewhere (e.g., temporomandibular, acromioclavicular, sternoclavicular).
††Negative refers to IU values that are less than or equal to the upper limit of normal (ULN) for the laboratory and assay; low-positive refers to IU values that are higher than the ULN but ≤3 times the ULN for the laboratory and assay; high-positive refers to IU values that are ≥3 times the ULN for the laboratory and assay. When rheumatoid factor (RF) information is only available as positive or negative, a positive result should be scored as low positive for RF.
‡‡Normal and abnormal are determined by local laboratory standards.
§§Duration of symptoms refers to patient self-report of the duration of signs or symptoms of synovitis (e.g., pain, swelling, tenderness) of joints that are clinically involved at the time of assessment regardless of treatment status.

1987 ACR (formerly ARA) classification criteria

Criterion	Description
Morning stiffness	Morning stiffness in and around the joints, lasting at least one hour before maximal improvement.
Arthritis of three or more joint areas	At least three joint areas (out of 14 possible areas; right or left PIP, MCP, wrist, elbow, knee, ankle, MTP joints) simultaneously have had soft tissue swelling or fluid (not bony overgrowth alone) as observed by a physician.
Arthritis of hand joints	At least one area swollen (as defined above) in a wrist, MCP, or PIP joint.
Symmetric arthritis	Simultaneous involvement of the same joint areas (as defined above) on both sides of the body (bilateral involvement of PIPs, MCPs, or MTPs, without absolute symmetry is acceptable).
Rheumatoid nodules	Subcutaneous nodules over bony prominences or extensor surfaces, or in juxta-articular regions as 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 percent of normal control subjects.
Radiographic changes	Radiographic changes typical of rheumatoid arthritis on posteroanterior hand or wrist radiographs, which must include erosions or unequivocal bony decalcification localised in, or most marked adjacent to, the involved joints (osteoarthritis changes alone do not qualify).
Note: For classification purposes, a patient has RA if at least four of these criteria are satisfied (the first four must have been present for at least six weeks).

Systemic Lupus Erythematosus

2019 ACR/EULAR classification criteria

The entry criterion is necessary to classify SLE.
ANA at a titer of ≥1:80 on HEp-2 cells or an equivalent positive test (ever).*
At least 1 clinical criterion required to classify SLE. Additional additive (clinical or immunology) criteria are counted toward the total score : ¶
Do not count a criterion if there is a more likely explanation than SLE.   Occurrence of a criterion on ≥1 occasion is sufficient.   Criteria need not occur simultaneously.   Within each domain (eg, mucocutaneous, complement proteins), only the highest-weighted criterion is counted toward the total score if more than 1 is present.
Clinical domains and criteria	Weight
Constitutional
Fever	2
Hematologic
Leukopenia	3
Thrombocytopenia	4
Autoimmune hemolysis	4
Neuropsychiatric
Delirium	2
Psychosis	3
Seizure	5
Mucocutaneous
Nonscarring alopecia	2
Oral ulcers	2
Subacute cutaneous or discoid lupus	4
Acute cutaneous lupus	6
Serosal
Pleural or pericardial effusion	5
Acute pericarditis	6
Musculoskeletal
Joint involvement	6
Renal
Proteinuria >0.5 g per 24 hours	4
Renal biopsy Class II or V lupus nephritis	8
Renal biopsy Class III or IV lupus nephritis	10
Immunology domains and criteria	Weight
Antiphospholipid antibodies
Anti-cardiolipin antibodies or anti-beta-2GP1 antibodies or lupus anticoagulant	2
Complement proteins
Low C3 or low C4	3
Low C3 and low C4	4
SLE-specific antibodies
Anti-dsDNA antibodyΔ or anti-Smith antibody	6
A total score of ≥10 and ≥1 clinical criterion are required to classify SLE.
* If ANA is absent, do not classify as SLE.
¶ Additional criteria within the same domain will not be counted.
Δ In an assay with 90% specificity against relevant disease controls.

