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Systemic lupus erythematosus

What's new

Updated 2023 EULAR guidelines for the management of systemic lupus erythematosus.

Background

Overview

Definition
SLE is a chronic autoimmune disease with a heterogeneous clinical course and progressive involvement of multiple organs.
1
Pathophysiology
SLE is caused by a complex interaction between genetic (SNP polymorphisms, deficiency of C1q and C4), epigenetic (DNA hypomethylation), hormonal (X chromosome and estrogen), immunoregulatory (T and B cell rewiring) and environmental factors (UV exposure, smoking).
2
Epidemiology
In the US, the estimated incidence of SLE ranges from 4.9 to 7.4 cases per 100,000 person-years, while the estimated prevalence ranges from 72.8 to 178 persons per 100,000 population.
3
Disease course
Autoantibody production, immune complex formation, and inflammatory T cell activation result in inflammation and end-organ damage (particularly in the skin, kidneys, heart, lungs, and brain), which causes increased morbidity and mortality.
2
Prognosis and risk of recurrence
The 10-year survival rate of patients with SLE is approximately 70%. The all-cause standard mortality rate is increased 2.6 fold in patients with SLE, and the risk of mortality is increased by 4.98, 4.67, and 2.25 fold for infections, renal disease, and CVD, respectively.
2
3

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of systemic lupus erythematosus are prepared by our editorial team based on guidelines from the European League Against Rheumatism (EULAR 2024,2022,2019), the American College of Rheumatology (ACR 2023), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021), the European Dialysis and Transplant Association (ERA-EDTA/EULAR 2020), the Royal College of Ophthalmologists (RCOphth ...
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Screening and diagnosis

Clinical manifestations: as per BSR 2018 guidelines, recognize that clinical manifestations in SLE May be due to disease activity (inflammation or thrombosis), damage, drug toxicity or the presence of comorbidity.
B
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  • Diagnosis

Classification and risk stratification

Assessment of disease activity: as per BSR 2018 guidelines, classify disease activity into the following categories according to the occurrence of flares
B
:
Situation
Guidance
Mild
Clinically stable disease with no life-threatening organ involvement, mainly manifesting as arthritis, mucocutaneous lesions and mild pleuritis
Moderate
More serious manifestations
Severe
Organ- or life-threatening disease
B

Diagnostic investigations

Initial evaluation: as per EULAR 2019 guidelines, screen for antiphospholipid syndrome in all patients with SLE at diagnosis.
A

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  • Anti-Ro/La antibodies

  • Screening for chronic infections

  • Evaluation of cardiac risk

  • Evaluation of fracture risk

  • Evaluation for osteonecrosis

  • Screening for malignancy

Medical management

General principles: as per EULAR 2024 guidelines, include management of bone health, nephroprotection, and cardiovascular risk in the treatment of patients with SLE.
B

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  • Goals of treatment

  • Hydroxychloroquine

  • Corticosteroids

  • Immunosuppressants

  • Biologics

  • IVIG

Nonpharmacologic interventions

Sun protection: as per BSR 2018 guidelines, advise using high-sun protection factor UVA and UVB sunscreen
B
, avoiding sun exposure and wearing protective clothing for the management and prevention of UV radiation-induced skin lesions in patients with SLE.
B

Therapeutic procedures

Plasmapheresis: as per BSR 2018 guidelines, consider performing plasmapheresis
C
in patients with refractory cytopenias or TTP,
C
rapidly deteriorating acute confusional state or catastrophic variant of antiphospholipid syndrome.

Specific circumstances

Pregnant patients
As per BSR 2018 guidelines:
Recognize that the presence of antiphospholipid antibodies is associated with thrombotic events, damage, and adverse outcomes in pregnancy.
B
Reevaluate antiphospholipid antibodies, if previously negative, before pregnancy or surgery, or in the presence of a new severe manifestation or vascular event.
B
Screen for anti-Ro and anti-La antibodies before pregnancy because of the risk of neonatal lupus (including congenital heart block).
B

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  • Patients with lupus nephritis

  • Patients with diffuse alveolar hemorrhage

  • Patients with pericarditis

  • Patients with neuropsychiatric manifestations (evaluation)

  • Patients with neuropsychiatric manifestations (management)

  • Patients with hemolytic anemia

  • Patients with thrombocytopenia

  • Patients with antiphospholipid syndrome

  • Patients with musculoskeletal manifestations

  • Patients with cutaneous manifestations

Patient education

Patient education: as per CRA 2018 guidelines, educate adult and pediatric patients with SLE (in particular, patients who have a history of corticosteroid exposure) regarding the symptoms of osteonecrosis (including progressive or sudden deep joint pain that is worse with weight-bearing).
E

Preventative measures

Routine immunizations: as per EULAR 2024 guidelines, offer immunizations against HZV, HPV, influenza, COVID-19, and pneumococcus.
B

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  • Prophylaxis for P. jirovecii pneumonia

Follow-up and surveillance

Indications for specialist referral: as per PANLAR 2018 guidelines, refer all adult patients with suspected SLE to an SLE specialist, most often a rheumatologist, to confirm diagnosis and facilitate ongoing care.
B

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  • Serial clinical and laboratory monitoring

  • Surveillance for hydroxychloroquine retinopathy