T3 - Lupus Eritematoso Sistémico e Síndroma do Anticorpo Antifosfolipídico

Question 1

Definição de LES?

Answer 1

Multisystem chronic autoimmune disorder
Broad-spectrum of clinical presentations
A waxing and waning course
Variable prognosis

Question 2

Epidemiologia de LES?

Answer 2

Women are affected up to nine times more frequently than men

Peak age of onset among women is between the late teens and early 40s

Question 3

Etiologia de LES?

Answer 3

Genetic factors
Environmental factors
Hormonal factors

Question 4

Patogénese de LES?

Answer 4

Irreversible breakdown of immunological tolerance manifested by aberrant immune responses against endogenous nuclear and other self-antigens and deposition of immune complexes.

Question 5

Critérios de Classificação de LES?

Answer 5

ACR e SLICC

No mínimo 4 critérios , 1 imunológico e outro clínico

Question 6

Exemplos de critérios clínicos de LES?

Answer 6

Lupus cutaneo cronico ou agudo
Ulveras nasais ou orais
Alopecia nao cicatrizante
Artrite
Serosite

Question 7

Exemplos de critérios imunológicos de LES?

Answer 7

ANA
Anti-dsDNA
Anti-Sm
Low complement (C3, C4 e CH50)

Question 8

Achados clínicos constitucionais de LES?

Answer 8

Febre
Perda de peso
Fadiga
Anorexia

Question 9

Manifestações mucocutâneas específicas de LES?

Answer 9

Rash malar, Rash difuso, lesões bulhosas

Question 10

Manifestações mucocutâneas não específicas de LES?

Answer 10

Photosensitivity 
Alopecia (non-scarring)
Vasculitis
Periungal erythema
Raynaud phenomenon
Oral/Nasal ulcers

Question 11

Manifestações musculoesqueléticas de LES?

Answer 11

Non-erosive, non-deforming arthralgias/arthritis primarily affecting the small joints of the hands, wrists and knees
Jaccoud arthropathy
Erosive arthropathy overlapping rheumatoid arthritis features (ruphus)

Question 12

Diferenças entre artrite de LES e AR?

Answer 12

Menos inflamatórias, não deformantes e não erosivas.

Question 13

Outras manifestações musculoesqueléticas de LES?

Answer 13

Tenosynovitis
Myositis
Avascular bone necrosis

Question 14

Manifestações renais de LES?

Answer 14

Hypertension – new onset
Nephrotic syndrome
Renal failure
Proteinuria
Haematuria

Question 15

Confirmação de Nefrite Lúpica?

Answer 15

Renal biopsy

Question 16

Manifestações Neuropsiquiátricas de LES?

Answer 16

Very heterogeneous

Question 17

Manifestações Cardiovasculares de LES?

Answer 17

Pericarditis
Pericardial Infusion
Myocarditis
Cardiovascular Disease (accelerated, premature atherosclerosis)
Endocarditis

Question 18

Manifestações Pleuropulmonares de LES?

Answer 18

Pleuritic chest pain (45-60%)
Pleural effusion (50%)
Acute pneumonitis (1-4%)
Interstitial lung disease (3-13%)
Diffuse alveolar hemorrhage
Shrinking-lung syndrome
Pulmonary hypertension

Question 19

Manifestações Hematológicas de LES?

Answer 19

Lymphadenopathy (40%)
Splenomegaly (10-45%)
Anaemia - normocrómica normocítica
Leucopenia (30-40%)
Trombocitopenia

Question 20

Manifestações Gastrointestinais de LES?

Answer 20

Mesenteric vasculitis
Pancreatitis
Hepatitis

Question 21

Manifestações Oftálmicas de LES?

Answer 21

Vasculitis of retinal artery

Corneal and conjunctiva involvement

Question 22

Diagnostic workup in LES?

Answer 22

Full blood count
Urea, creatinine, ionogram
Liver function tests
Inflammatory markers (CRP, ESR)
CK, DHL
Coagulation tests
Urinalysis

Question 23

Antibodies in Diagnostic workup of LES?

Answer 23

Antinuclear antibodies
Anti-dsDNA
Complement (C1q, C3, C4)
Lupus anticoagulant
Anticardiolipin
Direct Coombs test
Anti-ENA (Anti-SSa, Anti-SSb, Anti-Sm, Anti-RNP)
Anti-ribosomal
Anti-Histone

Question 24

Gravidez e LES?

Answer 24

Pregnancy may increase lupus activity but flares are usually mild
Pregnancy outcome is optimal when in clinical remission for 6-12 months, renal function is normal and appropriate drugs
Patients with lupus nephritis and antiphospholipid antibodies: ↑ pre-eclampsia

Question 25

Síndrome Antifosfolipídico?

Answer 25

Vascular thrombosis
Pregnancy morbidity

Lupus anticoagulant positivity
Anticardiolipin antibody

Question 26

Complicações da gravidez em LES?

Answer 26

Miscarriage
Stillbirth
Premature delivery
Intrauterine growth restriction

Question 27

Lúpus neo-natal?

Answer 27

Anticorpos Anti-SSa e SSb ultrapassam a placenta.

Possível bloqueio completo auriculo-ventricular.

Question 28

Lupus Juvenil?

Answer 28

15-20% of SLE cases
More aggressive disease in children than in adults
↑ renal and neurologic involvement
↓ pleuritis, sicca symptoms

Question 29

SLE in the elderly?

Answer 29

10-20% of SLE cases
Delayed diagnosis and less common occurrence of severe manifestations
↑ serositis, pulmonary involvement, sicca symptoms, and musculoskeletal manifestations
↓ malar rash, photosensitivity, purpura, alopecia, Raynaud’s phenomenon, renal and central nervous system (CNS) involvement

Question 30

Drug-Induced Lupus?

Answer 30

Patient with no previous diagnosis or history of SLE
Develops ANA positivity and at least one clinical feature of SLE after drug exposure
Typical clinical features
Anti-histone antibodies > 95%
Hypocomplementaemia and Anti-dsDNA antibodies rare

Question 31

Tratamento de LES?

Answer 31

General measures (Sun avoidance)
Immunization
Bone health

Question 32

Tratamento Farmacológico de LES?

Answer 32

Corticosteroids
Hydroxychloroquine (Atenção MACULOPATIA)
Cyclophosphamide (severe disease)
Azathioprine
Mycophenolate mofetil
Methotrexate

Question 33

Terapias biológicas de LES?

Answer 33

Rituximab

Belilmumab

Question 34

How to monitor LES?

Answer 34

Disease Activity Indices

Chronicity and Damage Index