Systemic Lupus Erythematosus Flashcards
What is the definition of SLE?
Chronic multisystem autoimmune disease characterised by autoantibody production and immune complex deposition, which leads to organ inflammation and ultimately organ damage
Why is SLE defined as a multisystem disease?
Many organs are affected at same time
Is the incidence and prevalence of SLE equal in men and women?
No, incidence and prevalence is much higher in women than men
What percentage of individuals with SLE are female?
90%
What is the peak age onset range for SLE?
20-30
Is the incidence of SLE equal in non-white and white ethnic populations?
No, incidence is higher in non-white ethnic populations
Give 3 examples of ethnic populations in which SLE incidence is high?
Afro-American
Hispanic
Asian
What is the prevalence percentage of SLE in Caucasians?
0.03%
What is the percentage of prevalence of SLE in Afro-Caribbeans?
0.2%
How does SLE affect the risk of CVD mortality compared to age and gender controls?
Risk of CVD mortality increase 5x more by SLE than other age or genetic controls
What type of siblings have higher chance of developing SLE?
Monozygotic twins
What is a polymorphic gene?
Gene that has 2 or more forms when variation occurs in the population
Give one example of polymorphism that causes genetic predisposition to SLE?
Polymorphic variants at HLA gene locus
What 3 complement proteins can increase chance of SLE when they have inherited mutations?
C1q, C2,C4
What part of the immunoglobulin receptor can increase chance of SLE when affected by an inherited mutation?
FcγRIIIb
What enzyme can increase chance of SLE when affected by an inherited mutation?
TREX1 (DNA endonuclease)
What do most of the polymorphic genes of SLE express?
Regulation of immune cell function
How can poor apoptosis cause autoantibody production in SLE?
Poor apoptosis means that not all antigens are destroyed, which activates B-cells and T-cells, this stimulates autoantibody production
Autoantibodies seem to target antigens in the nucleus or in cell
In SLE, are the symptoms always the same in a patient at any given time?
No, SLE can cause flares of varying intensity, duration, frequency and can affect any organ
Give 5 examples of systemic features of SLE?
Weight loss
Arthralgia
Fever
Fatigue
Mild lymphadenopathy
What is lymphadenopathy?
Disease activity in lymph nodes
In what percentage of SLE cases does arthralgia occur?
90%
What are the 2 common symptoms in which arthralgia is presented, in SLE cases?
EMS
Tendosynovitis (inflammation of tendon sheath lining)
In SLE, how can tendosynovitis caused by arthralgia progress if untreated?
Causes tendon damage
Give 2 examples of rare symptoms in which arthralgia presents, that is caused by SLE?
Synovitis
Jaccoud’s arthropathy
What is Raynaud’s phenomenon, and explain what each colour change?
Improper blood circulation to extremities, where skin colour change indicates state of blood flow and occurs in response to cold, stress, anxiety
skin turns white when there is restricted blood flow, blue when vessels dilate, red when blood flows to extremities
In SLE cases, if examination of Raynaud’s phenomenon shows a colour change, what condition does this indicate?
Chronic ischaemia
In SLE cases, how does Raynaud’s phenomenon affect capillary nail loop patterns?
Loss of normal capillary nail loop patterns
In severe SLE cases, what structure can Raynaud’s phenomenon cause to develop on digits?
Digit ulcers
Describe the onset of secondary Raynaud’s phenomenon in SLE?
Common, early symptom of SLE, usual onset occurs in males over 25 with no family history of Raynaud’s phenomenon
Give 2 common symptoms of neural involvement in SLE?
Headaches, poor concentration
Give 5 examples of uncommon symptoms of neural involvement in SLE?
Visual hallucinations
Chorea
Lymphocytic meningitis
Transverse myelitis
Organic psychosis
What is chorea, and what does it indicate when presented in SLE cases?
Involuntary, irregular muscle movements that indicate neural involvement in SLE
Give 2 common symptoms of GI involvement in SLE cases?
Mouth ulcers
Acute pain caused by peritoneal serositis (inflammation of peritoneum/inner abdomen lining)
What are 2 severe, rare symptoms of GI involvement in SLE cases?
Mesenteric vasculitis (abdomen pain, bowel infarction/perforation)
Hepatitis
If a patient with SLE has symptoms of renal disease, what does this indicate about the state of SLE progression?
Renal disease is a hallmark of severe SLE
In severe SLE cases, what 2 tests are regularly carried out to monitor renal function?
Urinalysis
Blood pressure
Give 4 common symptoms of renal disease in severe SLE cases?
Severe haematuria
Severe proteinuria
Proliferative glomerulonephritis
Urinary casts on urine microscopy
What are urinary casts, and what can they indicate in a patient with SLE?
Urinary cast is a tube-like structure that forms in urine as the result of conditions, infections, or problems with renal function
Therefore indicates that SLE patient has renal disease
What are the 3 common symptoms of heart disease in SLE cases?
Pericarditis
Myocarditis
Libman-Sacks endocarditis
What is Libman-Sacks endocarditis, and what does it indicate when present in SLE cases?
Non-bacterial endocarditis that presents in SLE when there is cardiovascular involvement, specifically heart disease
How does arterial involvement in SLE cases cause an overall increased risk of MI and stroke?
