風濕過敏免疫科 疾病診斷標準

Diagnostic Criteria

林口長庚醫院 風濕過敏免疫科 余光輝 醫師

 The 1997 Revised Criteria for Classification of SLE
SYSTEMIC LUPUS ERYTHEMATOSUS (2-1)
Criterion Definition

1. Malar rash Fixed erythema,flat or raised, over the malar eminences, tending to spare the nasolabial folds.
2. Discoid rash Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic
scarring may occur in older lesions.

3. Photosensitivity Skin rash as a result of unusual reaction to sunlight , by patient history or physician observation.

4. Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by a physician.

5. Arthritis Nonerosive arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion.

6. Serositis (a) Pleuritis － convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion OR
(b) Pericarditis －documented by ECG or rub or evidence of pericardial effusion.

7. Renal disorder (a) Persistent proteinuria greater than 0.5gm per day or greater than 3+ if quantitation not performed OR

(b) Cellular casts－may be red cell, hemoglobulin, granular, tubular, or mixed.

8. Neurologic disorder (a) Seizures－in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis,
or electrolyte imbalance OR

(b) Psychosis－in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis,
or electrolyte imbalance.

9. Hematologic disorder (a) Hemolytic anemia－with reticulocytosis OR

(b) Leukopenia－less than 4000/mm3 total on two or more occasions

(c) Lymphopenia－less than 1500/mm3 on two or more occasions

(d) Thrombocytopenia－less than 100,000/mm3 in the absence of offending drugs.

THE 1997 REVISED CRITERIA FOR CLASSIFICATION OF
SYSTEMIC LUPUS ERYTHEMATOSUS (2-2)
Criterion Definition

10. Immunologic disorder (a) Anti – DNA : antibody to native DNA in abnormal titre; or

(b) Anti – Sm : presence of antibody to Sm nuclear antigen; or

Š Modified 1997
(c) Positive finding of antiphospholipid antibodies based on : (1) an abnormal serum level of IgG or IgM
anticardiolipin antibodies; (2) a positive test result for lupus anticoagulant using a standard method; or
(3) a false-positive serological test for syphilis known to be positive for at least 6 months and
confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test

11. Antinuclear antibody An abnormal titer of antinuclear antibody 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.”

*The proposed classification is based on 11 criteria. For the purpose of identifying patients in clinical studies, a person shall be said to have
systemic lupus erythematosus if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation.
(Arthritis and Rheumatism 25:1271, 1982. The American Rheumatism Association.)
Š Hochberg MC for the Diagnostic and Therapeutic Criteria Committee for the American College of Rheumatology. Updating the American
College of Rheumatology revised criteria for systemic lupus erythematosus [Letter]. Arthritis Rheum 1997;40:1725.
 DRUG-INDUCED LUPUS

Clinical Features Spontaneous Lupus(%) Drug-Induced Lupus(%)

Age 20-40 50
Sex(F:M) 9 Equal
Race All “No blacks”
Acetylation type S＝F Slow
Onset of symptoms Gradual Abrupt
Constitutional symptoms(fever, malaise, myalgia) 83 50
Arthralgia and arthritis 90 95
Pleuropericarditis 50 50(procainamide)
Hepatomegaly 25 25
Skin rash (all types) 74 10-20
Discoid lesions 20 0
Malar erythema 42 2
Renal disease 53 5
CNS disease 32 0
Hematologic disease Common Unusual
Immune abnormalities
ANA 95 95
LE cells 90 90
Anti-RNP 40-50 20
Anti-Sm 20-30 Rare
Anti-DNA 80 Rare
Antihistone 25 90
Complement Reduced Normal
Immune complexes Elevated Normal
 SUGGESTED CRITERIA FOR THE ANTIPHOSPHOLIPID SYNDROME

Clinical Laboratory
Lupus anticoagulant
Venous thrombosis
IgG anticardiolipin ( > 20 GPL units)
Arterial thrombosis
IgM anticardiolipin ( > 20 MPL units)
Pregnancy loss (unexplained)
Thrombocytopenia

Patients should have at least one clinical and one laboratory feature to make the
diagnosis. The laboratory test should be positive on at least two occasions, 8 weeks apart.
Antibodies Found in SLE and Their Association
Antibody Clinical Association
Anti-ssDNA Nonspecific
Anti-dsDNA Renal disease
Antihistone Renal disease, drug-related lupus
Anti-Sm Renal and central nervous system diseease
Anti-RNP Mild disease, infrequent renal involvement
Anti-Ro Subacute cutaneous lupus erythematosus; photosensitive skin
disease; keratoconjunctivitis sicca; renal diseasee; lymphopenia;
interstitial pneumonitis; neonatal lupus; homozygous C-2 and C-4
deficiencies
Anti-La Keratoconjunctivitis sicca, infrequent renal disease
Anti-Ku Overlap with systemic sclerosis and polymyositis
Anti-Ma Recalcitrant skin disease; renal disease; neurologic involvement;
hepatosplenomegaly; lymphadenopathy
* ssDNA= single-stranded DNA; dsDNA= double-stranded DNA
 Antibodies, Antigens, and Diseases*
Antibody Disease Antigen
Anti-ssDNA Nonspecific Purine- and pyrimidine- related antigens
Anti-dsDNA SLE Deoxyribose phosphate backbone of DNA
Antihistone SLE, DRL, rheumatoid arthritis H1, H2A, H2B, H3, H4, H2A-H2B
Anti-Sm SLE Proteins complexed with U1-U6 RNAs (splicesome)
Anti-RNP MCTD, SLE Proteins complexed with U1a, U1b (splicesome)
Anti-Ro/SS-A SLE, Sjogren’s Proteins complexed with hY, hY3, hY4, hY5
Anti-La/SS-B SLE, Sjogren’s Phosphorylated protein complexed with small RNA
polymerase Ⅲ transcripts
Anti-Scl-70 Systemic sclerosis DNA Topoisomerase Ⅰ
Anti-centromere CREST Proteins in kinetochore
Anti-RNA polymerase Ⅰ Diffuse systemic sclerosis RNA polymerase Ⅰ
Antifibrillarin Systemic sclerosis Nucleolar protein with U3-RNP complex
Anti-PM-Scl Polymyositis, systemic sclerosis, and overlap Not known
Anti-Jo-1 Polymyositis and dermatomyositis Histidyl-tRNA synthetase
Anti-PL-7 Polymyositis and dermatomyositis Threonyl-tRNA synthetase
Anti-PL-12 Polymyositis and dermatomyositis Naked alanyl-tRNA and alanyl-tRNA synthetase
Anti-Ku Systemic sclerosis and polymyositis, overlap. SLE overlap Proteins binding to DNA
Anti-Mi-1 Dematomyositis and SLE Nucleolar protein
Anti-Mi-2 Dermatomyosiis Not known
Anti-PCNA SLE Auxiliary protein of DNA polymerase delta
Anti-RANA Rheumatoid arthritis Possibly EBNA-1
Anti-Ma SLE Nuclear acidic protein

