Diagnostic Criteria for the Rheumatic Diseases

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Ankylosing Spondylitis
Gout
Juvenile Rheumatoid Arthritis
Mixed connective tissue disease
Polymyositis/dermatomyositis
Rheumatic fever
Rheumatoid Arthritis
Diagnostic criteria
Criteria for determining progression
Functional capacity staging
Systemic Sclerosis/Scleroderma
Systemic Lupus Erythematosus

A. RHEUMATOID ARTHRITIS.

ARNETT ET AL ARTH RHEUM 30:S17, 1987

1 MORNING STIFFNESS FOR AT LEAST ONE HOUR AND PRESENT FOR AT LEAST SIX WEEKS.
2. SWELLING OF THREE OR MORE JOINTS FOR AT LEAST SIX WEEKS.
3. SWELLING OF WRIST,MCP OR PIP FOR SIX OR MORE WEEKS.
4. SYMMETRICAL JOINT SWELLING.
5. HAND XRAY CHANGES TYPICAL OF RA THAT MUST INCLUDE EROSIONS OR UNEQUIVOCAL BONY DECALCIFICATION.
6. RHEUMATOID NODULES.
7. SERUM RHEUMATOID FACTOR BY A METHOD POSITIVE IN LESS THAN 5% OF NORMALS.

REQUIRE AT LEAST FOUR CRITERIA.

CRITERIA FOR CLINICAL REMISSION IN RHEUMATOID ARTHRITIS.

1. DURATION OF MORNING STIFFNESS NOT EXCEEDING 15 MINUTES.
2. NO FATIGUE.
3. NO SYMPTOMS OF JOINT PAIN.
4. NO JOINT TENDERNESS OR PAIN ON MOTION.
5. NO SOFT TISSUE SWELLING IN JOINTS OR TENDON SHEATHS.
6. ESR < 30 (FEMALE) AND <20 (MALE)

REQUIRE 5 OR MORE FOR AT LEAST 2 CONSECUTIVE MONTHS.
THERE MUST BE NO CLINICAL MANIFESTATION OF ACTIVE VASCULITIS, PERICARDITIS, PLEURITIS, OR MYOSITIS OR UNEXPLAINED RECENT WEIGHT LOSS OR FEVER ATTRIBUTABLE TO RA.

CRITERIA FOR DETERMINATION OF PROGRESSION OF RA.

STAGE 1. EARLY. NO DESTRUCTIVE CHANGE ON X-RAYS. OSTEOPOROSIS MAY BE PRESENT.
STAGE 2. MODERATE. XRAY EVIDENCE OF OSTEOPOROSIS +/- SLIGHT SUBCHONDRAL BONE DESTRUCTION. SLIGHT CARTILAGE DAMAGE MAY BE PRESENT. NO JOINT DEFORMITY, ALTHOUGH LIMITATION OF JOINT MOBILITY MAY BE PRESENT. ADJACENT MUSCLE ATROPHY. EXTRA ARTICULAR SOFT TISSUE LESIONS MAY BE PRESENT INCLUDING NODULES AND TENOSYNOVITIS.
STAGE 3. XRAY CHANGES OF CARTILAGE AND BONE DESTRUCTION IN ADDITION TO OSTEOPOROSIS. JOINT DEFORMITY, INCLUDING SUBLUXATION, ULNAR DEVIATION OR HYPER EXTENSION WITHOUT FIBROUS OR BONY ANKYLOSIS. EXTENSIVE MUSCLE ATROPHY. EXTRA ARTICULAR SOFT TISSUE LESIONS MAY BE PRESENT INCLUDING NODULES AND TENOSYNOVITIS.
STAGE 4. TERMINAL. FIBROUS OR BONY ANKYLOSIS. CRITERIA OF STAGE 3.

CLASSIFICATION OF FUNCTIONAL CAPACITY IN RHEUMATOID ARTHRITIS.

CLASS 1. COMPLETE. CAN DO ALL USUAL DUTIES WITHOUT HANDICAP.
CLASS 2. CAN DO NORMAL ACTIVITIES DESPITE DISCOMFORT OR LIMITED MOBILITY OF ONE OR MORE JOINTS. CLASS 3. CAN DO ONLY FEW OR NONE OF THE DUTIES OF USUAL OCCUPATION OR OF SELF-CARE.
CLASS 4. LARGELY OR WHOLLY INCAPACITATED WITH PATIENT CONFINED TO BED OR CHAIR, PERMITTING LITTLE OR NO SELF-CARE.

