21/5/22, 12:02 Lupus eritematoso sistémico (LES): fundamentos prácticos, fisiopatología, etiología

Este sitio está destinado a profesionales de la salud.

Lupus Eritematoso Sistémico

(LES)
Actualizado: 28 de abril de 2022
Autor: Christie M Bartels, MD, MS; Editor jefe: Herbert S Diamond, MD  más...

OVERVIEW

Fundamentos de la práctica
El lupus eritematoso sistémico (LES) es un trastorno autoinmune caracterizado por anticuerpos
contra antígenos nucleares y citoplasmáticos, inflamación multisistémica, manifestaciones clínicas
proteicas y un curso recurrente y remitente. Más del 90% de los casos de LES ocurren en mujeres,
comenzando frecuentemente en edad fértil. Vea la imagen a continuación.

Las erupciones por lupus eritematoso sistémico fotosensible (LES) generalmente ocurren en la cara o las extremidades, que
son regiones expuestas al sol. Aunque los espacios interfalángicos están afectados, las articulaciones metacarpofalángicas
(MCP), interfalángicas proximales (PIP) e interfalángicas distales (DIP) están intactas. Foto cortesía del Dr. Erik Stratman,
Marshfield Clinic.

See Cutaneous Clues to Accurately Diagnosing Rheumatologic Disease, a Critical Images slideshow,
to help recognize cutaneous manifestations of rheumatologic diseases. Also, see the Autoimmune
Disorders: Making Sense of Nonspecific Symptoms slideshow to help identify several diseases that
can cause a variety of nonspecific symptoms.

Signs and symptoms of SLE

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SLE is a chronic inflammatory disease that can affect almost any organ system, although it mainly
involves the skin, joints, kidneys, blood cells, and nervous system. Its presentation and course are
highly variable, ranging from indolent to fulminant.

In childhood-onset SLE, the following clinical manifestations are more commonly found than in adults
[1] :

Malar rash
Ulcers/mucocutaneous involvement
Kidney involvement, proteinuria, urinary cellular casts
Seizures
Thrombocytopenia
Hemolytic anemia
Fever
Lymphadenopathy.

In adults, Raynaud pleuritis and sicca are twice as common as in children and adolescents.
[1]

The classic presentation of a triad of fever, joint pain, and rash in a woman of childbearing age should
prompt investigation into the diagnosis of SLE.
[2, 3]

Patients may present with any of the following manifestations

[4] :

Constitutional (eg, fatigue, fever, arthralgia, weight changes)

Musculoskeletal (eg, arthralgia, arthropathy, myalgia, frank arthritis, avascular necrosis)
Dermatologic (eg, malar rash, photosensitivity, discoid lupus)
Renal (eg, acute or chronic kidney failure, acute nephritic disease)
Neuropsychiatric (eg, seizure, psychosis)
Pulmonary (eg, pleurisy, pleural effusion, pneumonitis, pulmonary hypertension, interstitial lung
disease)
Gastrointestinal (eg, nausea, dyspepsia, abdominal pain)
Cardiac (eg, pericarditis, myocarditis)
Hematologic (eg, cytopenias such as leukopenia, lymphopenia, anemia, or thrombocytopenia)

In patients with suggestive clinical findings, a family history of autoimmune disease should raise
further suspicion of SLE.

See Presentation for more detail.

Diagnosis of SLE
The diagnosis of SLE is based on a combination of clinical findings and laboratory evidence.
Familiarity with the diagnostic criteria helps clinicians to recognize SLE and to subclassify this
complex disease based on the pattern of target-organ manifestations.

The American College of Rheumatology (ACR) and the European League Against Rheumatism
(EULAR) published new criteria for the classification of SLE in 2019.
[5, 6]  These criteria represent
current concepts of SLE, and have excellent specificity and sensitivity. They replace the 1997 ACR
criteria for SLE diagnosis.
[7]

The ACR/EULAR classification requires an antinuclear antibody (ANA) titer of at least 1:80 on HEp-2
cells or an equivalent positive test at least once. If that is present, 22 "additive weighted" classification
criteria are considered, comprising seven clinical domains (constitutional, hematologic,

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neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three immunologic

domains (antiphospholipid antibodies, complement proteins, SLE-specific antibodies).

Each criterion is assigned points, ranging from 2 to 10. Patients with at least one clinical criterion and
10 or more points are classified as having SLE. See Workup.

Testing

Laboratory studies used in the diagnosis of SLE are as follows:

CBC with differential

Serum creatinine
Urinalysis with microscopy
ESR or CRP level
Complement levels
Liver function tests
Creatine kinase assay
Spot protein/spot creatinine ratio
Autoantibody tests

Imaging studies

The following imaging studies may be used to evaluate patients with suspected SLE:

Joint radiography
Chest radiography and chest CT scanning
Echocardiography
Brain MRI/MRA
Cardiac MRI

Procedures

Procedures that may be performed in patients with suspected SLE include the following:

Arthrocentesis
Lumbar puncture
Kidney biopsy

See Workup for more detail.

Management
Management of SLE often depends on the individual patient’s disease severity and disease
manifestations,
[8] although hydroxychloroquine has a central role for long-term treatment in all SLE
patients.

Pharmacotherapy

Medications used to treat SLE manifestations include the following:

Antimalarials (eg, hydroxychloroquine)

Corticosteroids (eg, methylprednisolone, prednisone), short-term use recommended
Nonbiologic DMARDS: Cyclophosphamide, methotrexate, azathioprine, mycophenolate,
cyclosporine 
Nonsteroidal anti-inflammatory drugs (NSAIDS; eg, ibuprofen, naproxen, diclofenac)
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Biologic DMARDs (disease-modifying antirheumatic drugs): Belimumab, rituximab, anifrolumab,

and/or IV immune globulin

See Treatment and Medication for more detail.

See also the following Medscape articles:

Pediatric Systemic Lupus Erythematosus

Systemic Lupus Erythematosus and Pregnancy
Bullous Systemic Lupus Erythematosus (BSLE)

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