* ssDNA = single-stranded DNA; dsDNA= double-stranded DNA; DRL= drug-related lupus; tRNA= transfer RNA; PCNA= proliferating cell
nuclear antigen; RANA= rheumatoid arthritis-associated nuclear antigen; EBNA-1= Epstein-Barr nuclear antigen.
Antibody Clinical Manifestation
Antinuclear antibody Most sensitive test for systemic lupus erythematosus; detects 95% or
more of cases; poor specificity; positive in many other diseases; other
autoimmune states, chronic inflammatory processes, tumors, aging
Anti-double-stranded DNA Relatively specific for systemic lupus erythematosus; positive in 50% to
75% of cases; associated with DR2; high titers suggest increased risk for
nephritis; high titers often correlate with disease activity
Anti-single-stranded DNA No sensitivity or specificity; occurs in many acute and chronic illnesses
Anti-Ro Occurs in systemic lupus erythematosus and Sjogren syndrome;
associated with DR3; frequent in antinuclear antibody-negative systemic
lupus erythematosus; may cause neonatal lupus risk for congenital heart
block; frequent in subacute cutaneous lupus
Anti-Sm Relatively specific for systemic lupus erythematosus; titers do not
fluctuate with disease activity
Antiribonucleoprotein Occurs in many autoimmune diseases; high titers common in patients
(Anti-RNP) with an overlap among systemic lupus erythematosus, rheumatoid
arthritis, polymyositis and scleroderma; occurs in mixed connective
tissue disease
Patients with systemic lupus erythematosus with only
antiribonucleoproteins are unlikely to develop nephritis or central nervous
disease
Anticardiolipin Present in 50% of systemic lupus erythematosus and in other
autoimmune diseases and some healthy persons; associated with
thrombosis and fetal loss.
Lupus anticoagulant Direct against phospholipids clotting casecade; associated with
thrombosis and fetal loss, merely associated with bleeding diathesis
Antihistone Associated with drug-induced lupus; also common in spontaneous
systemic lupus erythematosus
 1988 ARA criteria for classification of
RHEUMATOID ARTHRITIS
Criteria Definition

1. Morning stiffness Morning stiffness in and around the joints lasting at least 1 hour before maximal
improvement
2. Arthritis of three or more joint areas At least three joint areas have simultaneously had soft tissue swelling or fluid
(not bony overgrowth alone) observed by a physician. The 14 possible joint
areas are (right or left): PIP, MCP, wrist, elbow, knee, ankle, and MTP joints
3. Arthritis of hand joints At least one joint area swollen as above in wrist, MCP, or PIP joint
4. Symmetric arthritis Simultaneous involvement of the same joint areas (as in 2) on both sides of the
body (bilateral involvement of PIP, MCP, or MTP joints is acceptable without
absolute symmetry)
5. Rheumatoid nodules Subcutaneous nodules, over bony prominences, or extensor surfaces, or in
juxta-articular regions, observed by a physician
6. Serum rheumatoid factor Demonstration of abnormal amounts of serum “rheumatoid factor” by any
method that has been positive in less than 5 percent of normal control subjects
7. Radiographic changes Radiographic changes typical of RA on PA hand and wrist x-rays, which must
include erosions or unequivocal bony decalcification localized to or most marked
adjacent to the involved joints (osteoarthritis changes alone do not qualify)

For classification purposes, a patient is said to have RA if he or she has satisfied at least 4 of the above 7 criteria. Criteria 1
through 4 must be present for at least 6 weeks. Patients with two clinical diagnoses are not excluded. Designation as classic,
definite, or probable rheumatoid arthritis is not to be made.
 Criteria for Determination of Progression of
Rheumatoid Arthritis and of Functional Capacity
of Patients with the Disease
Classification of Progression of Rheumatoid Arthritis

Stage Ⅰ, Early Stage Ⅲ, Severe

*1. No destructive changes on roentgenographic *1. Roentgenologic evidence of cartilage and bone
examination destruction, in addition to osteoporosis
2. Roentgenologic evidence of osteoporosis, may *2. Joint deformity, such as subluxation, ulnar
be present. deviation, or hyperextension, without fibrous or
Stage Ⅱ, Moderate bony ankylosis.

*1. Roentgenologic evidence of osteoporosis, with 3. Extensive muscle atrophy

or without slight subchondral bone destruction; 4. Extraarticular soft tissue lesions, such as nodules
slight cartilage destruction may be present. and tenosynovitis may be present.
*2. No joint deformities, although limitation of joint Stage Ⅳ, Terminal
mobility may be present. *1. Fibrous or bony ankylosis
3. Adjacent muscle atrophy 2. Criteria of stage Ⅲ
4. Extraarticular soft tissue lesions, such as
nodules and tenosynovitis may be present.
* The criteria prefaced by an asterisk are those that must be present to permit classification of a patient in
any particular stage or grade.
Classification of Functional Capacity in
Rheumatoid Arthritis
Class Ⅰ: Complete functional capacity with ability to carry on all usual
duties without handicaps

Class Ⅱ: Functional capacity adequate to conduct normal activities

despite handicap of discomfort or limited mobility of 1 or
more joints
Class Ⅲ: Functional capacity adequate to perform only few or none of
the duties of usual occupation or of self care

Class Ⅳ: Largely or wholly incapacitated with patient bedridden or

confined to wheelchair, permitting little or no self care
Steinbrocker O. Traeger CH, Batterman RC. Therapeutic criteria in rheumatoid arthritis.
JAMA 140:659-662,1949