CRITERIA FOR DIAGNOSIS OF JUVENILE RHEUMATOID ARTHRITIS.

SUBDIVIDED INTO 3 SUB-TYPES:
1. SYSTEMIC.
2. POLYARTICULAR.
3. PAUCIARTICULAR.

A. GENERAL CRITERIA FOR DIAGNOSIS OF JRA.

1. PERSISTENT ARTHRITIS FOR AT LEAST 6 WEEKS IN ONE OR MORE JOINTS.
2. EXCLUSION OF OTHER TYPES OF ARTHRITIS / OTHER RHEUMATIC DISEASE rheumatic fever systemic lupus erythematosus Ankylosing spondylitis Polymyositis/dermatomyositis vasculitis syndromes Scleroderma psoriatic arthritis Reiter's syndrome Sjogrens syndrome mixed connective tissue disease Behcets syndrome INFECTIOUS ARTHRITIS. INFLAMMATORY BOWEL DISEASE. NEOPLASTIC DISEASES. NON RHEUMATIC DISEASES OF BONES AND JOINTS. HEMATOLOGICAL DISEASES. PSYCHOGENIC ARTHRALGIA. MISCELLANEOUS Sarcoid hypertrophic osteoarthropathy villonodular synovitis chronic active hepatitis familial Mediterranean fever

B. SYSTEMIC ONSET JRA.

JRA WITH PERSISTENT INTERMITTENT FEVER TO 103 degrees FARENHEIGHT OR MORE, WITH OR WITHOUT RHEUMATOID RASH OR OTHER ORGAN INVOLVEMENT. TYPICAL FEVER AND RASH WITHOUT ARTHRITIS MAY BE TERMED PROBABLE SYSTEMIC ONSET JRA.....
SUBTYPES:
polyarthritis
oligoarthritis

C. PAUCIARTICULAR JRA.

JRA WITH ARTHRITIS OF 4 OR FEWER JOINTS DURING THE FIRST SIX MONTHS OF DISEASE......
SUBTYPES:
ANF +VE chronic uveitis
rheumatoid factor +ve
seronegative B27 +VE
not otherwise classified

D. POLYARTICULAR JRA.

JRA WITH ARTHRITIS OF 5 OR MORE JOINTS IN THE FIRST 6 MONTHS OF DISEASE

SUBTYPES:
rheumatoid factor +ve
not otherwise classified

REVISED CRITERIA FOR SYSTEMIC LUPUS ERYTHEMATOSUS.

1. MALAR RASH. FIXED ERYTHEMA, FLAT OR RAISED OVER MALAR EMINENCES, TENDING TO SPARE THE NASOLABIAL FOLDS.
2. DISCOID RASH. ERYTHEMATOUS RAISED PATCHES WITH ADHERENT KERATOTIC SCALING AND FOLLICULAR PLUGGING. ATROPHIC SCARRING CAN OCCUR IN OLDER LESIONS.
3. PHOTOSENSITIVITY. SKIN RASH AS A RESULT OF UNUSUAL REACTION TO SUNLIGHT BY PATIENT HISTORY OR CLINICAL OBSERVATION.
4. ORAL ULCERS. ORAL OR NASOPHARYNGEAL ULCERATION, USUALLY PAINLESS, OBSERVED BY PHYSICIAN.
5. ARTHRITIS. NON-EROSIVE ARTHRITIS INVOLVING 2 OR MORE PERIPHERAL JOINTS CHARACTERIZED BY TENDERNESS, SWELLING OR EFFUSION.
6. SEROSITIS. PLEURITIS HISTORY/RUB/EFFUSION...OR..PERICARDITIS ECG/RUB/EFFUSION.
7. RENAL DISORDER. PERSISTENT PROTEINURIA >0,5g/DAY OR >3+ ..OR. CELLULAR CASTES (RBC/HAEMOGLOBIN/GRANULAR/TUBULAR/MIXED).
8. NEUROLOGICAL DISORDER. SEIZURES, IN THE ABSENCE OF OFFENDING DRUGS OR KNOWN METABOLIC DERANGEMENTS, i.e. UREMIA, KETOACIDOSIS, OR ELECTROLYTE IMBALANCE...OR PSYCHOSIS, IN THE ABSENCE OF OFFENDING DRUGS OR KNOWN METABOLIC DERANGEMENTS, i.e. UREMIA, KETOACIDOSIS, OR ELECTROLYTE IMBALANCE.
9. HEMATOLOGICAL DISORDER. HEMOLYTIC DISORDER WITH RETICULOCYTOSIS. LEUKOPENIA <4000/mm3, ON 2 OR MORE OCCASIONS. LYMPHOPENIA <1500/mm3, ON 2 OR MORE OCCASIONS. THROMBOCYTOPENIA <100000/mm3 IN THE ABSENCE OF OFFENDING DRUGS.
10. IMMUNOLOGIC DISORDER. POSITIVE LE PREPARATION. ANTI DNA. ANTI Sm NUCLEAR ANTIGEN. FALSE POSITIVE TEST FOR SYPHILIS FOR AT LEAST SIX MONTHS, CONFIRMED BY TREPONEMA PALLIDUM IMMOBILIZATION OR FLUORESCENT TREPONEMAL ANTIBODY ABSORPTION TEST.
11. ANTINUCLEAR ANTIBODY. ANTINUCLEAR ANTIBODY BY IMMUNOFLUORESCENCE OR EQUIVALENT ASSAY IN THE ABSENCE OF DRUGS ASSOCIATED WITH DRUG INDUCED SLE.

