Connective Tissue Disease Part 1 Flashcards
What are connective tissue diseases?
Conditions associated with spontaneous overactivity of the immune system (not diseases of connective tissue)
What are some features of connective tissue disease?
Specific auto-antibodies are present, evolve over months to years sometimes leading to organ failure and death
What are some examples of connective tissue diseases?
Systemic Lupus Erythematous (SLE), Sjogren’s syndrome, dermatomyositis/polymyositis, anti-phospholipid syndrome
What is SLE?
Systemic autoimmune condition that can affect almost any part of the body
What occurs in lupus?
Immune system attacks cells and tissues resulting in inflammation and tissue damage, immune complexes form and precipitate causing further immune response
Who gets SLE?
Female to male ratio is 9:1, commonly presents in childbearing years, commoner and more severe in those of Afro-Caribbean, Hispanic American and Asian ethnicity
What does the loss of immune regulation cause?
Increased and defective apoptosis
What do necrotic cells release?
Nuclear materials, which then act as auto-antigens
How does auto-immunity arise?
Results from exposure to nuclear and cellular auto-antigens
What does the stimulation of B and T cells cause?
Production of auto-antibodies
Where do the immune complexes of antigens and auto-antibodies that form and circulate deposit in the body?
The basement membrane
What does the activation of complement cause?
Attracts leucocytes which release cytokines = perpetuates inflammation which causes necrosis and scarring
What score on the EULAR/ACR 2019 classification criteria indicates lupus?
Score > 10, ANA must be positive
What are some cutaneous features of SLE?
Subacute cutaneous or discoid lupus, acute cutaneous lupus, non-scarring alopecia, Malar rash, oral ulceration
What are the features of arthritis associated with lupus?
Synovitis or tenderness of at least 2 joints with > 30mins morning stiffness, jaccouds arthropathy
What are some neurological manifestations of lupus?
Delirium, psychosis, seizure, headache, cranial nerve disorder
What are the manifestations of serositis associated with SLE?
Pleural/pericardial effusions, acute pericarditis
What are some haematological and renal features of lupus?
Haematological = leucopenia, thrombocytopenia, haemolytic anaemia, lymphadenopathy Renal = proteinuria > 0.5g in 24hrs, biopsy proven nephritis, red cell casts
What are some antibodies associated with SLE?
Anti-nuclear antibody (ANA), Anti-double stranded DNA antibody (dsDNA), anti-phospholipid antibodies (APLS),
Anti-Ro antibody, Anti Sm antibody
What are some features of anti-nuclear antibody (ANA)?
Positive in a titre 1:80 in almost all SLE patients
What are some features of anti-double stranded DNA antibody (dsDNA)?
Present in 60% of patients, specific for SLE, titre correlates with disease activity, associated with lupus nephritis, most important antibody for diagnosing lupus
What are some features of antiphospholipid antibodies (APLS)?
Lupus anticoagulant/anti-cardiolipin antibodies/anti-beta2glycoprotein antibodies, associated with venous/arterial thrombosis and recurrent miscarriage, livedo reticularis
What are some features of complement in regards to its relationship with SLE?
Activation and consumption is related to disease activity, particular association with renal and haematological disease, C3 and C4
What are some features of anti-Ro antibody and anti-Sm antibody?
Anti-Ro = can be associated with neonatal lupus and congenital heart block Anti-Sm = highly specific for lupus
What is the treatment for lupus in all patients?
Sun protection measures, hydroxychloroquine, minimise steroid use, monitor disease activity using SLEDAI score
What should be prescribed for patients positive for antiphospholipid antibody?
Anticoagulants/antiplatelets