Diagnosis: Rheumatoid arthritis, stage II, functional class III

 Criteria for the Classification of Sjögren’s Syndrome*
1. Ocular symptoms
Definition. A positive response to at least one of the following three questions;
(a) Have you had daily, persistent, troublesome dry eyes for more than 3 months?
(b) Do you have a recurrent sensation of sand or gravel in the eyes?
(c) Do you use tear substitutes more than three times a day?
2. Oral symptoms
Definition. A positive response to at least one of the following three questions:
(a) Have you had a daily feeling of dry mouth for more than 3 months?
(b) Have you had recurrent or persistently swollen salivary glands as an adult?
(c) Do you frequently drink liquids to aid in swallowing dry foods?
3. Ocular signs
Definition. Objective evidence of ocular involvement, determined on the basis of a positive result on at least one of the following two tests:
(a) Schirmer-I test (≦5 mm in 5 minutes)
(b) Rose bengal score (≧4, according to the van Bijsterveld scoring system)
4. Histopathologic features
Definition. Focus score ≧1 on minor salivary gland biopsy (focus defined as an agglomeration of at least 50 mononuclear cells; focus score defined
as the number of foci per 4 min2 of glandular tissue)
5. Salivary gland involvement
Definition. Objective evidence of salivary gland involvement, determined on the basis of a positive result on at least one of the following three tests:
(a) Salivary scintigraphy
(b) Parotid sialography
(c) Unstimulated salivary flow (≦1.5 ml in 15 minutes)
6. Autoantibodies
Definition. Presence of at least one of the following serum autoantibodies:
(a) Antibodies to Ro/SS-A or La/SS-B antigens
(b) Antinuclear antibodies
(c) Rheumatoid factor
Exclusion criteria: preexisting lymphoma, acquired immunodeficiency syndrome, sarcoidosis, or graft-versus-host disease
* For primary Sjögren’s syndrome, the presence of three of six items showed a very high sensitivity (99.1%), but insufficient specificity (57.8%). Thus, this
combination could be accepted as the basis for a diagnosis of probable primary Sjögren’s syndrome. However, the presence of four of six items (accepting as
serologic parameters only positive anti-Ro/SS-A and anti La/SS-B antibodies ) had a good sensitivity (93.5%) and specificity (94.0%), and therefore may be
used to establish a definitive diagnosis of primary Sjögren’s syndrome.
Vitali C, Bombardieri S, Moutsopoulos HM, et al: Preliminary criteria for the classification of Sjögren’s syndrome. Arthritis Rheum 36:340-347, 1993
 Preliminary Criteria for Classification of
Progressive Systemic Sclerosis (Scleroderma)
A. Major criteria
Proximal scleroderma: Symmetric thicken-ing,
tightening, and induratioon of the skin of the
fingers and the skin proximal to the metacarpo-
phalangeal or metatarsophalangeal joints. The
changes may affect the entire extremity, face,
neck, and trunk (thorax and abdomen).
B. Minor criteria
1. Distal sclerodactyly: Above-indicated skin
changes limited to the fingers
2. Digital pitting scars or loss of substance
from the finger pad: Depressed areas at tips of
fingers or loss of digital pad tissue as a result of
ischemia
3. Bibasilar pulmonary fibrosis: Bilateral
reticular pattern of linear or lineonodular densities
most pronounced in basilar portions of the lungs
on standard chest roentgenogram; may assume
appearance of diffuse mottling or “honeycomb
lung.” These changes should not be attributable
to primary lung disease.
For the purposes of classifying patients in
clinical trials, population surveys, and
other studies, a person shall be said to
have progressive systemic sclerosis
(scleroderma), if the one major or 2 or
more minor criteria listed baove are
present. Localized forms of scleroderma,
eosinophilic fasciitis, and the various
forms of pseudoscleroderma are excluded
from these criteria.
Masi AT. Rodnan GP. Medsger TA Jr.
Altman RD, D’ Angelo WA. Fries JF.
LeRoy EC. Kirsner AB. Mackenzie AH.
McShane DJ. Myers AR. Sharp GC:
Preliminary criteria for the classification of
systemic sclerosis (scleroderma). Arthritis
Rheum 23:581-590, 1980
 Classification of Scleroderma
Ⅰ. SYSTEMIC SCLEROSIS (Progressive Systemic Sclerosis; Systemic Scleroderma)

with diffuse scleroderma: symmetrical widespread (diffuse) skin involvement. Affecting

the trunk, face, and proximal as well as distal extremities: tendency to rapid progression
and early appearance of visceral involvement.
with CREST syndrome: relatively limited (restricted) skin involvement, often confined to the
fingers and face: prolonged delay in appearance of distinctive internal manifestations, e.g.,
pulmonary arterial hypertension and biliary cirrhosis; prominence of calcinosis and
telangiectases.
with “overlap”: having typical features of one or more members of the connective tissue
disease family.

Ⅱ. LOCALIZED SCLERODERMA
Morphea: single or multiple (generalized) plaques.
Linear scleroderma: with or without melorheostosis; includes scleroderma en coup de sabre
(with or without facial hemiatrophy).

Ⅲ. EOSINOPHILIC FASCIITIS
 Criteria for Polymyositis & Dermatomyositis

1. Typical skin rash of dermatomyositis

2. Symmetrical proximal muscle weakness
3. Elevation of one or more serum muscle enzymes
4. Myopathic changes on electromyogram
5. Typical polymyositis on muscle biopsy

Bohan and Peter’s Criteria

Dermatomyositis Polymyositis
Definite (1) + any 3 of (2),(3),(4) or (5) All 4 of (2),(3),(4) and (5)
Probable (1) + any 2 of (2),(3),(4) or (5) Any 3 of (2),(3),(4) or (5)

Possible (1) + any 1 of (2),(3),(4) or (5) Any 2 of (2),(3),(4) or (5)

 Dermatologic features in Dermatomyositis

1. heliotrope sign: a lilac discoloration over the eyelids with periorbital

edema.
2. Gottron’s sign: the most distinctive and characteristic eruption is a
scaly erythematous dermatitis overlying the dorsum of the hands
(especilaay the metacarpophalangeal and proximal interphalangeal
joints).

