SLE, Systemic manifestations and RA Flashcards
what is rheumatoid arthritis
Chronic joint inflammation (synovium) can result in joint damage
Associated with autoantibodies (RF and Anti-CCP)
Which autoantibodies are associated with rheumatoid arthritis
Rheumatoid factor
Anti-CCP
What is ankylosing spondylitis
Chronic spinal inflammation that can result in spinal fusion and deformity
Site of inflammation includes the enthesis
No autoantibodies (‘seronegative’)
What are the consequences with ankylosing spondylitis
Spinal fusion
How is spinal fusion evidently seen on a radiograph
Bamboo spine
examples of seronegative spondyloarthopathies
Ankylosing spondylitis
Reactive arthritis (Reiter’s Syndrome)
Psoriatic arthritis
Arthritis associated with gastrointestinal inflammation (enterohepatic synovitis)
main features of SLE
Chronic tissue inflammation in the presence of antibodies directed against self antigens
Multi-site inflammation but particularly the joints, skin and kidney
key autoantibodies in SLE
Antinuclear antibodies
Anti-double stranded DNA antibodies
Anti-phospholipid antibodies
arthralgia
Pain in joints
Tenderness but not obvious inflammation
Common in connective tissue disorders
What is 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
main manifestations of SLE
Malar rash – butterfly, photosensitive
Mouth ulcers
Hair loss
Raynaud’s phenomenon
Arthralgia and sometimes arthritis
Serositis (pericarditis, pleuritis, less commonly peritonitis)
Renal disease – glomerulonephritis (‘lupus nephritis’)
Cerebral disease – ‘cerebral lupus’ e.g. psychosis
demographic of SLE
females 15-45
Which rheumatoid conditions are seronegative
OA
Reactive arthritis
Gout
Ankylosing Spondylitis
What type of hypersensitivity is associated with SLE
Type 3 hypersensitivity -due to formation of immune complexes
three stages of Raynaud’s
1) Vasospasm
2) Cyanosis
3) Reactive hyperaemia
key investigations for SLE
ESR
CRP is usually normal
Autoantibodies
Antiphospholipid antibodies
haematological findings SLE
Haemolytic anaemia, Lymphopenia, Thrombocytopenia
What is the main clinical risk in a neonate with neonatal lupus syndrome
Permanent heart block - conduct ECG
renal investigations performed in patients with SLE
Measure urine protein: Creatinine ratio
Albumin
eGFR
How can we measure disease activity in SLE
Immune complexes
Unwell lupus patient has LOW complement C3 and C4 levels and HIGH levels of anti-ds-DNA antibodies
aim of SLE treatment
aims at remission or low disease activity and prevention of flares
What should patients with SLE be assessed for
antiphospholipid antibody status
infectious and cardiovascular diseases risk profile
What is Sjorgren’s syndrome
Autoimmune exocrinopathy
lymphocytic infiltration of especially exocrine glands and sometimes of other organs (extra-glandular involvement)
non-erosive arthritis and raynauds common
What is inflammatory muscle disease
Proximal muscle weakness due to autoimmune-mediated inflammation either with (dermatomyositis) or without (polymyositis) a rash
associated with malignancy and pulmonary fibrosis
skin changes in dermatomyositis
heliotrope rash on eyelids, malar regionm nsao-labial folds
red or purple lesions on knuckles - gottrons papules
subcutaneous calcinosis
mechanics hands - cracking over finger pads
Which serum markers are elevated in dermatomyositis
Creatine phosphokinase
symptoms of sjorgens
dey eyes
dry mouth
parotid gland enlargement
What is systemic sclerosis
Thickened skin with Raynaud’s phenomenon
Dermal fibrosis, cutaneous calcinosis and telangiectasia
Skin changes may be limited or diffuse
What is overlap syndrome
When features of more than 1 connective tissue disorder are present e.g. SLE and inflammatory muscle disease we can use the term overlap syndrome
investigation findings inflammatory muscle disease
elevated CPK
abnormal electromyography
abnormal muscle biopsy
manifestations of diffuse systemic sclerosis
Fibrotic skin proximal to elbows or knees (excluding face and neck)
Anti-topoisomerase-I (Anti-Scl-70) antibodies.
Pulmonary fibrosis, renal (thrombotic microangiopathy) involvement.
Short history of Raynaud’s phenomenon
manifestations of limited systemic sclerosis
Fibrotic skin hands, forearms, feet, neck and face
Anti-centromere antibodies
Pulmonary hypertension
Long history of Raynaud’s phenomenon
Which antibodies are associated with CREST and limited systemic sclerosis
Anti-centromere antibodies
CREST
sub-type of limited systemic sclerosis:
Calcinosis, Raynaud’s phenomenon, Oesophageal dysmotility, Sclerodactyly, Telangiectasia
autoantibodies are associated with polymyositis
Anti-tRNA transferase
auto-antibody is associated with mixed connective tissue disorder
Anti-U1 RNP antibody
auto-antibody is associated with diffuse systemic sclerosis
Anti-Scl-70
anti nuclear antibodies
seen in all SLE
not specific for SLE
anti double stranded DNA antibodies
specific for SLE
levels indicate disease activity
anti phospholipid antibodies
associated with risk of arterial and venous thrombosis in SLE
can occur without SLE - primary antiphospholipid antibody syndrome
investigations for synovitis
ultrasound - thickening, increased blood flow
treatment of rheumatoid arthritis
steroids short term for the inflammation DMARDs - methotrexate and hydrochloriquine if DMARDs dont work: anti TNFa, anti IL-6, B cell biologic JaK inhibitors
clinical signs for checking for psoriatic arthritis
nail signs - pitting, thickening under nail, oncholysis (separation)
