lúpus eritematoso sistêmico (les)
DESCRIPTION
Lúpus Eritematoso Sistêmico (LES). Diagnóstico. Avaliação para LES. 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. - PowerPoint PPT PresentationTRANSCRIPT
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