1997 ACR & 2012 SLICC

1997 Updated of the 1982 ACR revised Criteria		2012 SLICC classification criteria
(4 of 11 criteria)*		(4 of 17 criteria, including at least 1 clinical criterion and 1 immunologic criterion;¶ OR biopsy-proven lupus nephritisΔ)
Criterion	Definition	Criterion	Definition
		Clinical criteria
Malar rash	Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds	Acute cutaneous lupus	Lupus malar rash (do not count if malar discoid); bullous lupus; toxic epidermal necrolysis variant of SLE; maculopapular lupus rash; photosensitive lupus rash (in the absence of dermatomyositis); OR subacute cutaneous lupus (nonindurated psoriaform and/or annular polycyclic lesions that resolve without scarring, although occasionally with postinflammatory dyspigmentation or telangiectasias)
Photosensitivity	Skin rash as a result of unusual reaction to sunlight, by patient history or clinician observation
Discoid rash	Erythematosus raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions	Chronic cutaneous lupus	Classic discoid rash; localized (above the neck); generalized (above and below the neck); hypertrophic (verrucous) lupus; lupus panniculitis (profundus); mucosal lupus; lupus erythematosus tumidus; chilblains lupus; OR discoid lupus/lichen planus overlap
		Nonscarring alopecia	Diffuse thinning or hair fragility with visible broken hairs (in the absence of other causes, such as alopecia areata, drugs, iron deficiency, and androgenic alopecia)
Oral ulcers	Oral or nasopharyngeal ulceration, usually painless, observed by a clinician	Oral or nasal ulcers	Palate, buccal, tongue, OR nasal ulcers (in the absence of other causes, such as vasculitis, Behçet syndrome, infection [herpesvirus], inflammatory bowel disease, reactive arthritis, and acidic foods)
Arthritis	Nonerosive arthritis involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion	Joint disease	Synovitis involving 2 or more joints, characterized by swelling or effusion OR
			Tenderness in 2 or more joints and at least 30 minutes of morning stiffness
Serositis	Pleuritis – Convincing history of pleuritic pain or rubbing heard by a clinician or evidence of pleural effusion OR	Serositis	Typical pleurisy for more than 1 day, pleural effusions, or pleural rub, OR
	Pericarditis – Documented by ECG, rub, or evidence of pericardial effusion		Typical pericardial pain (pain with recumbency improved by sitting forward) for more than 1 day, pericardial effusion, pericardial rub, or pericarditis by electrocardiography in the absence of other causes, such as infection, uremia, and Dressler syndrome
Renal disorder	Persistent proteinuria greater than 500 mg/24 hours or greater than 3+ if quantitation not performed OR	Renal	Urine protein-to-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hours, OR
	Cellular casts – May be red cell, hemoglobin, granular, tubular, or mixed		Red blood cell casts
Neurologic disorder	Seizures OR psychosis – In the absence of offending drugs or known metabolic derangements (uremia, ketoacidosis, or electrolyte imbalance)	Neurologic	Seizures; psychosis; mononeuritis multiplex (in the absence of other known causes, such as primary vasculitis); myelitis; peripheral or cranial neuropathy (in the absence of other known causes, such as primary vasculitis, infection, and diabetes mellitus); OR acute confusional state (in the absence of other causes, including toxic/metabolic, uremia, drugs)
Hematologic disorder	Hemolytic anemia – With reticulocytosis OR Leukopenia – Less than 4000/mm3 total on 2 or more occasions OR Lymphopenia – Less than 1500/mm3 on 2 or more occasions OR Thrombocytopenia – Less than 100,000/mm3 (in the absence of offending drugs)	Hemolytic anemia	Hemolytic anemia
		Leukopenia or lymphopenia	Leukopenia (<4000/mm3 at least once) (in the absence of other known causes, such as Felty syndrome, drugs, and portal hypertension), OR
			Lymphopenia (<1000/mm3 at least once) (in the absence of other known causes, such as glucocorticoids, drugs, and infection)
		Thrombocytopenia	Thrombocytopenia (<100,000/mm3) at least once in the absence of other known causes, such as drugs, portal hypertension, and thrombotic thrombocytopenic purpura
		Immunologic criteria
ANA	An abnormal titer of ANA by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with "drug-induced lupus" syndrome	ANA	ANA level above laboratory reference range
Immunologic disorders	Anti-DNA – Antibody to native DNA in abnormal titer OR Anti-Sm – Presence of antibody to Sm nuclear antigen OR Positive antiphospholipid antibody on: An abnormal serum level of IgG or IgM anticardiolipin antibodies OR A positive test result for lupus anticoagulant using a standard method OR A false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test	Anti-dsDNA	Anti-dsDNA antibody level above laboratory reference range (or >2-fold the reference range if tested by ELISA)
		Anti-Sm	Presence of antibody to Sm nuclear antigen
		Antiphospholipid	Antiphospholipid antibody positivity as determined by any of the following: Positive test result for lupus anticoagulant; false-positive test result for rapid plasma reagin; medium- or high-titer anticardiolipin antibody level (IgA, IgG, or IgM); or positive test result for anti-beta 2-glycoprotein I (IgA, IgG, or IgM)
		Low complement	Low C3; low C4; OR low CH50
		Direct Coombs test	Direct Coombs test in the absence of hemolytic anemia
* For the ACR criteria, no distinction is made between clinical and immunologic criteria in determining whether the required number has been met. The classification is based upon 11 criteria. For the purpose of identifying patients in clinical studies, a person is said to have SLE if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation.
¶ For the SLICC criteria, criteria are cumulative and need not be presently concurrently. A patient is classified as having SLE if he or she satisfies 4 of the clinical and immunologic criteria used in the SLICC classification criteria, including at least 1 clinical criterion and 1 immunologic criterion.
Δ Alternatively, according to the SLICC criteria, a patient is classified as having SLE if he or she has biopsy-proven nephritis compatible with SLE in the presence of ANAs or anti-dsDNA antibodies.

