Systemic lupus erythematosus
Summary
Definition
History and exam
Key diagnostic factors
- malar (butterfly) rash
- photosensitive rash
- discoid rash
Other diagnostic factors
- fatigue
- weight loss
- fever
- oral ulcers
- alopecia
- arthralgia/arthritis
- Raynaud phenomenon
- telangiectasia
- livedo reticularis
- vasculitis
- chest pain and shortness of breath
- venous or arterial thrombosis
- hypertension
- signs of nephrosis (e.g., edema)
- lymphadenopathy
- abdominal pain, vomiting, or diarrhea
- nose ulcers
- poorly localized proximal limb inflammatory pain with weakness
- dysrhythmias (e.g., tachycardia), conduction defects, or unexplained cardiomegaly
- neuropsychiatric symptoms
- dysphagia
Risk factors
- female sex
- age >30 years
- African descent in Europe and US
- use of certain drugs
- sun exposure
- family history of SLE
- tobacco smoking
- air pollution
- free crystalline silica
Diagnostic tests
1st tests to order
- complete blood count with differential
- activated partial thromboplastin time
- BUN and electrolytes
- erythrocyte sedimentation rate and C-reactive protein
- antinuclear antibodies, double-stranded (ds)DNA, Smith antigen
- urinalysis
- chest x-ray
- ECG
Tests to consider
- blood and urine cultures
- antiphospholipid antibodies
- Coombs test
- 24-hour urine collection for protein or spot urine for protein/creatinine ratio
- complement levels
- creatinine phosphokinase
- plain x-rays of affected joint(s)
- renal ultrasound
- CT chest
- pulmonary function tests
- pleural aspiration
- brain magnetic resonance imaging
- echocardiogram
- skin biopsy
- kidney biopsy
- thyroid-stimulating hormone
Treatment algorithm
nonrenal SLE: constitutional symptoms or joint manifestations/serositis
nonrenal SLE: mucocutaneous manifestations
renal SLE (lupus nephritis)
nonrenal SLE: neuropsychiatric manifestations
nonrenal SLE: hematologic manifestations
Colaboradores
Autores
Jacob J. E. Koopman, MD PhD
Medical Specialist in Clinical Immunology
Internal Medicine
Erasmus Medical Center
Rotterdam
Netherlands
Divulgaciones
JJEK is a member of the editorial board for the Dutch Journal of Allergy, Asthma and Clinical Immunology. He has received research grants from SLEuro, European Alliance of Associations for Rheumatology, American College of Rheumatology, Erasmus Trust Fund and NVLE, and has also acted as an unpaid subinvestigator as a medical specialist for clinical trials which were sponsored by UMC Utrecht, GlaxoSmithKline, Nierstichling Nederland, Aurinia Pharmaceuticals and Biogen.
Agradecimientos
Dr Jacob Koopman would like to gratefully acknowledge Dr Meng May Chee, Dr Rajan Madhok, and Dr Gayle McKellar, as previous contributors to this topic.
Divulgaciones
MMC was sponsored by Abbvie to attend the European League Against Rheumatism 2020 online conference. RM holds shares in GSK to the value less than £12,000. GM declares that she has no competing interests.
Revisores por pares
James C. Oates, MD
Associate Director
Medical University of South Carolina
Charleston
SC
Divulgaciones
JCO declares that he has no competing interests.
Nancy J. Olsen, MD, PhD
Professor of Medicine
University of Texas Southwestern Medical Center
Dallas
TX
Divulgaciones
NJO declares that she has no competing interests.
Agradecimiento de los revisores por pares
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Divulgaciones
Las afiliaciones y divulgaciones de los revisores por pares se refieren al momento de la revisión.
Referencias
Artículos principales
Fanouriakis A, Kostopoulou M, Andersen J, et al. EULAR recommendations for the management of systemic lupus erythematosus: 2023 update. Ann Rheum Dis. 2024 Jan 2;83(1):15-29.Texto completo Resumen
Sammaritano LR, Askanase A, Bermas BL, et al. 2024 American College of Rheumatology (ACR) guideline for the screening, treatment, and management of lupus nephritis. Arthritis Rheumatol. 2025 Sep;77(9):1115-35.Texto completo Resumen
Kidney Disease: Improving Global Outcomes (KDIGO) Lupus Nephritis Work Group. KDIGO 2024 clinical practice guideline for the management of LUPUS NEPHRITIS. Kidney Int. 2024 Jan;105(1s):S1-69.Texto completo Resumen
Parodis I, Girard-Guyonvarc'h C, Arnaud L, et al. EULAR recommendations for the non-pharmacological management of systemic lupus erythematosus and systemic sclerosis. Ann Rheum Dis. 2024 May 15;83(6):720-9.Texto completo Resumen
Artículos de referencia
Una lista completa de las fuentes a las que se hace referencia en este tema está disponible para los usuarios con acceso a todo BMJ Best Practice.
Diferenciales
- Rheumatoid arthritis
- Antiphospholipid syndrome
- Systemic sclerosis
Más DiferencialesGuías de práctica clínica
- American College of Rheumatology guidelines for the screening, treatment, and management of lupus nephritis
- American College of Rheumatology guidance statements for addressing mental health concerns in youth with pediatric rheumatologic diseases
Más Guías de práctica clínicaFolletos para el paciente
Systemic lupus erythematosus
Más Folletos para el pacienteVideos
Venepuncture and phlebotomy: animated demonstration
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