SLE, APS, Raynauds Flashcards
What is the pathology of autoimmune connective tissue diseases? Which type of immune reaction is it?
body raises immune response to its own cells
Creates Antinuclear antibodies - IgG which forms complexes with the antigen (cells nucleus)
Deposition of these complexes into tissues attracts complements
Reaction with complements attracts neutrophils leading to inflammation
This inflammation is referred to as ‘immune complex mediated’
This inflammation leads to tissue damage
Type 3 hypersensitivity reaction (3 letters in SLE)
What are the two components of SLE pathogenesis?
Inflammation leading to tissue damage
Thrombosis: phospholipid antibodies
What is the disease course of SLE? What are leading causes of death?
relapsing - remitting
chronic inflammation means patients have reduced life expectancy cardiovascular disease and infection are leading causes of death
What autoantibodies are important markers of SLE? Which can be used as a marker of disease activity?
- Antinuclear Antibody - initial step (also present in autoimmune hepatitis)
- Anti-double stranded DNA (anti-dsDNA) - specific to SLE
- Anti-Smith (highly specific but not very sensitive)
Anti double stranded DNA titres can be used to monitor disease, not present in all patients however
What markers are used to monitor disease in SLE?
- ESR usually used. during active disease CRP may be normal, raised CRP may indicate underlying infection
- C3, C4 - low during active disease (formation of complexes leads to consumption of complements)
- anti-dsDNA titres - can be used for disease monitoring, not present in all patients
Besides autoantibodies and markers to monitor disease activity, which other investigations can be done in SLE?
FBC - normocytic anaemia of chronic disease
Urine analysis and urine protein: creatinine ratio to assess for Lupus nephritis
Renal biopsy: Lupus nephritis
Who does SLE tend to affect?
- females - Asian/ Afro-Caribbean
- onset 20-40 years
general features of SLE ?
tiredness
fever
lymphadenopathy
mouth ulcer
Skin features of SLE?
MALAR rash, sparing nasolabial folds
discoid rash - well demarcated rash in sun exposed areas
photosensitivity
Livedo-reticularis (mottled skin)
Raynaud’s phenomenon
non-scarring alopecia
Cardiac SLE features
Pericarditis (most common)
Myocarditis
Lung features of SLE
Pleurisy
MSK features of SLE
arthralgia
myalgia
non-erosive arthritis
Renal features of SLE
proteinurea
Glomerulonephritis (diffuse, proliferative glomerulonephritis most common)
Neuropsychiatric
Anxiety and depression
Psychosis
Seizures
How is SLE diagnosed?
SLICC and ACR criteria - ANA antibodies and a number of symptoms (at least 4)
What are important complications of SLE?
- CVS disease - chronic inflammation leading to htn CAD
- infection - as part of disease and secondary to immunosuppressants
- Anaemia of chronic disease, leucopenia, neutropenia, thrombocytopenia
- Pericarditis, Pleuritis
- Pulmonary fibroris (inflammation of lung tissue)
- Lupus nephritis - can lead to end stage renal failure
- Neuropsychiatric SLE - optic neuritis, transverse myelitis, psychosis
- Recurrent miscarriage - IUGR, Pre-eclampsia, pre-term labour
- Venous thromboembolism - anti-phospholipid syndrome
What is the management of SLE? First line immunosuppressant for mild and another that may be used? How is SLE Renal disease managed?
NSAIDs Steroids (prednisolone)
Hydroxychloroquine (first line for mild SLE)
Suncream and sun avoidance for the photosensitive the malar rash
Other immunosuppressants - methotrexate, azathioprine etc -
manage htn - steroid - immunosuppressants - aza/cyclophosphamide
Which autoantibodies can occur in 40% of patients with SLE? what is it associated with an increased risk of?
Anti-phospholipid antibodies
Associated with increased risk of venous thromboembolism
What is the pathology of the Antiphospholipid syndrome?
autoantibodies which interfere with coagulation and lead to a hypercoaguable state
What autoantibodies are associated with APS?
Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies
What is antiphospholipid syndrome associated with? 7
Venous thrombosis: DVT and PE
Arterial thrombosis: MI, Stroke, Renal thrombosis
Pregnancy complications: recurrent miscarriage, stillbirth, pre-eclampsia
Livedo reticularis
Libmann-Sacks Endocarditis - non bacterial vegetation on the valves (usually mitral) seen in SLE and APS Thrombocytopenia
Prolonged aPTT, low platelets
How do you diagnosie APS
history of thrombosis persistent autoantibodies
How do you manage APS?
Primary thromboprophylaxis - low dose aspirin
Secondary thromboprophylaxis - Warfarin INR target 2-3 (lifelong, 3-4 if recurrent)
Pregnant: LMWH + Aspirin
What is raynaud’s ?
exaggerated vasoconstriction in digital arteries and cutaneous arterioles in response to cold or emotional stress
What is the difference between Raynaud’s disease and Raynaud’s phenomenon?
Raynaud’s disease is primary
Raynaud’s phenomenom is secondary to another condition
What are causes of Raynaud’s phenomenon?
Connective tissue disease: Scleroderma, Rhumatoid arthritis, SLE
Leukaemia
Use of Vibrating tools
Drugs (OCP, ergot)
Cervical rib
How do you manage raynauds ?
all suspected cases should be referred to secondary care
1st line: CCB - nifedipine
2nd line: IV prostacyclin infusion - effect lasts weeks to months
