Systemic Lupus Erythematosus Flashcards
SLE - General Features
Systemic lupus erythematosus (SLE) is a multisystem, autoimmune disorder. It typically presents in early adulthood and is more common in women and people of Afro-Caribbean origin.
General features fatigue fever mouth ulcers lymphadenopathy
SLE - Skin Fx
Skin
malar (butterfly) rash: spares nasolabial folds
discoid rash: scaly, erythematous, well demarcated rash in sun-exposed areas. Lesions may progress to become pigmented and hyperkeratotic before becoming atrophic
photosensitivity
Raynaud’s phenomenon
livedo reticularis
non-scarring alopecia
SLE - Musculoskeletal Fx
Musculoskeletal
arthralgia
non-erosive arthritis
SLE - Cardiovascular Fx
Cardiovascular
myocarditis
SLE - Respiratory Fx
Respiratory
pleurisy
fibrosing alveolitis
SLE - Renal Fx
Renal
proteinuria
glomerulonephritis (diffuse proliferative glomerulonephritis is the most common type)
SLE - Neuropsychiatric Fx
Neuropsychiatric
anxiety and depression
psychosis
seizures
SLE - Mx
SLE: management
Basics
NSAIDs
sun-block
Hydroxychloroquine
useful for skin disease
If internal organ involvement e.g. renal, neuro, eye then consider prednisolone, cyclophosphamide
SLE and Mx in Pregnancy
A 28-year-old woman with systemic lupus erythematosus attends the pre-conception clinic. She would like some advice regarding her mediations prior to getting pregnant. She has never been pregnant before and her lupus has been stable on her current medications: mycophenolate and hydroxychloroquine for over 12 months. She also has asthma, which is well controlled with beclomethasone and salbutamol inhalers, and she takes regular omeprazole for gastro-oesophageal reflux.
What is the most appropriate medication amendment?
Half omeprazole dose Stop beclomethasone inhaler Stop hydroxycholorquine Add ramipril 1.25mg > Change mycophenolate to azathioprine
Unlike the majority of autoimmune conditions, pregnancy increases the likelihood of a lupus flare. It is essential that a patients lupus is well controlled and quiescent for at least six months prior to pregnancy. Given that mycophenolate is teratogenic in pregnancy, it must be stopped. It is common practice to change to azathioprine, which has been shown to be safe in pregnancy - but still used with caution! However knowledge of common medications used/not used in pregnancy should also lead the candidate to this option.
There is no need to stop the beclomethasone or hydroxycholoquine, as they are both safe in pregnancy. Omeprazole is also safe in pregnancy and the dose does not need to be changed. ACE inhibitors are contraindicated in pregnancy and must be stopped/changed to another agent.
Jaccoud’s Arthropathy - Diagnosis: Example Question
A 40-year-old woman is seen in an outpatient rheumatology clinic. She was diagnosed with Systemic lupus erythematosus 5 years previously when she has presented with fatigue, anaemia and a rash. She also has a past medical history of hypertension, gout and psoriasis.
She had noticed that the joints in her hands were becoming deformed, however, she was able to complete day to day task without any functional impairment and denied pain in the affected joints.
On examination, she had symmetrical marked reducible ulnar subluxation and deviation at the MCP joints. X-rays of her hands showed no erosions.
What is the most likely diagnosis?
> Jaccoud's arthropathy Rheumatoid arthritis Gout Psoriatic arthritis Sarcoid arthropathy
The key here is the absence of pain which is more than likely to be present in options B to E hence the presence of psoriasis is a distracting factor. Additionally, gout usually affects the first metatarsal head and sarcoid arthropathy is very rare hence Jaccoud’s arthropathy which is none erosive and associated with systemic lupus erythematosus is the correct answer.
SLE and Pregnancy - Neonatal Cx
Overview
- risk of maternal autoantibodies crossing placenta
- leads to condition termed neonatal lupus erythematous
- neonatal complications include congenital heart block
- strongly associated with anti-Ro (SSA) antibodies
Anti-Ro antibodies can cross the placenta and can lead to neonatal lupus and congenital heart block of the newborn, which can require pacing at birth. Miscarriage is another common complication of SLE. These can occur beyond the first trimester.