3. This rash also may appear on the knees, elbows, medial malleoli, face,
neck, and upper anterior torso (shawl sign).

4. Hyperemia of the base, sides, and ends of the nails and fingers is also
commonly seen. Less frequently, sclerodermatous-like thickening of
the hands and arms may occur with telangiectasia and pigmentary
changes.
 Undifferentiated, Overlapping and Mixed
Connective Tissue Disorder
The term undifferentiated is usually employed when there are signs and symptoms
suggestive of a connective tissue disease, but the clinical picture is not distinctive
enough to permit a precise diagnosis. The usual implication is that it is too early in the
course of the disease to make a diagnosis and the passage of time will allow one to be
made later.

However, an “overlap” implies that more than one distinctive connective tissue disease
may be diagnosed in one patient either at one point in time or sequentially. The usual
implication of this diagnosis is that the patient has met the established or formal criteria
for two or more different connective tissue diseases; however, the coexistence of one of
these diseases and Sjogren’s syndrome is usually not designated an overlap. An
overlapping syndrome is suggested when a patient with one of the distinctive connective
tissue diseases begins to exhibit features considered characteristic of another.

Mixed connective tissue disease is regarded as a distinctive entity by some authorities

because of its association with an anti-nuclear antibody - anti-nuclear ribonucleoprotein.
Other authorities consider it to be a variant of systemic lupus erythematosus or a
nonspecific overlap. It is usually defined as an undifferentiated or overlapping connective
tissue disorder accompanied by high titers of circulating ribonucleoprotein antibodies
and none of the other anti-nuclear antibodies considered to be characteristic of systemic
lupus erythematosus.
 GUIDELINES FOR DIAGNOSING
Mixed Connective Tissue Disease

General

Clinical features of a diffuse connective tissue disorder

Serologic

1. Positive ANA, speckled pattern, titer>1:1000

2. Antibodies to U1 RNP
3. Absence of antibodies to dsDNA, histones, Sm, Scl70, PMScl, Ro, La, and other
specificities
4. Commonly: Hypergammaglobulinemia and positive rheumatoid factor

Clinical

1. Sequential evolution of overlap features over course of several years, including Raynaud’s
phenomenon, serositis, gastrointestinal dysmotility, myositis, arthritis, sclerodactyly, skin
rashes, and an abnormal DLCO on pulmonary function tests
2. Absence of truncal scleroderma, severe renal disease, and severe CNS involvement
3. A nail fold capillary pattern identical to that seen in PSS (dropout and dilated vessels)
Classification of the Vasculitis Syndromes
Systemic necrotizing vasculitis
Classic polyarteritis nodosa
Allergic angiitis and granulomatosis of Churg-Strauss
Polyangiitis overlap syndrome
Hypersensitivity vasculitis
Exogenous stimuli proved or suspected
Henoch-Schonlein purpura
Serum sickness and serum sickness-like reactions
Other drug-induced vasculitis
Vasculitis associated with infectious diseases
Endogenous antigens likely involved
Vasculitis associated with neoplasms
Vasculitis associated with connective tissue diseases
Vasculitis associated with other underlying diseases
Vasculitis associated with congenital deficiencies of the complement system
Wegener’s granulomatosis
Giant cell arteritis
Temporal arteritis
Takayasu’s arteritis
Miscellaneous vasculitic syndromes
Mucocutaneous lymph node syndrome (Kawasaki’s disease)
Isolated central nervous system vasculitis
Thromboangiitis obliterans (Buerger’s disease)
Behcet’s syndrome, Cogan’s syndrome, Erythema elevatum diutinum
 Clinical Characteristics of Major Vasculitic Syndromes*
SYNDROME COMMENT (2-1)

PAN group of SNV

Classic PAN Necrotizing vasculitis of small and medium-sized muscular arteries, involvement of renal and visceral arteries with
sparing of pulmonary circulation, associated with hepatitis B antigenemia in 30%. Multisystem disease common,
with kidney, muscle, peripheral nerves, bowel, liver, skin involvement frequent. Visceral and renal artery
aneurysms common.

Allergic angiitis and Similar to classic PAN regarding multisystem involvement; unique syndrome due to frequent involvement of
granulomatosis (Churg- pulmonary vessels, involvement of vessels of various types and sizes (small and mdeium-sized muscular arteries,
Strauss variant) veins, arterioles, and vessels), intravascular and extravascular granuloma formation, eosinophilic tissue infiltrates,
and association with severe asthma and eosinophilia.

Polyangiitis “overlap Shares clinical and pathologic features of classic PAN, allergic angiitis and granulomatosis, and small-vessel
syndrome” hypersensitivity vasculitis, bue does not fit precisely into any of these categories. Multiple organ systems involved,
with vasculitis, but does not fit precisely into any of these categories. Multiple organ systems involved, with
vasculitis involving vessels of any size and type. Visceral and renal vessel aneurysms may or may not be present.

HSP Includes heterogeneous group of clinical syndromes: characterized by inflammation of small vessels such as
arterioles, capillaries, and most commonly, venules. In contrast, classic PAN involves small and intermediated-
sized muscular arteries. HSP may affect any organ system, but skin involvement (manifested by palpable purpura)
dominates. While PAN group of SNV is life threatening, HSP only rarely is. HSP can be associated with infections
(bacterial, viral , parasitic, and mycobacterial), foreign proteins, chemicals, and drugs, and endogenous
substances such as DNA and tumor antigens.

Wegener’s granulomatosis A syndrome of unknown cause, with necrotizing granulomatous vasculitis of upper and lower respiratory tracts,
glomerulonephritis, and varying degrees of disseminated small vessel vasculitis.

Lymphomatoid granulomatosis
Granulomatous multisystem vasculitis involving primarily lungs, CNS, skin and kidneys with angiocentric and
angiodestructive infiltration with atypical lymphocytoid and plasmacytoid cells. Renal disease is not
glomerulonephritis, but rather lymphoid interstitial infiltrate. Not immune complex-mediated vasculitis, but rather
premalignant lymphoproliferative disease.
.
 Clinical Characteristics of Major Vasculitic Syndromes*
SYNDROME COMMENT (2-2)

Giant cell arteritides

Temporal arteritis Systemic panarteritis in elderly patients. May involve any medium or large-sized artery. Primary symptoms from
vasculitis of branches of carotid artery. Vasculitis of retinal arteries (ischemic optic neuropathy) can lead to
blindness. Associated polymyalgia rheumatica syndrome in at least 50% of cases

Takayasu’s arteritis Inflammation and stenosis of large and intermediate-sized arteries, with frequent involvement of the aortic arch
and branches. Cause unknown; primarily affects young women.

