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Reumatologia > T3 - Lupus Eritematoso Sistémico e Síndroma do Anticorpo Antifosfolipídico > Flashcards

T3 - Lupus Eritematoso Sistémico e Síndroma do Anticorpo Antifosfolipídico Flashcards

1
Q

Definição de LES?

A

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

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2
Q

Epidemiologia de LES?

A

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

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3
Q

Etiologia de LES?

A

Genetic factors
Environmental factors
Hormonal factors

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4
Q

Patogénese de LES?

A

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

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5
Q

Critérios de Classificação de LES?

A

ACR e SLICC

No mínimo 4 critérios , 1 imunológico e outro clínico

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6
Q

Exemplos de critérios clínicos de LES?

A 
Lupus cutaneo cronico ou agudo
Ulveras nasais ou orais
Alopecia nao cicatrizante
Artrite
Serosite
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7
Q

Exemplos de critérios imunológicos de LES?

A

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

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8
Q

Achados clínicos constitucionais de LES?

A

Febre
Perda de peso
Fadiga
Anorexia

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9
Q

Manifestações mucocutâneas específicas de LES?

A

Rash malar, Rash difuso, lesões bulhosas

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10
Q

Manifestações mucocutâneas não específicas de LES?

A 
Photosensitivity 
Alopecia (non-scarring)
Vasculitis
Periungal erythema
Raynaud phenomenon
Oral/Nasal ulcers
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11
Q

Manifestações musculoesqueléticas de LES?

A

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)

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12
Q

Diferenças entre artrite de LES e AR?

A

Menos inflamatórias, não deformantes e não erosivas.

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13
Q

Outras manifestações musculoesqueléticas de LES?

A

Tenosynovitis
Myositis
Avascular bone necrosis

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14
Q

Manifestações renais de LES?

A 
Hypertension – new onset
Nephrotic syndrome
Renal failure
Proteinuria
Haematuria
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15
Q

Confirmação de Nefrite Lúpica?

A

Renal biopsy

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16
Q

Manifestações Neuropsiquiátricas de LES?

A

Very heterogeneous

17
Q

Manifestações Cardiovasculares de LES?

A 
Pericarditis
Pericardial Infusion
Myocarditis
Cardiovascular Disease (accelerated, premature atherosclerosis)
Endocarditis
18
Q

Manifestações Pleuropulmonares de LES?

A 
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
19
Q

Manifestações Hematológicas de LES?

A 
Lymphadenopathy (40%)
Splenomegaly (10-45%)
Anaemia - normocrómica normocítica
Leucopenia (30-40%)
Trombocitopenia
20
Q

Manifestações Gastrointestinais de LES?

A

Mesenteric vasculitis
Pancreatitis
Hepatitis

21
Q

Manifestações Oftálmicas de LES?

A

Vasculitis of retinal artery

Corneal and conjunctiva involvement

22
Q

Diagnostic workup in LES?

A 
Full blood count
Urea, creatinine, ionogram
Liver function tests
Inflammatory markers (CRP, ESR)
CK, DHL
Coagulation tests
Urinalysis
23
Q

Antibodies in Diagnostic workup of LES?

A 
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
24
Q

Gravidez e LES?

A

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

25
Q

Síndrome Antifosfolipídico?

A

Vascular thrombosis
Pregnancy morbidity

Lupus anticoagulant positivity
Anticardiolipin antibody

26
Q

Complicações da gravidez em LES?

A

Miscarriage
Stillbirth
Premature delivery
Intrauterine growth restriction

27
Q

Lúpus neo-natal?

A

Anticorpos Anti-SSa e SSb ultrapassam a placenta.

Possível bloqueio completo auriculo-ventricular.

28
Q

Lupus Juvenil?

A

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

29
Q

SLE in the elderly?

A

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

30
Q

Drug-Induced Lupus?

A

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

31
Q

Tratamento de LES?

A

General measures (Sun avoidance)
Immunization
Bone health

32
Q

Tratamento Farmacológico de LES?

A 
Corticosteroids
Hydroxychloroquine (Atenção MACULOPATIA)
Cyclophosphamide (severe disease)
Azathioprine
Mycophenolate mofetil
Methotrexate
33
Q

Terapias biológicas de LES?

A

Rituximab

Belilmumab

34
Q

How to monitor LES?

A

Disease Activity Indices

Chronicity and Damage Index

Reumatologia (12 decks)

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