Sjogren syndrome

2016 ACR/EULAR classification criteria for primary Sjogren syndrome

The classification of primary SS applies to any individual who meets the inclusion criteria§, does not have any of the conditions listed as exclusion criteria¥, and has a score ≥4 when the weights from the five criteria items are summed.
Item	Weight/score
Labial salivary gland with focal lymphocytic sialadenitis and focus score of ≥1 foci/4 mm2*	3
Anti-Ro/SSA positive	3
Ocular staining score ≥5 (or van Bijsterveld score ≥4) in at least one eye¶Δ	1
Schirmer test ≤5 mm/5 minutes in at least one eye¶	1
Unstimulated whole saliva flow rate ≤0.1 mL/minute¶◊	1
* Labial salivary gland with focal lymphocytic sialadenitis and focus score ≥1 foci/4 mm2. The histopathologic examination should be performed by a pathologist with expertise in the diagnosis of focal lymphocytic sialadenitis and focus score count, following a protocol described by Daniels, et al[1].
¶ Patients who are normally taking anticholinergic drugs should be evaluated for objective signs of salivary hypofunction and ocular dryness after a sufficient interval without these medications in order for these components to be a valid measure of oral and ocular dryness.
Δ Ocular staining score described by Whitcher, et al[2]; van Bijsterveld score described by van Bijsterveld[3].
◊ Unstimulated whole saliva flow rate measurement described by Navazesh and Kumar[4].
§ Inclusion criteria: These criteria are applicable to any patient with at least one symptom of ocular or oral dryness, defined as a positive response to at least one of the following questions:   (1) Have you had daily, persistent, troublesome dry eyes for more than three months?;   (2) Do you have a recurrent sensation of sand or gravel in the eyes?;   (3) Do you use tear substitutes more than three times a day?;   (4) Have you had a daily feeling of dry mouth for more than three months?;   (5) Do you frequently drink liquids to aid in swallowing dry food?        or in whom there is suspicion of SS from the EULAR SS Disease Activity Index questionnaire (at least one domain with positive item).
¥ Prior diagnosis of any of the following conditions would exclude diagnosis of SS and participation in SS studies or therapeutic trials because of overlapping clinical features or interference with criteria tests:   History of head and neck radiation treatment   Active hepatitis C infection (with positive PCR)   Acquired immunodeficiency syndrome   Sarcoidosis   Amyloidosis   Graft-versus-host disease   IgG4-related disease

2012 ACR proposed Classification criteria for Sjogren syndrome

The classification of Sjögren syndrome (SS), which applies to individuals with signs or symptoms that may be suggestive of SS, will be met in patients who have at least two of the following three objective features
1. Positive serum anti-SSA/Ro and/or anti-SSB/La or (positive RF 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 past 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 immunodeficiency syndrome
Sarcoidosis
Amyloidosis
Graft-versus-host disease
IgG4-related disease
*We excluded participants with rheumatoid arthritis, systemic lupus erythematosus, scleroderma, and other connective tissue disease from these analyses because there were only 87 (6%) such participants.
†Using histopathologic definitions and focus score assessment methods as described by Daniels et al (1).
‡Using ocular staining score as described by Whitcher et al (2).