Behcet’s disease Recurrent oral ulcers, uveitis, genital ulcers and cutaneous lesions. Pathology is vasculitis of small vessels,
predominantly venules.

Vasculitis associated with PAN syndrome associated with hairy cell leukemia; granulomatous angiitis of CNS associated with Hodgkin’s
malignancies disease; hypersensitivity vasculitis, usually associated with lymphoid malignancies.

Miscellaneous

Cogan’s syndrome Syndrome, predominantly in young women, of acute- onset interstitial keratitis and vestibuloauditory dysfunction.
Associated with aortitis and Takayasu’s-like arteritis syndrome in 10%.

Mucocutaneous lymph node
syndrome (Kawasaki’s
disease)
Thromboangiitis obliterans
(Buerger’s disease)
Acute febrile illness of children, with lymphadenopathy, fever, desquamation of skin, and conjunctivitis. 1% to 2%
of patients die from cardiac complications, usually resulting from coronary arteritis.
Disease in men with history of smoking; inflammatory occlusive vascular disease of intermediate to small-sized
arteries and veins of extremities.

Hypocomplementemic Hypersensitivity vasculitis syndrome, with depression of early components of complement and persistent urticaria..
vasculitis
Erythema nodosum Recurrent painful erythematous nodular lesions on dorsal surface of extremities; associated with a variety of
infectious diseases and sarcoidosis. Histopathology may include vasculitic lesions.

Erythema elevatum diutinum Rare chronic skin disorder of persistent red, purple, and yellow papules, nodules, and plaques. Histology is
leukocytoclastic vasculitis.

*Compiled and adapted from Cupps, T. R., and Fauci, A.S.: The Vasculitides. Philadelphia, W. B. Saunders Co., 1981; and Fauci, A. S., Haynes, B. F.,
and Katz, P.:The spectrum of vasculitis. Clinical, pathologic, immunologic, and therapeutic considerations. Ann. Intern. Med., 89:660, 1978.
PAN = polyarteritis nodosa. SNV = systemic necrotizing vasculitis. HSV = hypersensitivity vasculitis.
Criteria for the Classification of Wegener’s
Granulomatosis*
CRITERION DEFINITION
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 Histologic changes showing granulomatous inflammation within

on biopsy the wall of an artery or in the perivascular or extravascular area
(artery or arteriole)

*For purposes of classification, a patient shall be said to have Wegener’s granulomatosis if at least two of
these four criteria are present. The presence of any two or more criteria yields a sensitivity of 88.2% and a
specificity of 92.0%.

Leavitt RY, Fauci AS, Bloch DA, et al: The American College of Rheumatology 1990 criteria for the
classification of Wegener’s granulomatosis. Arthritis Rheum 33:1101-1107,1990
 Criteria for the Classification of Acute Gouty Arthritis
A. The presence of characteristic urate crystals in the joint fluid, or

B. A tophus proved to contain urate crystals by chemical means or polarized light

microscopy, or

C. The presence of 6 of the following 12 clinical, laboratory, and x-ray phenomena

listed below:

1. More than one attack of acute arthritis

2. Maximal inflammation developed within 1 day
3. Attack of monarticular arthritis
4. Joint redness observed
5. First metatarsophalangeal joint painful or swollen
6. Unilateral attack involving first metatarsophalangeal joint
7. Unilateral attack involving tarsal joint
8. Suspected tophus
9. Hyperuricemia
10. Asymmetric swelling within a joint (roentgenogram)
11. Subcortical cysts without erosions (roentgenogram)
12. Negative culture of joint fluid for microorganisms during attack

Yu KH: Recurrent acute monoarthritis of low limb joints in male.

 高尿酸血症
高尿酸血症

痛風

約10-20%

高尿酸血症中只有10-20%得到痛風
 Crystal Deposition Diseases of Joints

Crystal Distribution of deposits Disorder Chronic disease

Monosodium urate Peripheral (feet, hands) Acute gout Chronic tophaceous gout
monohydrate (MSU)

Calcium pyrophosphate Intermediate (knees, wrists, elbows, Pseudogout Chronic destructive

dihydrate (CPPD) hips, shoulders, hands) ‘pyrophosphate arthropathy’

Hydroxyapatite Central (shoulders, hips, spine, knees) Acute calcific periarthritis Some forms of osteoarthritis
 Classification of Arthritis
( How to describe a disease by one sentence )

™ Rheumatoid arthritis
™ Dermatomyositis/ Polymyositis (DM/PM)
™ Scleroderma (PSS)
™ Systemic lupus erythematosus (SLE)
™ Vasculitis
™ Osteoarthritis (OA)
™ Gout/ Pseudogout/HA (Crystal arthritis)
™ Seronegative spondyloarthropathy or
arthritis (Ankylosing Spondylitis, Psoriatic
arthritis, Reiter syndrome, Inflammatory Bowel
Disease)
Seronegative spondyloarthropathy
Ankylosing Spondylitis,
Psoriatic arthritis,
Reiter syndrome,
Inflammatory Bowel Disease

• Sacroiliitis (SI joint blurring)

• Enthesopathy
• Sausage finger/toe
• Eye/skin/GI/GU
• Familial aggregation
• HLA-B27
 Definition of Reiter’s Syndrome

Seronegative asymmetric arthropathy (predominantly

lower extremity)
Plus one or more of the following:
Urethritits/cervicitis
Dysentery
Inflammatory eye disease
Mucocutaneous disease: balanitis, oral ulceration, or keratodermia
Exclusions:
Primary ankylosing spondylitis
Psoriatic arthropathy
Other rheumatic disease
 Criteria for Behçet’s Disease
Recurrent oral ulceration Minor aphthous, major aphthous, or herpetiform
ulceration observed by physician or patient, which
recurred at least 3 times in one 12-month period
Plus 2 of:

Recurrent genital ulceration Aphthous ulceration or scarring, observed by

physician or patient
Eye lesions Anterior uveitits, posterior uveitis, or cells in
vitreous on slit lamp examination; or Retinal
vasculitis observed by ophthalmologist
Skin lesions Erytherma nodosum observed by physician or
patient, pseudofolliculitis, or papulopustular
lesions; or acneiform nodules observed by
physician in postadolescent patients not on
corticosteroid treament
Positive pathergy test Read by physician at 24-48h.

(Findings applicable only in absence of other clinical explanations.)

