Lupus and autoimmune connective tissue disease Flashcards
What are the 4 dif autoimmune connective tissue disorders
SLE
Sjorgen’s syndrome
Systemic sclerosis (scleroderma)
Autoimmune inflammatory muscle disease
What is overlap syndrome
Patients who exhibit characteristics of multiple autoimmune connective tissue disorders
- mainly children
What is SLE?
Systemic Lupus Erythematosus:
chronic tissue inflammation from autoantibodies
Involves both innate and adaptive immune systems
What type of antibodies are formed in SLE and what does this cause
Autoantibodies against nuclear components
Causes multi-site inflammation esp. jts, skin, kidney
As basically all cells have a nucelus
Where does SLE present?
Multi-site but mostly skin (rash), joints (arthritis), kidney
What are the key symptoms of connective tissue disorders?
Arthralgia, Arthritis, Raynaud’s Phenomenon
What is the difference between arthralgia and arthritis?
Arthralgia - joint pain no swelling/clinical signs
Arthritis - joint pain with swelling
What is Raynaud’s phenomenon?
Vasospasm of digits with cold exposure
What are the triphasic colour changes seen in Raynaud’s
WHITE - contraction of blood vessels .: lack of blood to digit
BLUE - cyanosis due to deoxygenation of venous blood
RED - when circulation opens up again and blood returns (painful)
Consequences of severe Raynaud’s
tissue ischaemia
ulcers
necrosis
What is the rash associated with SLE?
Photosensitive malar rash - erythema on cheeks and nose sparing nasolabial fold
What are the systemic clinical manifestations of SLE?
Malar rash (butterfly)
Mouth ulcers
Alopecia
Raynaud’s
Serositis
Arthralgia/Arthritis
Glomerulonephritis
Haemolytic anaemia
Cerebral disease
Lymphadenopathy
fever w/o infection
What autoantibodies are present in SLE?
Anti-nuclear antibodies (ANA) e.g. (Anti-Ro), (Anti-La)
Anti-double stranded DNA antibodies (Anti-ds-DNA)
Anti-Phospholipid antibodies (APL)
Significance of anti-nuclear antibodies in SLE
Found in all patients w/ SLE
Non-specific for SLE
What are anti-phospholipid antibodies associated with
Thrombosis
- Arterial e.g. stroke
- Venous e.g. DVT, PE
Pregnancy loss, miscarriage
Significance of Anti-dsDNA in SLE
Specific for SLE
Serum levels correlate with disease activity
Significance of Anti-Sm in SLE
Specific for SLE
No correlation with serum levels
What are the key autoantibodies in rheumatoid arthritis?
Rheumatoid Factor,
Anti-cyclic citrullinated peptide antibody (Anti-CCP)
SLE immunopathogenesis in innate and adaptive immunity
Innate immunity - Overactive Type 1 interferon pathway,
- Complement pathway abnormalities
Adaptive immunity - Autoreactive B/T cells
What is the theorised pathogenesis of SLE?
Apoptosis translocates nuclear antigens to cell surface.
In SLE, impaired clearance of apoptotic cells leads to prolonged presentation of antigens to immune cells - B cell autoimmunity + complement activation, leading to tissue damage
What will you see in SLE after blood investigating inflammation?
High ESR, Normal CRP (remember this! in rheumatoid this is high)
unless severe arthritis/serositis
What are the haematological manifestations of SLE?
Haemolytic anaemia, Lymphopenia, Thrombocytopenia
What immunological investigations can you do for SLE?
Antinuclear antibodies, Anti ds DNA antibodies, Low complement (C3 and C4)
What renal tests do you do in SLE?
Urine Albumin:Creatinine Ratio (for glomerulonephritis in SLE)
Urinalysis
Creatinine via U&E
Kidney biopsy w/ persistent proteinuria
What is seen in active SLE
Low complement -> C3/4
High Anti-dsDNA
Aim of SLE management
Remission/low disease activity
Prevention of flares
Immunosuppressant side effects in SLE
Preference to immunosuppressants due to:
infection
osteoporosis
Avascular necrosis (AVN) affecting hips due to APL
What is the primary lupus treatment?
Hydroxycholoroquine (DMARDs)
What are other treatments that can be initiated in SLE?
Immunosuppressants: Methotrexate, Azathioprine,
B cell monoclonal AB: Rituximab
Considerations of SLE and pregnancy
Antiphospholipid AB associated w/ miscarriage (red. risk with aspirin/heparin)
pregnancy worsens renal function
Anti-Ro can cause fetal heartblock
Some drugs cause fetal malformation e.g. warfarin/methotrexate
What are the skin signs of Dermatomyositis?
Lilac heliotrope rash on eyelids or malar regions,
gottron’s papules (red raised lesions),