REQUIRE 4 OR MORE CRITERIA FOR DIAGNOSIS, SERIALLY OR SIMULTANEOUSLY.

JONES CRITERIA FOR THE DIAGNOSIS OF RHEUMATIC FEVER.

REQUIRE: 2 MAJOR OR 1 MAJOR + 2 MINOR

CRITERIA FOR CLASSIFICATION OF SYSTEMIC SCLEROSIS (SCLERODERMA)

A. MAJOR CRITERION:
PROXIMAL SCLERODERMA:
SYMMETRICAL THICKENING, TIGHTENING, AND INDURATION OF THE SKIN OF THE FINGERS AND THE SKIN PROXIMAL TO THE METACARPOPHALANGEAL OR METATARSOPHALANGEAL JOINTS. THE CHANGES MAY AFFECT THE ENTIRE EXTREMITY, FACE, NECK AND TRUNK (THORAX AND ABDOMEN).

B. MINOR CRITERIA:
1. SCLERODACTYLY: SKIN CHANGES (AS ABOVE) LIMITED TO THE FINGERS.
2. DIGITAL PITTING SCARS OR LOSS OF SUBSTANCE OF THE FINGER PAD.
3. BASILAR PULMONARY FIBROSIS.

REQUIRE THE ONE MAJOR, OR TWO MINOR FOR DIAGNOSIS.

CRITERIA FOR THE CLASSIFICATION OF ACUTE GOUTY ARTHRITIS.

A. PRESENCE OF CHARACTERISTIC URATE CRYSTALS IN THE JOINT FLUID ..OR..
B. A TOPHUS PROVEN TO CONTAIN GOUTY CRYSTALS ..OR..
C. PRESENCE OF 6 OR MORE OF 12 CLINICAL/RADIOLOGICAL/LAB PHENOMENA :
1 > 1 attack.
2 maximal inflammation developed within 1 day.
3 attack of monoarticular arthritis.
4 joint redness observed.
5 1st MTP joint painful or swollen.
6 unilateral attack involving 1st MTP joint.
7 unilateral attack involving tarsal joint.
8 suspected tophus.
9 hyperuricaemia.
10 asymmetrical swelling within a joint (Xray).
11 subcortical cysts without erosions (Xray).
12 negative culture from joint fluid during attack.