International Study Group for Behcet’s Disease Lancet:1990; 335:1078-80

 CLINICAL CRITERIA FOR ANKYLOSING
SPONDYLITIS (NEW YORK, 1966)
Diagnosis

1. Limitation of motion of the lumbar spine in all three planes- anterior flexion, lateral flexion, and
extension
2. History or the presence of pain at the dorsolumbar junction or in the lumbar spine
3. Limitation of chest expansion to 1 inch (2.5 cm) or less, measured at the level of the fourth
intercostal space

Grading

Definite AS
1. Grade 3-4 bilateral sacroiliitis with at least one clinical criterion
2. Grade 3-4 unilateral or grade 2 bilateral sacroiliitis with clinical criterion 1 (limitation of back
movement in all three planes) or with both clinical criteria 2 and 3 (back pain and limitation of
chest expansion)
Probable AS
Grade 3-4 bilateral sacroiliitis with no clinical criteria
 Criteria for the Classification of Spondyloarthropathy*
Inflammatory spinal pain
or
Synovitis
Asymmetric or
Predominantly in the lower limbs
and one or more of the following
Positive family history
Psoriasis
Inflammatory bowel disease
Urethritis, cervicitis, or acute diarrhea within 1 month before arthritis

Buttock pain alternating between right and left gluteal areas

Enthesopathy
Sacroiliitis
* This classification method yields a sensitivity of 78.4% and a specificity of 89.6%. When radiographic evidence of sacroiliitis was included, the
sensitivity improved to 87.0% with a minor decrease in specificity to 86.7%. Definition of the variables used in classification criteria follow.
VARIBLE DEFINITION
Inflammatory spinal pain History or present symptoms of spinal pain in back, dorsal, or cervical region, with at least four of the following: (a)
onset before age 45, (b) insidious onset, (c) improved by exercise, (d) associated with morning stiffness, (e) at least 3
months’ duration
Synovitis Past or present asymmetric arthritis or arthritis predominantly in the lower limbs
Family history Presence in first-degree or second-degree relatives of any of the following: (a) ankylosing spondylitis, (b) psoriasis, (c)
acute uveitis, (d) reactive arthritis, (e) inflammatory bowel disease
Psoriasis Past or present psoriasis diagnosed by a physician
Inflammatory bowel Past or present Crohn’s disease or ulcerative colitis diagnosed by a physician and confirmed by radiographic
disease examination or endoscopy
Alternating buttock pain Past or present pain alternating between the right left gluteal regions
Enthesopathy Past or present spontaneous pain or tenderness at examination of the site of the insertion of the Achilles tendon or
plantar fascia
Acute diarrhea Episode of diarrhea occurring within one month before arthritis
Urethritis Nongonococcal urethritis or cervicitis occurring within one month before arthritis
Sacroiliitis Bilateral grade 2-4 or unilateral grade 3-4, according to the following radiographic grading system: 0 = normal, 1 =
possible, 2 = minimal, 3 = moderate, and 4 = ankylosis

Dougados M, Van Der Linden S, Juhlin R, et al: The European Spondylarthropathy Study Group preliminary criteria for the classification of
spondylarthropathy. Arthritis Rheum 34:1218-1227,1991
 The 1990 Criteria for the Classification of
Fibromyalgia*
1. History of widespread pain
Definition: Pain is considered widespread when all of the following are present: pain in the left side of the body,
pain in the right side of the body, pain above the waist, and pain below the waist. In addition, axial skeletal pain
(cervical spine or anterior chest or thoracic spine or low back ) must be present. In this definition shoulder and
buttock pain is considered as pain for each involved side. Low back pain is considered lower segment pain.
2. Pain in 11 of 18 tender point sites on digital palpation
Definition: Pain, on digital palpation, must be present in at least 11 of the following 18 tender point sites. Digital
palpation should be performed with an approximate force of 4 kg. For a tender point to be considered “positive” the
subject must state that the palpation was painful. “Tender” is not to be considered painful.
Occiput: bilateral, at the suboccipital muscle insertions
Low cervical: bilateral, at the anterior aspects of the inter-transverse spaces at C5-C7
Trapezius: bilateral, at the midpoint of the upper border
Supraspinatus: bilateral, at origins, above the scapula spine near the medial border
2nd rib: bilateral, at the second costochondral junctions, just lateral to the junctions on upper surfaces
Lateral epicondyle: bilateral, 2 cm distal to the epicondyles
Gluteal: bilateral, in upper outer quadrants of buttocks in anterior fold of muscle
Greater trochanter: bilateral, posterior to the trochanteric prominence
Knees: bilateral, at the medial fat pad proximal to the joint line

*For classification purposes patients will be said to have fibromyalgia if both criteria are satisfied. Widespread pain must have been present for
at least 3 months. The presence of a second clinical disorder does not exclude the diagnosis of fibromyalgia.
From Wolfe F, Smythe HA, Yunus MB, et al: The American College of Rheumatology 1990 criteria for the classification of fibromyalgia: Report
of the Multicenter Criteria Committee. Arthritis Rheum 33:160-172, 1990.
Criteria for the Classification of Giant Cell
Arteritis*
CRITERION DEFINITION
1. Age at disease onset ≧50 years Development of symptoms or findings beginning at age 50
or older
2. New headache New onset of or new type of localized pain in the head
3. Temporal artery abnormality Temporal artery tenderness to palpation or decreased
pulsation, unrelated to arteriosclerosis of cervical arteries
4. Elevated ESR Erythrocyte sedimentation rate ≧50 mm/hour by the
Westergren method
5. Abnormal artery biopsy Biopsy specimen with artery showing vasculitis
characterized by a predominance of mononuclear cell
infiltration or granulomatous inflammation, usually with
multinucleated giant cells.

* For purposes of classification, a patient shall be said to have giant cell (temporal) arteritis if at least three
of these five criteria are present. The presence of any three or more criteria yields sensitivity of 93.5% and
a specificity of 91.2%.