Causes greater incidence of atherosclerosis, which causes inflammatory disease in endothelial walls, long-term steroid therapy and is associated with antiphospholipid antibodies
These increase the overall risk
Give 2 common symptoms of lung involvement in SLE cases?
Pleuritic pain due to serositis
Pleural effusions
Give 4 uncommon symptoms of lung involvement in SLE cases?
Breathlessness due to pulmonary fibrosis
atelectasis (partial collapse/incomplete inflation of lung)
pneumonitis (lung inflammation)
reduced lung volume
Give 2 thrombosis conditions that can arise due to lung involvement in SLE cases, and why?
Lung involvement increases risk of thromboembolism, so can cause DVT and PE
What molecules when present increase the risk of thromboembolism, due to lung involvement in SLE?
Antiphospholipid antibodies
How do autoantibodies cause haematological abnormalities, and give 4 examples?
Autoantibodies destroy peripheral blood cells
neutropenia
lymphopenia (this level reflects disease activity), thrombocytopenia
haemolytic anaemia
What is antiphospholipid syndrome (ALS)?
Autoimmune disease that increases risk of vascular thrombosis/blood clots, and is associated with pregnancy complications such as early/late foetal loss
What is the procedure for lab diagnosis of antiphospholipid syndrome (ALS), and what are the 3 tests?
At least one type of APS test must be positive on 2 occasions with at least 12 week/3 month gap between them
Lupus anticoagulant test
Anticardiolipin test
Anti-beta-2-glycoprotein 1 test
What percentage of SLE patients develop the classic facial/malar rash, and describe its appearance?
Up to 20% patients
Erythematous, raised, itchy rash over cheeks and nose bridge, spares nasolabial regions
What other type of rash tends to form on the face in SLE patients, other than the classic facial/malar rash?
Discoid rash: round, shiny, scaly (hyperkeratosis) sore that commonly develop on face and scalp (this leads to scarring alopecia)
What 2 types of alopecia can develop in SLE patients?
Diffuse, non-scarring alopecia
Scarring alopecia that develops as result of discoid rash on scalp
Give 3 common symptoms of skin involvement in SLE, other than facial rash, discoid rash and alopecia?
Urticaria/hives: raised, itchy welts
livedo reticularis
vasculitis
What is the overall percentage of pregnancy loss that occurs in SLE, and what are the individual percentages of loss in first pregnancy and later pregnancies?
13.1% overall loss
6% first pregnancy
7.1% later pregnancies
What percentage of pregnancies result in preterm birth, in SLE patients?
40.5%
What is the pericardium?
Double-walled layer that surrounds heart
Give 3 symptoms of SLE in infants (neonatal lupus)?
Pericardial effusion, conduction defects, skin rash
Give 6 examples of areas that are affected by Raynaud’s phenomenon?
Fingers, toes, nipples, ears, nose, lips
Give 3 symptoms of Raynaud’s phenomenon that a patient feels?
pain
numbness
pins and needles
What are the 3 main target of SLE management?
Maintain normal function
Control symptoms
Prevent organ damage
Give 2 examples of what SLE patients should avoid when managing disease?
Smoking
Sun exposure (use spf 50)
What 2 factors are used to determine what drug therapies are used for SLE management?
Progression of SLE: mild, severe, end-organ disease
Type of organ involved
Give 3 examples of medications that can be administered for mild active pain in skin and joints, when managing SLE?
Analgesics, NSAIDs and hydroxychloroquine
Give 5 examples of medications that can be administered for severe active pain, when managing SLE?
prednisolone, MMF, MTX, azathioprine
belimumab (monoclonal antibody targets the β-cell growth factor BLyS)
What medications are used for end-organ disease in SLE?
High-dose glucocorticoids and immunosuppressants
IV methylprednisolone (10 mg/kg IV) and IV cyclophosphamide for six cycles
SE: infection, hemorrhagic cystitis, infertility
Rituximab may be of benefit in some
In maintenance therapy of SLE, how should prednisolone dosage be adjusted, and what is the duration of use?
Prednisolone tapered to low dose for long-term use
Give 3 examples of immunosuppressants that are used for maintenance therapy of SLE?
Azathioprine, MMF, MTX
If a patient with SLE has CVD involvement, what drug can be given and why?
Hydroxyquinone, as it is cardioprotective
If a patient with SLE is at risk of thrombosis or ALS, what drug can be given and why?
Warfarin, as it is an anticoagulant
What 2 factors should be checked when considering bone health of a patient with SLE?
Assess risk of osteoporosis and vitamin D deficiency
Give 5 examples of how to manage Raynaud’s phenomenon?
keeping warm
preventing smoking
calcium channel blockers
iloprost and sildenafil (vasodilators)
What type of blood test is highly sensitive but non-specific for lupus, and what percentage of lupus patients have a positive result?
Positive ANA test, which is found in 98% of lupus patients. Therefore it is highly sensitive but non-specific
After positive ANA blood test, what other blood test is performed to aid lupus diagnosis as it is highly specific?
Anti-dsDNA blood test identifies anti-dsDNA antibodies in blood, which are highly specific markers of lupus
