Systemic Lupus Erythematosus Flashcards
What type of disease is systemic Lupus Erythematosus
Chronic autoimmune
Describe the epidemiology of systemic Lupus erythematosus
M:F = 1:9
15-40
Afro-Caribbean, Asian, Chinese
What does systemic Lupus erythematosus
Principally the joints and skin
Lungs, kidneys, haematology
Which genes are associated with systemic Lupus erythematosus
Multiple genes implicated Complement deficiency e.g. C1q and C3 Fc receptors, IRF5, CTLA4, MHC class II HLA genes
Describe the presentation of systemic Lupus Erythematosus
Malaise Fatigue Fever Weight loss Lymphadenopathy
What are the specific features of systemic Lupus erythematosus
Butterfly rash
Alopecia
Arthralgia Raynaud’s phenomenon
What are some features of systemic Lupus erythematosus
Inflammation od kidney, CNS, heart, lungs
Accelerated atherosclerosis
vasculitis
Describe the pathogenesis of systemic Lupus erythematosus
- Genetic predisposition
- Environmental triggers
- Innate immune system activation
- Adaptive immune system activation
- Immune complexes
- (clinical disease onset)
- Aberrant amplification pathway
- Irreversible tissue damage
What are the laboratory tests done for Systemic Lupus Erythematosus
Antinuclear antibodies Anti-dsDNA and Sm Anti-Ro and/or La Haemotology Renal function
Describe the use of antinuclear antibodies in systemic Lupus Erythematosus
Relatively non-specific
Pattern is important
Describe the haematology of Systemic Lupus Erythematosus
Lymphopaenia Normochromic anaemia Leukopaenia AIHA Thrombocytopaenia
what are the renal function tests of Systemic Lupus Erythematosus
Proteinuria
Haematuria
Active urinary sediment
how is the disease severity of Systemic Lupus Erythematosus assessed
Identify Pattern of organ involvement
Monitor function of affected organs
Identify pattern of autoantibodies expressed
What are the clinical features that give signs of Systemic Lupus Erythematosus activity
Weight loss Fatigue Malaise Alopecia Rash
What are the lab markers that give signs of Systemic Lupus Erythematosus
ESR
Increased complement consumption
Increase anti-dsDNA
Other Antibodies
Describe mild Systemic Lupus Erythematosus
Joint +/- skin involvement
Describe moderate Systemic Lupus Erythematosus
Inflammation of other organs
Pleuritis
Pericarditis
Mild nephritis
Describe severe Systemic Lupus Erythematosus
Severe inflammation in vital organs Severe nephritis CNS disease Pulmonary disease Cardiac involvement AIHA, thrombocytopenia, TTP
What is the treatment for mild Systemic Lupus Erythematosus and what is monitored
Paracetamol +/- NSAID (Monitor renal function)
Hydroxychloroquine
Topical corticosteroids
What is the treatment for moderate Systemic Lupus Erythematosus
High dose corticosteroids
IV methylprednisolone
What is the treatment for severe Systemic Lupus Erythematosus
Azathioprine
Cyclophosphamide
What is the prognosis of Systemic Lupus Erythematosus
15 years:
no nephritis = 85%
nephritis = 60%
Prognosis worse if black, male + low socioeconomic status
Describe the bimodal mortality pattern of Systemic Lupus Erythematosus
Early
Active lupus (renal failure, CNS disease)
Infection
Late
Myocardial infarction
Describe the pathogenesis/pathophysiology of autoantibody formation in SLE
- Abnormal clearance of apoptotic cell material
- Dendritic cell uptake of auto antigens and activation of B cells
- B cell Ig class switching and affinity maturation
- IgG autoantibodies
- Immune complexes
- Complement activation, cytokine generation e.t.c
