11/14/24, 10\:42 AM Systemic Lupus Erythematosus (SLE)

Systemic Lupus Erythematosus (SLE)

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Systemic lupus erythematosus (SLE)\: multisystem autoimmune disease; type III hypersensitivity reaction; autoantibodies
against cell nuclear antigens; immune complex deposition.
Aetiology\: unknown; genetic factors (HLA-DR2, DR3), hereditary complement de
pathway; environmental triggers (drugs, Epstein-Barr virus).
Risk factors\: African American ethnicity, female sex (9\:1), childbearing age, HLA-DR2/3 carriers, sunlight exposure.
Clinical features\: constitutional symptoms (fatigue, fever, weight loss); malar rash, discoid rash, oral ulcers, photosensitivity;
non-erosive arthritis, pericarditis, pleuritis, lupus nephritis, haematological abnormalities (anaemia, leucopenia,
thrombocytopenia).
Antiphospholipid syndrome\: clotting disorder with antibodies to antiphospholipids; associated with SLE; treated with
hydroxychloroquine and anticoagulation for thrombosis.
Investigations\: urinalysis (haematuria, proteinuria); FBC, ESR, CRP, immunology (anti-nuclear, anti-dsDNA, anti-smith
antibodies, complement levels); imaging (MRI brain, echocardiography).
Diagnosis\: based on SLICC criteria; requires four criteria (at least one clinical, one immunological) or lupus nephritis with
anti-nuclear/anti-dsDNA antibodies.
Management\: education, sun protection, exercise, smoking cessation; mild disease (hydroxychloroquine, methotrexate,
NSAIDs, low-dose prednisolone); moderate disease (azathioprine, mycophenolate mofetil, cyclosporine); severe disease
(high-dose prednisolone, IV methylprednisolone, cyclophosphamide, biologics).
Complications\: end-stage renal disease, atherosclerosis, recurrent miscarriage, permanent skin damage, blindness,
medication-related complications (e.g. infection).
Prognosis\: 10-year survival rate around 90%; early diagnosis and proper management improve outcomes.
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Introduction

Systemic lupus erythematosus (SLE) is a complex multisystem autoimmune disease. It results from a type III
hypersensitivity reaction involving the formation of autoantibodies against cell nuclear autoantigens, which leads to the
deposition of immune complexes throughout the body.
The prevalence of SLE is approximately 73 per 100,000 population in the United States.
1

Aetiology

The exact aetiology of SLE is still unknown.
Twin studies show a higher-than-normal concordance rate in twins, which suggests that genetic factors may be
1
responsible for the development of SLE. Carriers of the HLA-DR2 and DR3 haplotypes are more likely to develop SLE. A
hereditary complement de
implicated.
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Environmental triggers for SLE include drugs (procainamide, hydralazine, minocycline, terbina
sulfasalazine, carbamazepine) and the Epstein-Barr virus.
2
It is hypothesised that the combination of genetic susceptibility and environmental triggers cause a failure of cellular
waste removal and a resulting breakdown of tolerance to self-antigens.

Risk factors

Risk factors for SLE include\:
1
African American ethnicity
Female sex (9\:1)
Childbearing age
HLA-DR2/3 carriers
Sunlight exposure

Clinical features

SLE is a common di
presentation.
The most common presentation of SLE includes constitutional symptoms (e.g. fatigue, fever, weight loss). Over 90% of
patients with SLE experience these symptoms, and these are often the
1
The most characteristic cutaneous feature of SLE is a malar (butter
bridge and cheeks (Figure 1). The rash is often pruritic and spares the nasolabial folds.
Other dermatological features of lupus are discoid erythematous plaques (discoid rash) that tend to occur in sun-exposed
areas (Figure 2), oral ulcers, scarring alopecia, and photosensitivity.
1
Figure 1. Malar rash in SLE.
3
Figure 2. Discoid lupus.
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A common feature of SLE is musculoskeletal symptoms. Many patients with SLE will experience non-erosive arthritis of
the small joints of the hands and feet bilaterally. 2
Patients may also experience early morning sti
in
Lupus has e
and endocardium) can be in
e
1
Pleuritis and pulmonary hypertension are common pulmonary manifestations of SLE, and central nervous system
involvement can result in seizures and other neural abnormalities.
2
Gastrointestinal involvement can lead to abdominal discomfort and vomiting that can result from enteritis, mesenteric
vasculitis, or pancreatitis.
Lupus nephritis is one of the most serious consequences of SLE. The kidneys are involved in 50-70% of SLE cases. Lupus
nephritis may present with haematuria, red blood cell casts, and proteinuria.
2
In many patients with SLE, haematological phenomena are observed. Patients often present with anaemia, leucopenia,
and thrombocytopenia. 2
Antiphospholipid antibody syndrome is an associated condition that can present with lupus.
Antiphospholipid syndrome
Antiphospholipid syndrome is a clotting disorder characterised by the formation of antibodies to antiphospholipid
(anticardiolipin, lupus anticoagulant, and anti-b2-microglobulin antibodies). It can be isolated or associated with SLE.
Treatment with hydroxychloroquine may be of help for patients with antiphospholipid syndrome. For those with
thrombosis, additional anticoagulation is required.
5

