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Systemic lupus erythematosus in Adult
See also in: Nail and Distal Digit
Other Resources UpToDate PubMed

Systemic lupus erythematosus in Adult

See also in: Nail and Distal Digit
Contributors: Vivian Wong MD, PhD, Yevgeniy Balagula MD, Belinda Tan MD, PhD, Michael W. Winter MD, Susan Burgin MD, Paritosh Prasad MD
Other Resources UpToDate PubMed


Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that can affect almost any organ and is characterized by pathogenic circulating autoantibodies. Sex and ethnicity / race are the strongest risk factors for developing SLE, with a 6:1 female-to-male ratio, and Black women demonstrating a fourfold higher incidence when compared with White individuals. Patients of childbearing potential are most commonly affected.

The etiology of SLE is poorly understood, but there is a strong association with autoantibodies and SLE. For example, even though the autoantibodies are not organ specific, only certain organs in a given patient demonstrate end-organ damage. It is hypothesized that a complex interplay between genetic proclivity and environmental influences leads to a perpetuated autoimmune response.

Autoantibodies play significant roles in the diagnosis, management, and prognosis of SLE. They are as follows:
  • Anti-dsDNA – Highly specific for SLE. Rising levels correlate with increased SLE activity and an increased risk for SLE nephritis. Seen in approximately 55%-65% of SLE patients.
  • Anti-Smith (anti-Sm) – Highly specific for SLE. Seen in approximately 25%-30% of SLE patients. Considerable diagnostic value, but levels do not correlate with disease activity.
  • Anti-RNP – Highly specific for SLE. Seen in approximately 5% of SLE patients.
  • Antinuclear antibody (ANA) – Highly sensitive for SLE. Seen in approximately 99% of SLE patients. In other words, it is very rare for an individual with SLE to have a negative ANA. Considerable screening value, but levels do not correlate with disease activity.
  • Anti-histones – Highly specific for drug-induced SLE.
  • Most SLE patients will have systemic symptoms of fever, fatigue, and weight loss at some time during their course.
The organ systems most commonly affected in SLE are the joints, skin, renal system, pulmonary system, central nervous system (including ischemic stroke), cardiovascular system, and hematologic system. Patients may be anemic. About half of SLE patients will have significant renal involvement that can take the form of several types of glomerulonephritis. A classification system is available from the International Society of Nephrology / Renal Pathology Society, in which most cases are class II through V. Classes III and IV have a poor prognosis. Class V is also known as membranous lupus nephritis. A very significant percentage of patients with membranous lupus nephritis and nephrotic proteinuria will progress to end-stage kidney disease. Renal biopsy is useful to define the type and extent of involvement and to guide therapy. Other complications include thromboembolic disease, particularly in the setting of antiphospholipid antibodies and vasculitis, gastrointestinal (GI), pulmonary, and cardiac involvement.

Skin and joint findings:
Of note, SLE patients often require a multidisciplinary team and, hence, efforts should be made to clarify the level and location of involvement to assist the various disciplines.

SLE is a chronic disease with no known cure. However, there are several disease-modifying medications that are effective in decreasing the burden of disease. The mortality from SLE has decreased in the last several decades. Certain patient characteristics portend a worse prognosis in SLE: male sex, age (both young and old), low socioeconomic status, and being of African descent. Disease phenotypes associated with a poor prognosis include hypertension, renal involvement, antiphospholipid antibody positivity, and antiphospholipid antibody syndrome.

The presence of erythema multiforme-like lesions in a patient with lupus, along with a speckled pattern of antinuclear antibody (ANA), positive anti-Ro/SSA or anti-La/SSB, and positive rheumatoid factor (RF) is known as Rowell syndrome. This syndrome has been described in patients with discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE), and SLE. Its existence as a distinct entity has been debated in the literature; some authors believe the association is coincidental. Prednisone with or without hydroxychloroquine, dapsone, or immunosuppressive drugs such as cyclosporin have been cited as therapy.

