Connective tissue diseases Flashcards
(50 cards)
Give examples for seronegative arthritis
Ankylosing spondylitis
Reactive Arthritis (Reiters syndrome)
Arthritis associated with psoriasis (psoriatic arthritis)
Arthritis associated with gastrointestinal inflammation (enteropathic synovitis)
What is SLE? What tissues does it affect and what autoantibodies is it associated with?
Chronic tissue inflammation in the presence of antibodies directed against self antigens
Multi-site inflammation but particularly the joints, skin and kidney
Associated with autoantibodies:
Antinuclear antibodies
Anti-double stranded DNA antibodies
Anti-phospholipid antibodies
What type of arthritis/ arthralgia can be seen in connective tissue disorders?
Non- erosive
Significance of serum auto-antibodies in connective tissue disorders?
May aid diagnosis
Correlate with disease activity
May be directly pathogenic
Name a common phenomenon seen in connective tissue disorders. What happens in this?
Does this phenomenon always indicate a connective tissue disorder
Raynaud’s phenomenon- Intermittent vasospasm of digits on exposure to cold
Typical colour changes – white to blue to red
Vasospasm leads to blanching of digit
Cyanosis as static venous blood deoxygenates
Reactive hyperaemia
Does NOT always indicated connective tissue disorder- most commonly an isolated and benign condition (‘Primary Raynaud’s phenomenon’)
Give examples for connective tissue disorders
Systemic Lupus Erythematosus (SLE)
Sjögren’s syndrome
Autoimmune inflammatory muscle disease: Polymyositis, dermatomyositis
Systemic sclerosis (scleroderma): Diffuse cutaneous, limited cutaneous
Overlap syndromes
What demographic is SLE commonly diagnosed in?
Females aged between 15-45
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’)- see IgG, immune complexes, inflammatory cells (monoctyes, macrophages), complement components
Cerebral disease – ‘cerebral lupus’ e.g. psychosis
SLE pathgenesis
Incompletely understood, current paradigm is as follows
Key auto-antibodies in osteoarthritis
None
Key auto-antibodies in reactive arthritis
None
Key auto-antibodies in gout
None
Key auto-antibodies in ankylosing spondylitis
None
Key auto-antibodies in systemic vasculitis
Anti nuclear cytoplasmic antibodies (ANCA)
Key auto-antibodies in rheumatoid arthritis
Rheumatoid Factor
Anti-cyclic citrullinated peptide antibody
also termed antibodies to citrullinated peptide antigens (ACPA)
Key auto-antibodies in SLE
Antinuclear antibodies (ANA)
Anti-double stranded DNA antibodies (anti-dsDNA)
Anti-phospholipid antibodies a.k.a anti-cardolipin antibodies
Give examples for different types of anti-nuclear autoantibodies (ANA)
Anti-Ro
Anti-La
Anti-centromere
Anti-Sm
Anti-RNP
Anti-ds-DNA antibodies
Anti-Scl-70
Cytoplasmic antibodies include:
Anti-tRNA synthetase antibodies
Anti-ribosomal P antibodies
Significance of ANA
Seen in all SLE cases
Not specific for SLE
Significance of anti-dsDNA
Specific for SLE
Serum level of antibody correlates with disease activity
Significance of anti-Sm antobodies
What is the antigen to these antobodies
Specific for SLE
Serum level of antibody does NOT correlates with disease activity
antigen is ribonucleoprotein
Significance of anti-phospholipid antibodies (a.k.a. anti-cardolipin antibodies)
associated with risk of arterial and venous thrombosis in SLE
may also occur in absence of SLE in what is termed the ‘primary anti-phospholipid antibody syndrome
Significance of Anti-Ro antibodies and anti-La antibodies (antigen is ribonucleoprotein for both btw)
Secondary Sjögren’s syndrome
Neonatal lupus syndrome (transient rash in neonate, permanent heart block)
Significance of Anti-ribosomal P antibodies
Cerebral lupus
SLE investigations
Inflammation:
high ESR but C-reactive protein is typically normal unless infection or serositis/arthritis
Haematology:
Haemolytic anaemia, Lymphopenia, Thrombocytopenia
Renal:
very important to measure urine protein (most commonly urine protein:creatinine ratio [uPCR])
look at albumin
Immunological:
Antinuclear antibodies
Anti-double-stranded DNA antibodies - highly specific, correlate with disease activity
Complement consumption – e.g. low C4 and C3
Clotting – antiphospholipid antibodies
Lupus anticoagulant and anti-cardiolipin antibodies
In treated patients SOME changes may reflect ADVERSE REACTIONS TO MEDICATION
e.g. abnormal liver function (‘transaminitis’) or fall in neutrophil count (neutropenia)
