4 Systemic Lupus Erythematosus Flashcards
SLE = systemic autoimmune disease
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SLE is more common in males / females
there is increased/ decreased risk in afro caribbean or asian
onset is usually young / old
SLE is more common in females
there is increased risk in afro caribbean or asian
onset is usually young
Describe presentations of SLE?
- malaise
- fatigue
- fever
- weight loss
- lymphadenopathy
- butterfly rash
- arthralgia (joint pain)
- long history of raynaud’s phenomenon
SLE Pathogenesis involves both environmental triggers + polygenic predisposition
- UV light induces apoptosis
- causes release of nuclear antigens
Describe the process of autoantibody formation in SLE ?
- UV light induces apoptosis
- causes release of nuclear antigens
- Normally, body clears dead cells in non-inflammatory way
- In SLE –> deficient clearance of apoptotic cells + associated w/ inflammation
- Apoptotic cells linger –> expose (self) nuclear antigens on surface to hyperactive immune system/B cells –> form autoantibodies e.g. ANAs
- Autoantibodies circulate + form immune complexes
- Complexes deposit in tissues (kidney + skin) –> activate complement in tissue
What are the 3 key tests to diagnose SLE ?
- ANA (not diagnostic)
–> non specific pattern is important
• Homogenous - Abs to DNA (typical of SLE)
• Speckled - Abs to Ro, La, Sm, RNP
• Nucleolar - topoisomerase - scleroderma
• Centromere - limited cutaneous scleroderma - anti dsDNA + Sm
- -> more specific but less sensitive
- anti Ro andor La
- -> common in subacute cutaneous LE
other tests
- increased - complement consumption
For SLE, How would you assess the disease severity?
- Identify pattern of organ involvement
- Monitor function of affected organs
- Identify pattern of autoantibodies expressed
• Anti-dsDNA, anti-Sm - renal disease
• Anti-cardiolipin antibodies
Try and pre-empt severe attacks
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What are 3 groups of SLE Disease?
and how do they differ ?
• MILD
- joint +/- skin involvement
• MODERATE
- inflammation of other organs pleuritis, pericarditis, mild nephritis
• SEVERE - severe inflammation in vital organs • severe nephritis • CNS disease • pulmonary disease • cardiac involvement • AIHA, Thrombocytopaenia, TTP
How would you treat mild SLE ?
- Hydroxychloroquine for skin, but can cause retinal issues (rare)
- Topical corticosteroids for skin rash
How would you treat moderate SLE?
- 1st line is still oral GCs
- Start off with initial HIGH DOSE (to get disease into remission) then titrate down to lower dose
- High dose steroids are toxic –> may give with steroid-sparing agents to lower dose
How would you treat severe SLE?
- Azathioprine useful as a steroid-sparing drug à can lower steroid dose whilst
maintaining efficacy à fewer SEs
- Azathioprine risks neutropenia + bone marrow suppression so need regular blood + biochemistry monitoring –> more prone to infection
- Cyclophosphamide only used if SEVERE organ involvement b/c risks INFERTILITY
newer treatment:
- Mycophenolate Mofetil
What is the prognosis of SLE?
- 15-year survival: if no nephritis = 85%; if have nephritis = 60%
Prognosis for SLE = worse if black + male + low socioeconomic status
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The Bimodal Mortality Pattern of SLE:
a) in early peaks
b) in late peak
early peaks (active lupus) --> due to renal failure, CNS disease, infection
late peak
–> due to MI
What will be seen on a blood film with SLE?
- Schistocytes (fragments) – showing microangiopathic haemolytic anaemia
- Spherocytes
- Tear drop cells
- Few leukocytes
- Thrombocytopenia + fibrin strands
- Anisocytosis + poikilocytosis
what would you see in renal biopsy of SLE patient?
o Bowman’s capsule distorted, not round
o Hypercellular - more cells > in healthy glomerulus
o Mesangial proliferation
o Crescent development (arrow) - inflammatory cells that have migrated into glomerulus
o = proliferative, crescentic glomerulonephritis à will lead to renal failure
What are the 3 main disease activity biomarkers for SLE? :
ESR,
Complement C3/C4,
dsDNA Abs
SLE - ACR Criteria - 4 or more of 11 criteria
SOAP BRAIN MD
- Malar rash
- Discoid rash
- Serositis: (a) pleuritis or (b) pericarditis
- Oral ulcers
- Arthritis
- Photosensitivity
- Renal disorder e.g. proteinuria > 0.5g/24h
- Neurological disorder e.g. seizures/ pyschosis
- Haematological disorder – leucopenias/thrombocytopenias/ AHA
- Immunologic disorder e.g anti-dsDNA Abs
- Antinuclear antibody in raised titre