History

Typical symptoms of SLE include\:
Constitutional symptoms\: fatigue, weight loss
Musculoskeletal\: arthralgia and joint sti
Cardiac and pulmonary\: chest pain, shortness of breath
Other\: gross haematuria, abdominal pain, dry eyes and mouth, skin and mucosal ulceration

Clinical examination

Typical
2,6
Vitals\: fever, hypertension
Mucocutaneous\: malar rash, discoid rash, oral ulcers, alopecia
Other\: leg oedema, point tenderness (associated
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Di

Di
1,2
Rheumatoid arthritis
Behcet’s disease
Antiphospholipid antibody syndrome
Sarcoidosis
Rosacea
Adult Still’s disease
Systemic sclerosis
Mixed connective tissue disease
Parvovirus B19
HIV
Epstein-Barr virus
Non-Hodgkin lymphoma

Investigations

Bedside investigations

Relevant bedside investigations include\:
Urinalysis\: for haematuria and proteinuria

Laboratory investigations

Relevant laboratory investigations include\:
Haematology\: FBC (may show anaemia, leucopenia, and thrombocytopenia)
Biochemistry\: will likely have an elevated ESR with a potentially normal CRP
Immunology\: anti-nuclear, anti-dsDNA, anti-smith, and antiphospholipid antibodies, and
complement levels (often low in SLE)
Serology in SLE
Anti-nuclear antibodies are the hallmark of SLE and have a sensitivity of over 95%.
1
Following a positive anti-nuclear antibody result, screening for more speci
These include anti-smith antibodies (>99% speci
known to cause rheumatological diseases.

Imaging

Use of imaging will depend on clinical presentation and may include\:
MRI brain
Echocardiography

Diagnosis

The diagnosis of SLE is made using the Systemic Lupus Erythematosus International Collaborating Clinics Group
(SLICC) Criteria.
7
The diagnosis requires ful
Alternatively, lupus nephritis with the presence of anti-nuclear, anti-dsDNA antibodies will also meet the diagnostic criteria.
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Management

Patients diagnosed with SLE will require education about their condition and should receive counselling on lifestyle
changes that can reduce disease burden. This includes avoiding sun exposure and wearing high SPF sunscreen, regular
exercise, and smoking cessation.
6

Medical management

Medical management of SLE varies based on the severity of the disease.
8
For mild disease, hydroxychloroquine and methotrexate are used to limit in
reduce in
articular injections. Methotrexate can be used for patients with skin and joint disease.
For moderate disease presentations, other immunosuppressants and higher doses of prednisolone may be used. These
may include azathioprine, mycophenolate mofetil, and cyclosporine.
For severe cases and for instances of lupus nephritis or central nervous system involvement, high-dose oral prednisolone
or intravenous methylprednisolone should be used in combination with immunosuppressants including
cyclophosphamide. Biologics (e.g. rituximab, belimumab) may also be considered.

Complications

Complications that can arise from the systemic e
End-stage renal disease
Atherosclerosis
Recurrent miscarriage
Permanent skin damage
Blindness
Moreover, complications resulting from medication use are common in SLE (for example, infection because of heavy
steroid use).
1

Prognosis

The 10-year survival rate for SLE is around 90%. 1
Lupus can contribute to early mortality from cardiovascular, renal, and
infectious complications. Early diagnosis and proper management can successfully improve outcomes.

References

Vaillant AAj, Goyal A, Bansal P, Varacallo M. S y s t e m i c L u p u s E r y t h e m a t o s u s . StatPearls. Updated in 2021. Available from\:
[LINK]
Chee MM, Madhok R. S y s t e m i c L u p u s E r y t h e m a t o s u s . BMJ Best Practice. Reviewed in 2021. Available from\: [LINK]
Doktorinternet. Mohammad2018. [LINK]
M a l a r r a s h i n a p a t i e n t w i t h s y s t e m i c l u p u s e r y t h e m a t o s u s . Licence\: [CC BY-SA]
D i s c o i d r a s h o n h e a d o f a p a t i e n t w i t h s y s t e m i c l u p u s e r y t h e m a t o s u s . Licence\: [CC BY-SA]
Bustamante JG, Goyal A, Bansal P, Singhal M. Antiphospholipid Syndrome. StatPearls. Updated in 2021. Available from\:
Tidy C. S y s t e m i c L u p u s E r y t h e m a t o s u s . Edited in 2020. Available from\: [LINK]
Petri M. SLE Criteria. Systemic Lupus Erythematosus International Collaborating Clinics Group. Published in 2017. Available
from\: [LINK]
Gordon C, Amissah-Arthur MB, Gayed M, Brown S, Bruce IN, D’Cruz D, et al. The British Society for Rheumatology guideline
for the management of systemic lupus erythematosus in adults. Rheumatology (Oxford). 2018;57(1)\:e1-e45. Available from\:
[LINK]
https\://app.geekymedics.com/notebook/2672/ 5/611/14/24, 10\:42 AM Systemic Lupus Erythematosus (SLE)

Reviewer

Dr Grainne Murphy
Consultant Rheumatologist
Cork University Hospital

Related notes

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Contents

Introduction
Aetiology
Risk factors
Clinical features
Di
Investigations
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