Hunder GG, Bloch DA, Michel BA, et al: the American College of Rheumatology 1990 criteria for the
classification of giant cell arteritis. Arthritis Rheum 33:1122-1128,1990
Criteria for the Classification of
Takayasu Arteritis*
CRITERION
1. Age at disease onset ≦40 years
2. Claudication of extremities
3. Decreased brachial artery pulse
4. BP difference > 10 mm Hg
5. Bruit over subclavian arteries or
aorta
6. Arteriogram abnormality
DEFINITION
Development of symptoms or findings related to Takayasu arteritis
at age ≦40 years
Development and worsening of fatigue and discomfort in muscles
of one or more extremity while in use, especially the upper
extremities
Decreased pulsation of one or both brachial arteries
Difference of >10 mm Hg in systolic blood pressure between arms
Bruit audible on auscultation over one or both subclavian arteries
or abdominal aorta
Arteriographic narrowing or occlusion of the entire aorta, its
primary branches, or large arteries in the proximal upper or lower
extremities, not due to arteriosclerosis, fibromuscular dysplasia,
or similar causes; changes usually focal or segmental

* For purposes of classification, a patient shall be said to have Takayasu arteritis if at least three of these six
criteria are present. The presence of any three or more criteria yields a sensitivity of 90.5% and a specificity of
97.8%. BP = blood pressure (systolic; difference between arms).

Arend WP, Michel BA, Bloch DA, et al: The American College of Rheumatology 1990 criteria for the
classification of Takayasu arteritis. Arthritis Rheum 33:1129-1132,1990
 Criteria for the Classification of
Polyarteritis Nodosa*
CRITERION
1. Weight loss ≧4kg
2. Livedo reticularis
3. Testicular pain or tenderness
4. Myalgias, weakness, or leg
tenderness
5. Mononeuropathy or
polyneuropathy
6. Diastolic BP >90 mm Hg
7. Elevated BUN or creatinine
8. Hepatitis B virus
9. Arteriographic abnormality
10. Biopsy of small or medium-
sized artery containing PMN
DEFINITION
Loss of 4 kg or more of body weight since illness began, not due to dieting or other
factors
Mottled reticular pattern over the skin of portions of the extremities or torso
Pain or tenderness of the testicles, not due to infection, trauma, or other causes
Diffuse myalgias (excluding shoulder and hip girdle) or weakness of muscles or
tenderness of leg muscles
Development of mononeuropathy, multiple mononeuropathies, or polyneuropathy
Development of hypertension with the diastolic BP higher than 90 mm Hg
Elevation of BUN >40 mg/dl or creatinine >1.5 mg/dl, not due to dehydration or
obstruction
Presence of hepatitis B surface antigen or antibody in serum
Arteriogram showing aneurysms or occlusions of the visceral arteries, not due to
arteriosclerosis, fibromuscular dysplasia, or other noninflammatory causes
Histologic changes showing the presence of granulocytes or granulocytes and
mononuclear leukocytes in the artery wall

* For classification purposes, a patient shall be said to have polyarteritis nodosa if at least three of these 10 criteria are present. The presence
of any three or more criteria yields a sensitivity of 82.2% and a specificity of 86.6%. BP = blood pressure; BUN = blood urea nitrogen; PMN =
polymorphonuclear neutrophils.

Lightfoot RW Jr, Michel BA, Bloch DA, et al: The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa.
Arthritis Rheum 33:1088-1093, 1990
Criteria for the Classification of
Henoch-Schönlein Purpura*
CRITERION
1. Palpable purpura
DEFINITION
Slightly raised “palpable” hemorrhagic skin lesions, not
related to thrombocytopenia

2. Age ≦20 at disease onset Patient 20 years or younger at onset of first symptoms
3. Bowel angina Diffuse abdominal pain, worse after meals, or the
diagnosis of bowel ischemia, usually including bloody
diarrhea

4. Wall granulocytes on biopsy Histologic changes showing granulocytes in the walls

of arterioles or venules

*For purposes of classification, a patient shall be said to have Henoch-Schönlein purpura if at least
two of these four criteria are present. The presence of any two or more criteria yields a sensitivity
of 87.1% and a specificity of 87.7%.

Mills JA, Michel BA, Bloch DA, et al: The American College of Rheumatology 1990 criteria for the
classification of Henoch-Schönlein purpura . Arthritis Rheum 33:1114-1121,1990
Criteria for the Classification of
Hypersensitivity Vasculitis*
CRITERION DEFINITION
Age at disease onset >16 years Development of symptoms after age 16

Medication at disease onset Medication was taken at the onset of symptoms that
may have been a precipitating factor
Palpable purpura Slightly elevated purpuric rash over one or more areas
of the skin; does not blanch with pressure and is not
related to thrombocytopenia
Maculopapular rash Flat and raised lesions of various sizes over one or
more areas of the skin
Biopsy including arteriole and venule Histologic changes showing granulocytes in a
perivascular or extravascular location

*For purposes of classification, a patient shall be said to have Hypersensitivity Vasculitis if at least two
of these five criteria are present. The presence of any two or more criteria yields a sensitivity of 71.0%
and a specificity of 83.9%.

Calabrese LH, Michel BA, Bloch DA, et al: The American College of Rheumatology 1990 criteria for the
classification of hypersensitivity vasculitis . Arthritis Rheum 33:1108-1113,1990
Criteria for the Classification of
Churg-Strauss Syndrome*
CRITERION DEFINITION
Asthma History of wheezing or diffuse high-pitched rales on expiration
Eosinophilia Eosinophilia >10% on white blood cell differential count
Mononeuropathy or Development of mononeuropathy, multiple mononeuropathies, or
polyneuropathy polyneuropathy (ie, glove/stocking distribution) attributable to a
systemic vasculitis
Pulmonary infiltrates, non-fixed Migratory or transitory pulmonary infiltrates on radiographs (not
including fixed infiltrates), attributable to a systemic vasculitis
Paranasal sinus abnormality History of acute or chronic paranasal sinus pain or tenderness or
radiographic opacification of the paranasal sinuses
Extravascular eosinophils Biopsy including artery, arteriole, or venule, showing
accumulations of eosinophils in extravascular areas

*For purposes of classification, a patient shall be said to have Churg-Strauss syndrome if at least four of these
six criteria are present. The presence of any four or more of the six criteria yields a sensitivity of 85% and a
specificity of 99.7%.

