Diagnóstico

Avaliação para LES

EVALUATION FOR SYSTEMIC LUPUS ERYTHEMATOSUS

History

Physical examination

  •    Specific cutaneous lesions

  •    Non-specific cutaneous lesions

  •    Lymphadenopathy, arthritis

Laboratory tests

  •    ANA with profile (dsDNA, Sm)

  •    Urinalysis

  •    CBC with differential, platelets

  •    Chemistries (BUN, creatinine)

  •    Erythrocyte sedimentation rate

  •    Complement levels (C3, C4)

FAN

• 1/3 indivíduos aparentemente normais vão ter FAN reator 1:40– 13% 1:80– 5% 1 :160

• FAN reator NÃO é indicador nem de doença sistêmica nem de Lúpus

THE AMERICAN COLLEGE OF RHEUMATOLOGY 1982 REVISED CRITERIA FOR CLASSIFICATION OF SYSTEMIC LUPUS ERYTHEMATOSUS

• Criterion Definition  

• 1.    Malar rashFixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds  

• 2.    Discoid rashErythematous raised patches with adherent

keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions   

• 3.    PhotosensitivitySkin rash as a result of unusual reaction to

sunlight, by patient history or physician observation   

• 4.    Oral ulcersOral or nasopharyngeal ulceration, usually

painless, observed by physician   

Eritema Malar

Lesões discóides

Ulcerações orais

Fotossensibilidade

THE AMERICAN COLLEGE OF RHEUMATOLOGY 1982 REVISED CRITERIA FOR CLASSIFICATION OF SYSTEMIC LUPUS

ERYTHEMATOSUS

• 5.    ArthritisNon-erosive arthritis involving two or more peripheral joints, characterized by tenderness, swelling or effusion   

• 6.    Serositis    a)    Pleuritis–convincing history of pleuritic pain,

rubbing heard by a physician, or evidence of pleural effusion OR    b)    Pericarditis–documented by ECG, rub or

evidence of pericardial effusion

• 7.    Renal disorder   a)    Persistent proteinuria greater than 0.5 g/day or greater than 3+ if quantitation not performed OR    b)    Cellular casts–may be red cell, hemoglobin,

granular, tubular or mixed

Artrite

Serosite

Alterações renais persistentes

• 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 WBC on two or

more occasions

OR   c)    Lymphopenia–less than 1500/mm3 on two or more

occasions   OR   d)    Thrombocytopenia–less than 100 000/mm3 in the absence of offending drugs  

Alterações neurológicas

Alterações hematológicas

• 10.  Immunologic disorder   a)    Anti-DNA antibody to native DNA in abnormal titer   OR    b)Anti-Sm: presence of antibody to Sm nuclear antigen   OR    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 methods; or (3) 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 (FTA-

ABS)   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

Alterações imunológicas

• Anti Smith

• Anti-DNA dupla hélice

• Anti-fosfolipídeos (1997 )

• Anti-cardiolipina (IgG or IgM)• VDRL falso positivo (> 6 meses)• Anticoagulante lúpico

FAN ou ANAPeriférico

Nucleolar

Difuso

Pontilhado

Auto-anticorpos em LES

AUTOANTIBODIES ASSOCIATED WITH LUPUS ERYTHEMATOSUSAutoantibody Median prevalence[*] Molecular specificity Clinical associationsHigh specificity for SLEDsDNA[†]

(homogéneo)

60% 75% A Double-stranded (native) DNA

LE nephritis; monitoring activity of SLE

Sm(pontilhado grosso)

30% 20% A Splicesome RNP (ribonucleoprotein particles involved in splicing pre-mRNA)

− Protecao renal

rRNP 7% Ribosomal P proteins (proteins involved in ribosome function)

Neuropsychiatric LE

Low specificity for SLE

ANA (most common IF patterns: homogeneous, peripheral)

99%    

ssDNA 70% 90% A Denatured DNA Possible risk for SLE in DLE patients; also seen in RA, DM/PM, MCTD, SSc, SjS, localized scleroderma

C1q 60% C1q component of complement

Severe SLE, hypocomplementemic urticarial vasculitis syndrome

PCNA 50% A component of multiprotein complexes involved in cell proliferation

−

U1RNP 50% Splicesome RNP Overlapping features with other CTD; MCTD (100%)

Ro (SS-A) 50% hYRNP (quality control function for misfolded RNA molecules)

SCLE (75–90%), neonatal LE/congenital heart block (99%), SCLE–SjS overlap, SjS

Histones 40% 70% A Histones Drug-induced SLE, RA

Cardiolipin 50% Cardiolipin, a negatively charged phospholipid

Recurrent spontaneous abortions, thrombocytopenia, and hypercoagulable state in SLE (cutaneous manifestations include livedo reticularis, leg ulcers, acral infarction/ulceration, hemorrhagic cutaneous necrosis); clinical associations strongest with IgG class of anticardiolipin

b2 glycoprotein I 25% An important cofactor for cardiolipin in cardiolipin autoantibody assays

Relatively high risk of thrombosis in SLE and primary antiphospholipid antibody syndrome

Rheumatoid factor 25% Fc portion of IgG  

La (SS-B) 20% hYRNP SCLE (30–40%), SCLE–SjS overlap, primary SjS (20%)

Ku 10% DNA end-binding repair protein complex

Overlap with other CTD such as DM/PM, SSc

Alpha-fodrin 10% An actin-binding protein found at the periphery of chromaffin cells that may be involved in secretion

SjS