CRITERIA FOR DIAGNOSIS OF POLYMYOSITIS AND DERMATOMYOSITIS

BOHAN ET AL NEJM 292:342-347 1975

MAJOR CRITERIA

1. SYMMETRICAL WEAKNESS OF TE LIMB GIRDLE MUSCLES AND ANTERIOR NECK FLEXORS, PROGRESSING OVER WEEKS OR MONTHS WITH OR WITHOUT DYSPHAGIA OR RESPIRATORY MUSCLE INVOLVEMENT.
2. MUSCLE BIOPSY EVIDENCE OF NECROSIS OF TYPE 1 AND 2 FIBRES, PHAGOCYTOSIS, REGENERATION WITH BASOPHILIA, LARGE VESICULAR SARCOLEMMAL NUCLEI AND PROMINENT NUCLEOLI, ATROPHY IN A PERIFASCIAL DISTRIBUTION, VARIATION IN FIBER SIZE AND ANY INFLAMMATORY EXUDATE, OFTEN PERIVASCULAR.
3. ELEVATED MUSCLE ENZYMES, ESPECIALLY CPK, ALDOLASE, LDH.
4. EMG TRIAD: SMALL POLYPHASIC ACTION POTENTIALS, POSITIVE SHARP WAVES AND INSERTIONAL IRRITABILITY AND BIZARRE HIGH FREQUENCY REPETITIVE DISCHARGES.
5. DERMATOLOGICAL COMPONENT: HELIOTROPE WITH PERIORBITAL OEDEMA. A SCALY DERMATITIS OVER DORSUM OF HANDS ESPECIALLY PIP'S (GOTTRONS SIGN), AND INVOLVEMENT OF KNEES, ELBOWS, MEDIAL MALLEOLI, FACE AND UPPER TORSO.

DEFINITE :
DERMATOMYOSITIS: 3 OR 4 CRITERIA PLUS THE RASH.
POLYMYOSITIS : 4 CRITERIA WITHOUT RASH.

PROBABLE :
DERMATOMYOSITIS: 2 CRITERIA PLUS THE RASH.
POLYMYOSITIS : 3 CRITERIA WITHOUT RASH.

POSSIBLE :
DERMATOMYOSITIS: 1 CRITERIA PLUS THE RASH.
POLYMYOSITIS : 2 CRITERIA WITHOUT THE RASH.

CLASSIFICATION.

GROUP
1. PRIMARY IDIOPATHIC POLYMYOSITIS.
2. PRIMARY IDIOPATHIC DERMATOMYOSITIS.
3. ASSOCIATION WITH NEOPLASM.
4. CHILDHOOD TYPE ASSOCIATED WITH VASCULITIS.
5. ASSOCIATION WITH COLLAGEN VASCULAR DISEASE.

CRITERIA FOR MIXED CONNECTIVE TISSUE DISEASE.

SHARP ET AL: AM J MED 52:148-159 1972

SEROLOGICAL FINDINGS.

1. HIGH TITERS OF ENA.
2. MARKED SENSITIVITY OF THE ENA TO RIBONUCLEASE, MODERATE SENSITIVITY TO TRYPSIN, AND RESISTANCE TO DNASE.
3. HIGH TITERS OF SPECKLED PATTERN ON FLUORESCENT ANTIBODY TEST.
4. NO DETECTABLE Sm ANTIBODY.

CLINICAL FINDINGS.

FEATURES SIMILAR TO SLE/SCLERODERMA/POLYMYOSITIS.

THE DIAGNOSTIC CRITERIA FOR ANKYLOSING SPONDYLITIS.

1. LIMITATION OF MOTION OF THE LUMBAR SPINE IN ALL THREE PLANES: ANTERIOR FLEXION, LATERAL FLEXION, EXTENSION.
2. HISTORY OF PAIN IN THE LUMBAR SPINE OR AT THE DORSO-LUMBAR JUNCTION.
3. LIMITED CHEST EXPANSION TO 2,5 cm OR LESS, MEASURED AT THE FOURTH INTERCOSTAL LINE.
4. SACROILIITIS.
GRADE 0: NORMAL.
GRADE 1: SUSPICIOUS.
GRADE 2: MINIMAL ABNORMALITY, SMALL AREAS OF EROSIONS OR SCLEROSIS, WITHOUT ALTERATION OF JOINT WIDTH.
GRADE 3: DEFINITE ABNORMALITY- MODERATE OR ADVANCED SACROILIITIS WITH IRREGULARITY, ONE OR MORE EROSIONS, EVIDENCE OF SCLEROSIS. PARTIAL ANKYLOSIS
GRADE 4: TOTAL ANKYLOSIS.

DEFINITE ANKYLOSING SPONDYLITIS:

GRADE 3-4 SACROILIITIS WITH AT LEAST ONE CLINICAL CRITERION. OR
GRADE 3-4 UNILATERAL OR
GRADE 2 BILATERAL SACROILIITIS, WITH CLINICAL CRITERION 1
OR CRITERION 2 AND 3.

PROBABLE ANKYLOSING SPONDYLITIS:

GRADE 3-4 SACROILIITIS WITHOUT ANY CLINICAL CRITERIA.