Lupus and related autoimmune connective tissue diseases Flashcards
what is the term given to arthritis associated with gastrointestinal inflammation?
enteropathic synovitis)
what is systemic lupus erythematosus?
Chronic tissue inflammation in the presence of antibodies directed against self antigens
what does systemic lupus erythematosus cause?
Multi-site inflammation but particularly the joints, skin and kidney
what antibodies are associated with systemic lupus erythematosus?
antinuclear antibodies
anti-double stranded DNA antibodies
anti-phospholipid antibodies
arthralgia and arthritis is typically_____
non-erosive
what is Raynauds phenomenon?
Intermittent vasospasm of digits on exposure to cold
Typical colour changes – white to blue to red
what causes the colour change in the digits seen in Raynauds phenomenon?
Vasospasm leads to blanching of digit
Cyanosis as static venous blood deoxygenates
Reactive hyperaemia
is Raynauds phenomenon a benign or metastatic condition?
benign, most commonly isolated
what demographic has the most common diagnosis of SLE?
females aged 15-45
what are the clinical manifestations of SLE?
Malar rash – erythema that spares the nasolabial fold
Photosensitive rash
Mouth ulcers
Hair loss
Raynaud’s phenomenon
Arthralgia and sometimes arthritis
Serositis (pericarditis, pleuritis, less commonly peritonitis)
Renal disease – glomerulonephritis (‘lupus nephritis’)
Cerebral disease – ‘cerebral lupus’ e.g. psychosis
outline the pathogenesis of SLE?
incompletely understood:
1. apoptosis leads to translocation of nuclear antigens to membrane surface
2. impaired clearance of apoptotic cells results in enhanced presentation of nuclear antigens to immune cells.
3. b cell autoimmunity
4. tissue damage by antibody effector mechanisms e.g complement activation and Fc receptor engagement
are the anti-phospholipid antibodies associated with SLE only found in SLE?
may also occur in absence of SLE in what is termed the ‘primary anti-phospholipid antibody syndrome
what is the other term given to anti-phospholipid antibodies in SLE and what are they associated with?
also termed anti-cardiolipin antibodies and associated with risk of arterial and venous thrombosis in SLE
what are the key auto-antibodies associated with systemic vasculitis?
Anti-neutrophil cytoplasmic antibodies (ANCA)
If ANA is positive the clinical laboratory will perform further tests to determine which type of ANA it is – typically these include screening for:
Anti-Ro
Anti-La
Anti-centromere
Anti-Sm
Anti-RNP
Anti-ds-DNA antibodies
Anti-Scl-70
Cytoplasmic antinuclear antibodies include:
Anti-tRNA synthetase antibodies
Anti-ribosomal P antibodies
which autoantibody in SLE correlates with disease activity?
Anti-double stranded DNA antibodies (anti-dsDNA)
what autoantibody is associated with secondary Sjogrens syndrome?
Anti-Ro antibodies
antigen is ribonucleoprotein
which autoantibody is associated with neonatal lupus syndrome?
Anti-La antibodies
antigen is ribonucleoprotein
what are the symptoms of neonatal lupus syndrome?
transient rash in neonate, permanent heart block)
which autoantibodies are associated with cerebral lupus?
Anti-ribosomal P antibodies
what is seen in the haematology of a patient with SLE?
inc. ESR but CRP normal unless infection or serositis/arthritis
Haemolytic anaemia, Lymphopenia, Thrombocytopenia
what renal markers indicate SLE?
very important to measure urine protein (most commonly urine protein:creatinine ratio [uPCR])
look at albumin
what happens to C3 and C4 levels in SLE?
decrease
