Критерии за класификация

Критерии за класификация на СЛЕ
(минимум 4 от критериите по–долу са нужни, за да се класифицира пациент като такъв имащ SLE и да се насочи към клинични изследвания)
1)орални ранички;
2)артрити;
3)серозити – плеврити или перикардити;
4)ренални нарушения – повече от 0.5 g/дневно белтък в урината или клетъчни частици (при микроскопско изследване);
5)фоточувствителност;
6)неврологични нарушения – психози, кризи, Синдром на Франк(?);
7)маларийни обриви по скулите;
8')дискообразни обриви;
9)антиядрените антитела са с увеличени титри (позитивен АНА – флуоресцентен тест);
10)позитивни анти-ДНК или анти-Sm антитела, или позитивен тест за антифосфолипидни антитела, позитивен фалшив тест за сифилис за не по-малко от 6 месеца, потвърден от Treponema pallidium immobilization test или флуоресцентен тест за антитела на същата бактерия;
11)хематологични отклонения и хемолитични отклонения с ретикулоцитоза - левкопения (<4000/mm3), лимфопения (<1500/mm3), хемолитична анемия, или тромбоцитопения (<100000/mm3) ,в отсъствие на индуциращи такива нива лекарства;
12)удължено време на съсирване на протромбина;

Панел за оценка на СЛЕ:
-dsDNA
-Sm(U2-U4 RNP)
-SS-A/Ro 60
-U1-RNP




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.

11. ANTINUCLEAR ANTIBODY. ANTINUCLEAR ANTIBODY BY IMMUNOFLUORESCENCE OR EQUIVALENT ASSAY IN THE ABSENCE OF DRUGS ASSOCIATED WITH DRUG INDUCED SLE.
Изискват се 4 или повече критерия за да се постави диагноза -появили се едновременно или в поредица.

1.Критерии за клинична ремисия при ревматоидните артрити
а. Продължението на сутрешната скованост не продължава повече от 15 мин.
б. Няма умора.
в. Липсват симптоми на ставни болки.
г. Няма ставна умора или болки при движение
д. Липсва подуване и отоци в меките тъкани или в обвивките на сухожилията
е. ESR < 30 (жени) AND <20 (мъже)

2.Синдром на Сьогрен
-SS - A/Ro
-SS – B/La
-Golgin
-GWB

3.Склеродермия
Критерии за класификация на Системна склероза (Склеродермия)
A. Основни критерии:
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. Минорни критерии:
1. Склеродактилия:кожни изменения(като по-горните) ограничени до пръстите;
2. Кератинизирани белези или загуба на субстанция по повърхността на пръстите;
3. Първична пулмонарна фиброза
Изискват се един основен или два минорни критерия за диагноза

-CENP
-CENP-A
-CENP-C
-фибриларин (U3-RNP)
-Scl-70 (topo 1)
-PM/Scl
-Ku

4.Васкулити
-антитела на ендотелните клетки
-pANCA и cANCA
-протеиназа 3 (PR-3)
-миелопероксидаза

5.ENA профил
-ANA
-dsDNA
-хромати
-ANA скрийн
-U1-RNP
-SS-B/La
-Ro52
-SS-A/Ro60
-Scl-70 (topo 1)
-Jo- 1
-Ribosomal P
-Hep-2 + Hep-2000

6.RA
-CCP
-RA33 антитела
-ревматоиден фактор
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.

Изискват се 5 или повече критерия за не по-малко от 2 последователни месеца.
THERE MUST BE NO CLINICAL MANIFESTATION OF ACTIVE VASCULITIS, PERICARDITIS, PLEURITIS, OR MYOSITIS OR UNEXPLAINED RECENT WEIGHT LOSS OR FEVER ATTRIBUTABLE TO RA.

Критерии за определяне на прогреса при 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.

Класификация на функционалния капацитет при ревматоидните артрити.
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.
Подразделени на 3 подтипа:
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


7.Болест на Крон
-ASCA

8.Артрити

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


-ENA профил
-ревматоиден фактор
-CCP
-RA33

9.Миозити

Критерии за диагноза на полимиозити и дерматомиозити
Главни критерии
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. Основни идиопатични полимиозити
2. Основни идиопатични дерматомиозити
3. Асоциия с неоплазма
4. Детски тип асоцииран с васкулити
5. Асоциация с съдова колагенна болест


-Jo-1 антитела
-Mi-2 антитела
-PM/Scl
-Ro52
-Ku
-PL-12, PL-7

10.DLE
-хроматинови антитела
-хистонови антитела

11.CNS-SLE
-невронални антитела (GM1, GM2, GM3)
-рибозомални P антитела

Критерии за Смесена болест на съединителната тъкан
Серологични белези.
1. Високи титри на 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. Не се откриват Sm-антитела.
Клинични белези.
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