SLE and Pregnancy: Diagnosis and Cx - Example Question
A 28 year old woman who is 20 weeks pregnant is referred to you by her GP. She has a 2 month history of arthralgia, myalgia, and fatigue. She had initially put this down to pregnancy but was finding it increasingly difficult to do her job as a health care assistant in a local nursing home. She denied any shortness of breath, swallowing difficulties or alopecia.
She had asthma since childhood but was relatively well controlled on inhaled salbutamol as required and beclomethasone 400 micrograms twice daily.
She was a smoker of 10 cigarettes per day and had not drunk any alcohol since learning she was pregnant. She lives with her husband and 2 year old son. Her mother has a history of rheumatoid arthritis.
Her observations show a blood pressure of 138/86 mmHg and a heart rate of 92 beats per minute. Urinalysis showed a trace of protein.
On examination there was tenderness of the 2nd and 3rd metacarpalphalangeal (MCP) joints bilaterally and both wrists but no evidence of active synovitis. There are several painless mouth ulcers. You notice a few bruises on her arms but no other evidence of a rash. Her chest was clear and heart sounds were normal. Neurological examination was normal including full visual fields and eye movements.
Her bloods showed the following:
Haemoglobin 108 g/L
White Cell Count 9.2 x 109/L
Platelets 103 x 109/L
Neutrophils 6.02 x 109/L
Lymphocytes 0.80 x 109/L
Eosinophils 0.90 x 109/L
ESR 29 mm/h
Urea 6.9 mmol/L
Creatinine 118 micromol/L
CRP 11 mg/L
Alkaline Phosphatase 87 iu/L
ALT 42 iu/L
Albumin 32 g/L
ANA 1: 320 dsDNA 24 Anti -Ro Positive Anti -La Positive Rheumatoid Factor Positive Anti CCP Negative Antiphospholipid antibody negative
Given the most likely diagnosis, what complication needs to be discussed with her?
Post partum haemorrhage > Congenital heart block Deep vein thrombosis Pre-eclampsia Scleritis
This question tests your knowledge of the diagnosis of Systemic Lupus Erythematosis (SLE). Diagnosis is based on the American College of Rheumatology (ACR) criteria written in 1982 and revised in 1997:
Four or more of the 11 criteria need to be fulfilled to be able to diagnose SLE. Note that while fatigue is a common feature it is not used in the diagnostic criteria. In this case the criteria are oral ulcers + arthritis + positive dsDNA + the presence of ANA (while there is lymphopenia this is a single test result only)
In this case there are sicca symptoms and Anti-Ro and -La antibodies suggesting an overlap with Sjogrens syndrome. Rheumatoid factor is positive in approximately 40% of SLE patients. The absence of anti CCP should point you away from rheumatoid arthritis.
The point to make with this question is to test the candidates knowledge of diagnosis of connective tissue diseases and their associated complications. Anti-Ro antibodies can cross the placenta and can lead to neonatal lupus and congenital heart block of the newborn, which can require pacing at birth. Miscarriage is another common complication of SLE. These can occur beyond the first trimester.
While post partum haemorrhage, pre-eclampsia and deep vein thrombosis are complications of pregnancy that will need discussed the presence of SLE does not increase the risk of either in this scenario. If the antiphospholipid antibody or lupus anticoagulant were positive then there is increased risk of arterial or venous thrombosis, in which case you might consider anticoagulation but obviously not with warfarin in pregnancy.
SLE - Immunology
IMMUNOLOGY
- ANA+ve = 99%
- Rheumatoid Factor +ve = 20%
- anti-dsDNA: Highly specific > 99% but less sensitive at 70%
- anti-Smith most specific at > 99% but least sensitive at 30%
SLE - Monitoring
MONITORING
- ESR - during active disease CRP is characteristically normal (a raised CRP may therefore indicate underlying infection)
- Complement Levels (C3,C4) - low during active disease (formation of complexes leads to consumption of complement)
- anti-dsDNA titres - can be used for disease monitoring (but not present in all SLE patients!)
SLE and Renal Disease
Diffuse proliferative GN = most common and most severe form of renal disease in SLE
Fx:
- Haematuria
- Proteinuria
- Oedema
- HTN