Systemic Sclerosis

2013 ACR/EULAR classification criteria

The ACR/EULAR criteria for the classification of SSc*
Item (Category)	Sub-item(s)	Weight/score¶
Skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints (sufficient criterion)	–	9
Skin thickening of the fingers (only count the higher score)	Puffy fingers	2
	Sclerodactyly of the fingers (distal to the metacarpophalangeal joints but proximal to the proximal interphalangeal joints)	4
Fingertip lesions (only count the higher score)	Digital tip ulcers	2
	Fingertip pitting scars	3
Telangiectasia	–	2
Abnormal nailfold capillaries	–	2
Pulmonary arterial hypertension and/or interstitial lung disease (maximum score is 2)	Pulmonary arterial hypertension	2
	Interstitial lung disease	2
Raynaud phenomenon	–	3
SSc-related autoantibodies (anticentromere, anti-topoisomerase I [anti-Scl-70], anti-RNA polymerase III) (maximum score is 3)	Anticentromere Anti-topoisomerase I Anti-RNA polymerase III	3
* These criteria are applicable to any patient considered for inclusion in an SSc study. The criteria are not applicable to patients with skin thickening sparing the fingers or to patients who have a scleroderma-like disorder that better explains their manifestations (eg, nephrogenic sclerosing fibrosis, generalized morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, erythromyalgia, porphyria, lichen sclerosis, graft-versus-host disease, diabetic cheiroarthropathy).
¶ The total score is determined by adding the maximum weight (score) in each category. Patients with a total score of ≥9 are classified as having definite SSc.
Definitions of items in the ACR/EULAR criteria for the classification of SSc
Item	Definition
Skin thickening	Skin thickening or hardening not due to scarring after injury, trauma, etc.
Puffy fingers	Swollen digits—a diffuse, usually nonpitting increase in soft tissue mass of the digits extending beyond the normal confines of the joint capsule. Normal digits are tapered distally with the tissues following the contours of the digital bone and joint structures. Swelling of the digits obliterates these contours. Not due to other causes such as inflammatory dactylitis.
Fingertip ulcers or pitting scars	Ulcers or scars distal to or at the proximal interphalangeal joint not thought to be due to trauma. Digital pitting scars are depressed areas at digital tips as a result of ischemia, rather than trauma or exogenous causes.
Telangiectasia	Telangiectasiae are visible macular dilated superficial blood vessels, which collapse upon pressure and fill slowly when pressure is released. Telangiectasiae in a scleroderma-like pattern are round and well demarcated and found on hands, lips, and inside of the mouth, and/or are large mat-like telangiectasiae. Distinguishable from rapidly filling spider angiomas with central arteriole and from dilated superficial vessels.
Abnormal nailfold capillary pattern consistent with systemic sclerosis	Enlarged capillaries and/or capillary loss with or without pericapillary hemorrhages at the nailfold. May also be seen on the cuticle.
Pulmonary arterial hypertension	Pulmonary arterial hypertension diagnosed by right-sided heart catheterization according to standard definitions.
Interstitial lung disease	Pulmonary fibrosis seen on high-resolution computed tomography or chest radiography, most pronounced in the basilar portions of the lungs, or occurrence of "Velcro" crackles on auscultation, not due to another cause such as congestive heart failure.
Raynaud's phenomenon	Self-reported or reported by a physician, with at least a two-phase color change in finger(s) and often toe(s) consisting of pallor, cyanosis, and/or reactive hyperemia in response to cold exposure or emotion; usually one phase is pallor.
SSc-related autoantibodies	Anticentromere antibody or centromere pattern seen on antinuclear antibody testing, anti-topoisomerase I antibody (also known as anti-Scl-70 antibody), or anti-RNA polymerase III antibody. Positive according to local laboratory standards.

1980 ACR

Idiopathic Inflammatory Myositis

Bohan and Peter (1975)

Criteria	Definition
A	Proximal and symmetrical muscle weakenss of the pelvic and scapular girdle, anterior flexors of the neck, progressing for weeks to months, with or without dysphagia or involvement of respiratory muscles
B	Elevation of the serum levels of skeletal muscle enzymes: creatine kinase, aspartate aminotrasnferase, lactate dehydrogenase and aldolase
C	Electromyography characteristic of myopathy (short and small motor units, fibrillation, positive pointy waves, insertional irritability and repetitive high-frequency firing)
D	Muscle biopsy showing necrosis, phagocytosis, regeneration, perifascicular atrophy, perivascular inflamnmatory exudate
E	Typical cutaneous changes: [1] Heliotrope rash with periorbital oedema and violaceous erythema [2] Gottron's sigh : vasculitis in the elbow, metacarpophalangeal and proximal interphalangeal joints
Polymyositis	[1] Definite - all of A-D [2] Probable - any three of A-D [3] Possible - any two of A-D
Dermatomyositis	[1] Definite - E plus three of A-D [2] Probable - E plus two of A-D [3] Possible - E plus one of A-D