Masi AT, Hunder GG, Lie JT, et al: The American College of Rheumatology 1990 criteria for the classification of
Churg-Strauss syndrome (allergic granulomatosis and angiitis) . Arthritis Rheum 33:1094-1100,1990
Diagnostic Criteria for the Classification of Juvenile
Rheumatoid Arthritis*

1. Age at onset less than 16 years

2. Arthritis in one or more joints defined as swelling or effusion, or the
presence of two or more of the following signs: limitation of range of
motion, tenderness or pain on motion, and increased heat.
3. Duration of disease of 6 weeks to 3 months
4. Type of onset of disease during the first 4 to 6 months classified as
a. Polyarthritis- 5 joints or more
b. Oligoarthritis- 4 joints or fewer (2-4 joints)
c. Systemic disease
(1) Arthritis
(2) Intermittent fever
(3) Rheumatoid rash
(4) Visceral disease (hepatosplenomegaly, lymphadenopathy, etc.)
5. Exclusion of other diseases

*Modified from Cassidy J.T. et al.: Arthritis Rheum. 29:274, 1986

 Subgroups of Juvenile Rheumatoid Arthritis
% of Age at Joints Serologic and Extraarticular
Subgroup total Sex ratio onset affected genetic tests manifestations Prognosis
Systemic onset 20 60% boys Any age Any joints ANA negative, High fever, rash 25% severe
RF negative organomegaly, arthritis
polyserositis,
leukocytosis
growth
retardation
Rheumatoid factor 25 90% girls Any age Any joints ANA 25%, RF Low-grade fever, 10-15% severe
Negative negative mild anemia, arthritis
polyarticular malaise, growth
reardation
Rheumatoid factor 5-10 80% girls Late Any joints ANA 75%, RF Low-grade fever, >50% severe
Positive childhood 100% anemia, malaise, arthritis
polyarticular rheumatoid
nodules
Pauciarticular with 35-40 80% girls Early Few large joints ANA 50%, RF Few constitutional Severe arthritis
chronic iridocyclitis childhood (hips and negative complaints; uncommon;
sacroiliac chronic 10-20% ocular
joints spared) iridocyclitis in damage from
50% iridocyclitis
Pauciarticular with 10 90% boys Late Few large joints ANA negative, Few constitutional Some have
sacroiliitis childhood (hips and RF negative, complaints; acute ankylosing
sacroiliac HLA-B27 75% iridocyclitis in 5- spondylitis at
involvement 10% during follow up
common) childhood
Diagnostic Guidelines for Kawasaki
Syndrome*
Fever lasting >5 days plus four of the following criteria
1. Polymorphous rash
2. Bilateral conjunctival injection
3. One or more of the following mucous membrane changes:
Diffuse injection of oral and pharyngeal mucosa
Erythema or fissuring of the lips
Strawberry tongue
4. Acute, nonpurulent cervical lymphadenopathy (one lymph node must be >1.5 cm)
5. One or more of the following extremity changes:
Erythema of palms and /or soles
Indurative edema of hands and /or feet
Membranous desquamation of the fingertips
* Other illnesses with similar clinical signs must be excluded.

Kawasaki T, Kosaki T, Okawa S, et al: A new infantile acute febrile mucocutaneous lymph node syndrome
(MLNS) prevailing in Japan. Pediatrics 54:271-276,1974
 Criteria for the Diagnosis of Rheumatic
Fever*
MAJOR MINOR MANIFESTATIONS SUPPORTING EVIDENCE OF PRECEDING
MANIFESTATIONS STREPTOCOCCAL INFECTION
Carditis Clinical findings Positive throat culture or rapid streptococcal
antigen test
Polyarthritis Arthralgia Elevated or rising streptococcal antibody titer
Chorea Fever
Erythema marginatum Laboratory findings
Subcutaneous nodules Elevated acute phase
reactants
Erythrocyte
sedimentation rate
C-reactive protein
Prolonged PR interval

If supported by evidence of preceding group A streptococcal infection, the presence of two major manifestations,
or of one major and two minor manifestations indicates a high probability of acute rheumatic fever.
Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council
on Cardiovascular Disease in the Young, American Heart Association: Guidelines for the diagnosis of rheumatic
fever: Jones criteria, updated 1992, JAMA 268:2069-2073,1992
 JONES CRITERIA ( REVISED )*
Major Manifestations Minor Manifestations

Carditis Fever
Polyarthritis Arthralgia
Chorea Previous rheumatic fever or rheumatic heart disease
Erythema marginatum Elevated ESR or positive CRP
Subcutaneous nodules Prolonged PR interval

Plus supporting evidence of preceding streptococcal infection:

History of recent scarlet fever; positive throat culture for group A streptococcus; increased
ASO titer or other streptococcal antibodies.

* Circulation 32:664, 1965 ** 1992 revised

 Criteria for the Diagnosis of
Adult Onset Still’s Disease
A diagnosis of adult Still’s disease requires the presence of all of
the following:
) Fever ≥ 39℃
) Arthralgia or arthritis
) Rheumatoid factor < 1:80
) ANA < 1:100
In addition to any two of the following
) WBC ≥ 15,000 cells/mm3
) Still’s rash
) Pleuritis or pericarditis
) Hepatomegaly or splenomegaly or generalized lymphadenopathy

Cush et al. Arthritis Rheum 30:186-194, 1987

Criteria Proposed for Guidance in the Diagnosis of
Adult Onset Still’s Disease

Major Manifestations Minor Manifestations

z Persistent or intermittent fever z Serositis
z Evanescent erythematous z Sore throat
maculopapular rash z Hepatic dysfunction
z Poly or oligoarthritis z Lymph node enlargement
z Neutrophilic leukocytosis z Splenomegaly
z Other organ involvement

Reginato et al. Seminar in Arthritis and Rheumatism 17:37-59, 1987

All four major criteria are suggestedto establish a diagnosis of definite AOSD.
Patients with fever, arthritis, one other major, and one or more minor criteria
would be considered probable AOSD.
 Preliminary Criteria for a Classification of Adult
Onset Still’s Disease

Major criteria
z Fever ≥ 39℃ lasting 1 week or longer
z Arthralgia lasting 2 weeks or longer
z Typical rash
z Leukocytosis (≥10,000/mm3) including ≥ 80% of granulocytes
Minor criteria
z Sore throat
z Lymphadenopathy and/or splenomegaly
z Liver dysfunction
z Negative RF and negative ANA
Exclusions
z Infections
z Malignancies
z Rheumatic diseases

Classification of adult Still’s disease requires 5 or more criteria including 2 or more major criteria. All criteria are
applicable only in the absence of other clinical explanations. Yamaguchi et al. J Rheumatology 1992;19:424-435