Related topic: neonatal lupus erythematosus


M32.9 – Systemic lupus erythematosus, unspecified

55464009 – Systemic lupus erythematosus

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Differential Diagnosis & Pitfalls

  • Rheumatoid arthritis
  • Mixed connective tissue disease – Check for anti-U1RNP antibody. Most patients are positive for this in mixed connective tissue disease.
  • Scleroderma – Check for anticentromere antibodies and anti-Scl-70 antibodies. Typified by sclerotic changes in skin not seen in dermatomyositis.
  • Scleroderma
  • Drug-induced lupus erythematosus
  • Dermatomyositis (DM) – Characteristic heliotrope rash (violaceous plaques surrounding the eyes), photodistributed cutaneous eruption, and nail fold changes. Look for elevated serum creatinine kinase (CK) levels and proximal symmetric extremity weakness. Erythema of DM is more violaceous than SLE. While in SLE erythematous macules and plaques on the dorsal fingers occur both in between and over joints, in DM they tend to favor joints (atrophic dermal papules of dermatomyositis, formerly called Gottron papules).
  • Antiphospholipid antibody syndrome / lupus anticoagulant – Can overlap with SLE; associated with recurrent thromboses and spontaneous abortions, elevated prothrombin time (PT).
  • Polymyositis – Without cutaneous findings.
  • Sarcoidosis
  • AL amyloidosis
  • Kikuchi-Fujimoto disease
  • Adult onset Still disease
    Differential diagnosis of cutaneous findings:
    • Erythrotelangiectatic Rosacea – ANA negative.
    • Cutaneous manifestations of Dermatomyositis
    • Mixed connective tissue disease
    • Noncarcinoid Flushing disorders
    • Drug-induced phototoxic reaction / Drug-induced photoallergic reaction
    • Seborrheic dermatitis – No systemic findings. Erythema and scale in sebaceous distribution.
    • Allergic contact dermatitis
    • Pityriasis rubra pilaris
    • Subacute cutaneous lupus erythematosus
    • Chronic cutaneous lupus erythematosus (Discoid lupus erythematosus, Tumid lupus erythematosus, Lupus panniculitis, Chilblain lupus erythematosus) without systemic involvement
    • Urticaria
    • Polymorphous light eruption (PMLE) – Most lesions resolve within several days; skin lesions are located primarily on sun-exposed areas (SLE can occur on sun-exposed and sun-protected areas). Note that previous studies have shown that up to 19% of patients with PMLE can be ANA positive. Hence, an ANA alone may not be sufficient in differentiating PMLE from SLE.
    • Acute lesions of Protoporphyria may have similar locations, especially on the dorsum of the hands, but usually there is no weakness.
    • Tinea faciei – Check potassium hydroxide (KOH); will also be ANA negative.
    • Erythromelalgia – Occurs very rarely on the face.
    • Generalized Morphea – Asymmetric induration, no Raynaud phenomenon, no systemic involvement.
    • Chilblains (perniosis)
    Differential diagnosis of severe cutaneous LE with necrosis:
    • Toxic epidermal necrolysis
    • Methotrexate-induced mucocutaneous toxicity
    • Acute graft-versus-host disease
    • Staphylococcal scalded skin syndrome
    Differential diagnosis of lupus nephritis:
    • Acute bacterial endocarditis
    • Acute poststreptococcal glomerulonephritis
    • IgA nephropathy
    • Immunoglobulin A vasculitis
    • Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis: Eosinophilic granulomatosis with polyangiitis (EGPA), Granulomatosis with polyangiitis (GPA), Microscopic polyangiitis (MPA)
    • Antiglomerular basement membrane disease

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      Last Reviewed:02/07/2019
      Last Updated:05/03/2023
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      Patient Information for Systemic lupus erythematosus in Adult
      Contributors: Medical staff writer
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      Systemic lupus erythematosus in Adult
      See also in: Nail and Distal Digit
      A medical illustration showing key findings of Systemic lupus erythematosus : Fatigue, Fever, Headache, Oral ulcers, ANA positive, Hematuria, Photosensitivity, Proteinuria, Arthralgia, WBC decreased, Malar rash, Reticular rash
      Clinical image of Systemic lupus erythematosus - imageId=63230. Click to open in gallery.  caption: 'Erythema of the cheeks and nose, with superimposed petechiae, purpura, and mottled brown discoloration. Note also the scaling and crusting of the lower lip.'
      Erythema of the cheeks and nose, with superimposed petechiae, purpura, and mottled brown discoloration. Note also the scaling and crusting of the lower lip.
      Copyright © 2024 VisualDx®. All rights reserved.