Lupus, Systemic Sclerosis & Sjogren's Flashcards
Define connective tissue diseases
Conditions associated with spontaneous overactivity of the immune system
Describe the epidemiology of SLE
9:1 female to male ratio commonly presents in childbearing years, involves genetic, hormonal, immunological and environmental factors
How are auto-antibodies produced in SLE?
- Loss of immune regulation
- Increased and defective apoptosis
- Necrotic cells release nuclear material which act as auto-antigens
- Immune system cannot distinguish between ‘normal’ and ‘unwanted’ cells
- B and T cells are stimulated
- Auto-antibodies are produced
After SLE auto-antibodies are produced what happens?
- Complexes of antigens and auto-antibodies form and circulate and get deposited in the wall of blood vessels
- This activates complement which attracts leucocytes and releases cytokines
- Cytokine release causes inflammation
- Necrosis and scarring occurs
Is complement high or low during active SLE?
Low due to complex formation
Where in the body are clinical features of SLE found?
Cutaneous Joints Neurological Serositis Haematological Renal
What are the cutaneous clinical features of SLE?
Malar rash (butterfly), oral ulcers, discoid rash
What joint symptoms are present in SLE?
Synovitis, tenderness, >2 joints, >30mins morning stiffness
- Jaccouds arthropathy
What neurological symptoms can present in SLE?
Delirium, psychosis, seizures
Define serositis
Inflammation of serous tissues (heart/lungs)
What haematological symptoms can SLE present with?
Leukopenia, thrombocytopenia, haemolytic anaemia
What renal symptoms can be present in SLE?
Proteinuria, nephritis, red cell casts
What is the first test done in SLE?
Urinalysis
What antibodies can be present in SLE?
Anti-nuclear antibody Anti-double stranded DNA antibody Anti-phospholipid antibodies Anti-Ro Anti-Sm
Which antibody is most specific for SLE?
Anti-double stranded DNA
Anti-Sm (rarely present)
Name three anti-phospholipid antibodies
Lupus anticoagulant
Anti-cardiolipin antibodies
Anti -beta2glycoprotein antibodies
What is the major risk of anti-phospholipid antibodies in women?
Associated with venous and arterial thrombosis and recurrent miscarriage
What are the risks of Anti-Ro?
Associated with neonatal heart block and lupus
What should all patients with lupus be given?
Sun protection
Hydroxychloroquine
Name the scoring system used to monitor SLE activity
SLEDAI
Define mild SLE
Mild arthritis/rash SLEDAI = 6
Define moderate SLE
RA-like arthritis, cutaneous vasculitis, SLEDAI 7-12
Define severe SLE
major organ threatening disease SLEDAI >12
What is meant by refractory disease?
Failure to attain clinical remission
What is remission in terms of SLE?
SLEDAI = 0
HCQ but no steroids
What should all patients with anti-phosolipid antibodies be prescribed?
Antiplatelets/anticoagulants
What is second line for all severities of SLE after HCQ?
Glucocorticoids - route of administration/dose will vary depending on severity
In mild refractory or 1st line moderate disease after HCQ and GC what is the next possible treatment option?
Methotrexate/Azathioprine
What is given in moderate refractory SLE after HCQ and GC?
Belimumab
What is given in moderate disease fourth line?
Calcineurin Inhibitors
If calcineurin inhibitors don’t work what is next line in moderate disease?
Mycophenolate Mofetil
What are the treatment options in severe disease after HCQ and GC?
Mycophenolate mofetil (1st)
Cyclophosphamide (1st and refractory)
Rituximab (refractory)
What is Sjogren’s syndrome?
Chronic inflammatory autoimmune disorder with lymphocytic infiltration and fibrosis of exocrine glands
What are the symptoms of sjogren’s?
Dry; eyes, mouth, throat, vagina Parotid gland enlargement Joint pain Fatigue Unexplained dental caries
What causes secondary sjogren’s?
Rheumatoid arthritis
SLE
How is sjogren’s diagnosed?
- Schimers test for conjunctival dryness
- IgG and ESR
- Anti-Ro/Anti-La antibodies
What are patients with sjogren’s at increased risk of?
Lymphoma
How is sjogren’s treated?
Symptomatic treatment
NSAIDs
Hydroxychloroquine for arthralgia
Immunosuppressants in severe disease
What is systemic sclerosis?
multisystem autoimmune disease
- vasculopathy
- autoimmunity
- fibrosis
What are the two types of systemic sclerosis?
Diffuse
Limited
Describe diffuse systemic sclerosis
Whole body affected Interstitial lung disease Pulmonary hypertension (SOB) Renal crisis Extremities above and below elbows and knees
Describe limited systemic sclerosis
Calcinosis Raynaud's Oesophageal dysmotility Sclerodactyly Telangiectasia Below elbows and knees + face
What antibody is associated with each type of systemic sclerosis?
Diffuse - Anti-topoisonmersase and Anti-Scl70
Limited - Anti-centromere
How will systemic sclerosis present in the face?
small mouth, beaked nose, tight skin, telangiectasia
What GI symptoms can systemic sclerosis cause?
Dysphagia, GORD, malabsorption, fluctuation in bowel habits, small intestinal bacterial overgrowth, faecal incontinence
What cardio/resp systems can systemic sclerosis cause?
ILD, pulmonary hypertension, myocardial disease
Describe the renal problems caused by systemic sclerosis?
Scleroderma renal crisis and renal dysfunction
How does Raynaud’s present?
Blanching
Hyperaemia
Acrocyanosis
What are the risk factors for renal crisis?
- anti-RNA polymerase antibody
- steroids
How does renal crisis present?
Uncontrolled hypertension, proteinuria, worsening renal function
How is renal crisis treated?
ACE inhibitors
What is the treatment for pulmonary fibrosis?
- mycophenolate mofetil
- cyclophosphamide
- rituximab
- nitedanib (anti-fibrotic)
How is skin fibrosis treated?
Methotrexate
Mycophenolate mofetil
What is the treatment for vasculopathy?
Calcium channel blockers Prostacyclin infusion (iloprost) Endothelin receptor antagonist (bosentan)
What can pulmonary hypertension lead to?
Right heart failure - SOB on exertion
How is pulmonary hypertension treated?
PDE-5 Inhibitor
Oxygen
Endothelin receptor antagonist