2017 ACR

When no better explanation for the symptoms and signs exists, these classification criteria can be used
	score points
Variable	without muscle biopsy	with muscle biopsy	Definition
Age of onset
Age of onset of first symptom assumed to be related to the disease ≥18 years and <40 years	1.3	1.5	18 ≤ age (years) at onset of first  symptom assumed to be related to the disease <40
Age of onset of first symptom assumed to be related to the disease ≥40 years	2.1	2.2	Age (years) at onset of first symptom assumed to be related to the disease ≥40
Muscle weakness
Objective symmetric weakness, usually progressive, of the proximal upper extremities	0.7	0.7	Weakness of proximal upper extremities as defined by manual muscle testing or other objective strength testing, which is present on both sides and is usually progressive over time
Objective symmetric weakness, usually progressive, of the proximal lower extremities	0.8	0.5	Weakness of proximal lower extremities as defined by manual muscle testing or other objective strength testing, which is present on both sides and is usually progressive over time
Neck flexors are relatively weaker than neck extensors	1.9	1.6	Muscle grades for neck flexors are relatively lower than neck extensors as defined by manual muscle testing or other objective strength testing
In the legs, proximal muscles are relatively weaker than distal muscles	0.9	1.2	Muscle grades for proximal muscles in the legs are relatively lower than distal muscles in the legs as defined by manual muscle testing or other objective strength testing
Skin manifestations
Heliotrope rash	3.1	3.2	Purple, lilac-colored, or erythematous patches over the eyelids or in a periorbital distribution, often associated with periorbital edema
Gottron’s papules	2.1	2.7	Erythematous to violaceous papules over the extensor surfaces of joints, which are sometimes scaly. May occur over the finger joints, elbows, knees, malleoli, and toes
Gottron’s sign	3.3	3.7	Erythematous to violaceous macules over the extensor surfaces of joints, which are not palpable
Other clinical manifestations
Dysphagia or esophageal dysmotility	0.7	0.6	Difficulty in swallowing or objective evidence of abnormal motility of the esophagus
Laboratory measurements
Anti–Jo-1 (anti–histidyl–transfer RNA synthetase) autoantibody present	3.9	3.8	Autoantibody testing in serum performed with standardized and validated test, showing positive result
Elevated serum levels of creatine kinase (CK)* or lactate dehydrogenase (LDH)* or aspartate aminotransferase (ASAT/AST/SGOT)* or alanine aminotransferase (ALAT/ALT/SGPT)*	1.3	1.4	The most abnormal test values during the disease course (highest absolute level of enzyme) above the relevant upper limit of normal
Muscle biopsy features—presence of:
Endomysial infiltration of mononuclear cells surrounding, but not invading, myofibers		1.7	Muscle biopsy reveals endomysial mononuclear cells abutting the sarcolemma of otherwise healthy, non-necrotic muscle fibers, but there is no clear invasion of the muscle fibers
Perimysial and/or perivascular infiltration of mononuclear cells		1.2	Mononuclear cells are located in the perimysium and/or located around blood vessels (in either perimysial or endomysial vessels)
Perifascicular atrophy		1.9	Muscle biopsy reveals several rows of muscle fibers, which are smaller in the perifascicular region than fibers more centrally located
Rimmed vacuoles		3.1	Rimmed vacuoles are bluish by hematoxylin and eosin staining and reddish by modified Gomori trichrome stain
* Serum levels above the upper limit of normal

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1. Mahroum N, Mahagna H, Amital H. Diagnosis and classification of adult Still’s disease. J Autoimmun. 2014;48-49:34-7. ↩

2. Cush JJ, Medsger Jr TA, Christy WC, et al. Adult-onset Still’s disease: clinical course and outcome. Arthritis Rheum. 1987;30:186e94. ↩

3. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19:424e30 ↩

4. Fautrel B, Zing E, Golmard JL, et al. Proposal for a new set of classification criteria for adult-onset still disease. Medicine (Baltimore). 2002;81:194e200 ↩

5. Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295-306. ↩

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Last update: January 4, 2022