1b// Lupus and Related Autoimmune Connective Tissue Diseases Flashcards
(62 cards)
What are the autoimmune connective tissue disorders?
Systemic Lupus Erythematosus (SLE)
Sjogren’s syndrome
Systemic sclerosis (scleroderma)
Autoimmune Inflammatory muscle disease
*overlap syndromes can occur, especially in children
*NB term “connective tissue disorder” not entirely accurate – often involvement of other organs ‘Multisystem autoimmune rheumatic diseases’ might be better
Give an overview of RA.
What is ankylosing spondylitis?
Chronic spinal inflammation that can result in spinal fusion and deformity
Where is the site of inflammation for ankylosing spondylitis? And what are the antibodies involved?
Site of inflammation includes the enthesis
no autoantibodies
What are the 4 seronegative inflammatory arthritis?
Ankylosing spondylitis
Reactive Arthritis
Arthritis associated with psoriasis (psoriatic arthritis)
Arthritis associated with gastrointestinal inflammation
(enteropathic arthritis)
What is SLE?
Systemic lupus erythematosus (SLE)
Autoimmune disease involving disturbance of both innate and adaptive immune systems
What is the v basic pathogenesis of SLE?
Autoantibodies (antibodies directed against self antigens)
-antibodies to nuclear components
Antibody-antigen (immune complexes) & other mechanisms -> chronic tissue inflammation:
Multi-site inflammation but particularly the joints, skin and kidney
What are the 3 general points of autoimmune connective tissue disorders?
1) Arthralgia and arthritis is typically non-erosive (unlike rheumatoid)
2) Serum autoantibodies are characteristic and…
- Useful diagnostically
- Some correlate with disease activity
- May be directly pathogenic
3) Raynaud’s phenomenon is common in these conditions
What is Raynaud’s phenomenon?
Intermittent vasospasm of digits
Usually triggered by cold exposure
Typical triphasic colour changes
WHITE -> Vasospasm leads to blanching of digit
BLUE -> Cyanosis as static venous blood deoxygenates
RED -> Reactive hyperaemia
What does severe Raynaud’s phenomenon lead to?
Severe Raynaud’s -> tissue ischaemia, ulcers and necrosis
What is the epidemiology of SLE?
What are the skin and mucosal clinical features of SLE?
Malar rash – erythema that spares the nasolabial fold Photosensitive rash
Mouth ulcers
Hair loss
What are the vascular clinical features of SLE?
Raynaud’s
What are the MSK clinical features of SLE?
Arthralgia and sometimes arthritis (usually non-erosive)
What are the internal organs clinical features of SLE?
Serositis (pericarditis, pleuritis, less commonly peritonitis) Renal disease – glomerulonephritis (‘lupus nephritis’) Cerebral disease – ‘cerebral lupus’ e.g. psychosis Myocarditis
What are the haematological clinical features of SLE?
autoimmune thrombocytopenia (low platelets) haemolytic anaemia
Lymphopenia
What are “other” clinical features of SLE? (2)
Lymphadenopathy
Fever in absence of infection
What antibody is a hallmark of SLE?
ANA anti-nuclear antibodies
In who is ANA found?
Found in all SLE patients
Negative ANA effectively rules out SLE
However, ANA is not specific for lupus;
- may be seen in other autoimmune diseases, infection or even healthy people
What will the lab do for ANA?
Lab will do further more specific autoAb tests to identify what ANA are reacting to…
- Anti-ds-DNA antibodies
- Anti-Ro
- Anti-La
- Anti-centromere
- Anti-Smith (Sm)
- Anti-RNP
- Anti-Scl-70
Apart from ANA, what antibody can be found in SLE patients?
As well as ANA, some SLE patients have antibodies directed to phospholipids on cell membrane
What are APLs associated with risk wise?
APL are associated with ↑ risk of:
1) Thrombosis
arterial (e.g. stroke)
venous (e.g. deep vein thrombosis, DVT)
2) Pregnancy loss (miscarriage)
What does persistent presence of APL lead to?
Persistent presence of APL + a clinical event = “anti-phospholipid antibody syndrome”
When can anti-phospholipid antibody syndrome occur asw?
Anti-phospholipid antibody syndrome can also occur in absence of SLE (‘primary anti-phospholipid antibody syndrome’ )
