Systemic lupus erythematosus in Adult
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Synopsis
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.
Skin and joint findings:
- Arthritis is classically migratory, polyarticular, and symmetrical and a common early finding.
- Skin and mucous membrane abnormalities are also common with the classic "butterfly" malar rash (involving the cheeks and nose) developing after sun exposure – This form of cutaneous lupus is sometimes referred to as acute cutaneous lupus erythematosus (ACLE). There is a range of other skin findings that can occur with SLE. For more details, see subacute cutaneous lupus erythematosus, discoid lupus erythematosus, tumid lupus erythematosus.
- Bullous systemic lupus erythematosus is a rare autoimmune blistering disease in patients with a known diagnosis of SLE.
- Lupus profundus is a rare panniculitis seen in patients with SLE.
- Nonbullous neutrophilic lupus erythematosus is an extremely rare eruption of widespread urticarial papules and plaques that has been reported in patients with SLE.
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
Codes
ICD10CM:M32.9 – Systemic lupus erythematosus, unspecified
SNOMEDCT:
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.
- Systemic sclerosis
- Drug-induced SLE
- 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
- Systemic amyloidosis
- Kikuchi disease
- Adult-onset Still disease
- Erythrotelangiectatic rosacea – ANA negative.
- Cutaneous manifestations of dermatomyositis
- Mixed connective tissue disease
- Noncarcinoid flushing
- Phototoxic / photoallergic drug eruptions
- Seborrheic dermatitis – No systemic findings. Erythema and scale in sebaceous distribution.
- Contact dermatitis
- Pityriasis rubra pilaris
- Subacute lupus erythematosus
- Chronic cutaneous lupus erythematosus (discoid lupus erythematosus, lupus tumidus, lupus panniculitis, chilblain lupus) 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 erythropoietic 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)
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Last Reviewed:02/07/2019
Last Updated:04/03/2023
Last Updated:04/03/2023
Patient Information for Systemic lupus erythematosus in Adult
Overview
Systemic lupus erythematosus (SLE), also called lupus, is a chronic inflammatory disease that can affect almost any part of the body, especially the skin, joints, kidneys, heart, lungs, bones, blood, or brain. Systemic lupus erythematosus is considered an autoimmune disorder, meaning that a person's own immune system attacks his or her own healthy cells and tissues, causing inflammation and damage.Because systemic lupus erythematosus can affect any organ system, no two people have identical forms of the disease. However, most people with systemic lupus erythematosus report periods of time in which their symptoms seem to be mild or absent (remission) and other periods of time when the inflammation is more severe (flare or relapse).
Who’s At Risk
Systemic lupus erythematosus can occur in people of all ages, all races, and both sexes. However, it is far more common in women, especially those between 15-45 years old. In America, it is also more commonly seen in people with darker skin than in light-skinned people.Although it is not directly inherited, lupus and other autoimmune diseases may run in families. Inheriting certain genes may make some people more susceptible to developing lupus.
In addition, certain environmental factors may trigger lupus in those who have a family (genetic) tendency toward the disease, including:
- Ultraviolet light, especially sunlight
- Certain medications, especially hydralazine and procainamide
- Infections
- Antibiotics, especially penicillins or sulfa-containing medicines
- Stress
- Hormonal changes, especially related to pregnancy and menstrual cycles
Signs & Symptoms
More than 90% of people with systemic lupus erythematosus have skin symptoms. The most common locations for the skin lesions of systemic lupus erythematosus include:- Face, especially cheeks and nose
- Sun-exposed skin on arms, backs of hands, upper chest, and upper back due to increased sensitivity to sunlight (photosensitivity)
- Fingers and fingernails
- Mouth or nose
- Scalp
A rash can develop in sun-exposed skin (photo-distribution), especially on the backs of the hands and fingers. This rash, which appears as red, scaly patches, can also affect the arms and trunk.
The skin around fingernails (nail folds) can be red and inflamed, and tiny, dilated blood vessels (telangiectasia) may be seen. In addition, people may develop Raynaud phenomenon, in which the fingers (and sometimes toes) turn pale and numb after exposure to cold temperatures.
Small, painless ulcers can develop in the nose or, more commonly, in the mouth, especially on the roof of the mouth.
When lupus affects the scalp skin, you may notice hair loss. It may be patchy, or there may be thinning across the scalp, especially at the temples.
In addition to the skin lesions of lupus, people may have:
- Joint pain or swelling, especially in hands, wrists, and knees
- Blood problems, including anemia and clotting disorders
- Kidney disorders
- Lung problems, such as painful breathing
- Seizures or other brain disorders
- Swollen lymph glands
- Fever
- Fatigue
Self-Care Guidelines
If you know you have systemic lupus erythematosus, several measures can help prevent flares:- Avoid intense sun exposure.
- Apply sunscreen with SPF of 30 or higher every day.
- Maintain healthy habits such as resting well, eating a balanced diet, and exercising regularly.
- Reduce stress.
- Avoid smoking and limit alcohol use.
When to Seek Medical Care
See your doctor if you develop a rash with fever and fatigue.Treatments
Lupus can be difficult to diagnose for 3 reasons: systemic lupus erythematosus can affect so many different organ systems, its symptoms can come and go, and no 2 people have exactly the same form of the disease. In addition to a careful review of your medical history, your doctor may perform blood tests, urinalysis, chest X-ray, or an electrocardiogram (ECG) before confirming the diagnosis of lupus.If you have a rash that is suspicious for lupus, you nay need a skin biopsy. The procedure involves:
- Numbing the skin with an injectable anesthetic.
- Sampling a small piece of skin by using a flexible razor blade, a scalpel, or a tiny cookie cutter (called a "punch biopsy"). If a punch biopsy is taken, a suture or two may be placed and will need to be removed 6-14 days later.
- Having the skin sample examined under the microscope by a specially trained physician (dermatopathologist).
- Anti-malarial drugs such as hydroxychloroquine, chloroquine, or quinacrine
- Corticosteroids
- Anti-inflammatory medications such as aspirin, ibuprofen, naproxen, or indomethacin
- Immune-suppressing medications including azathioprine, cyclophosphamide, methotrexate, cyclosporine, chlorambucil, or mycophenolate mofetil
References
Bolognia, Jean L., ed. Dermatology, pp.594-595. New York: Mosby, 2003.
Freedberg, Irwin M., ed. Fitzpatrick's Dermatology in General Medicine. 6th ed, pp.1619, 1677, 1681-1687. New York: McGraw-Hill, 2003.
Systemic lupus erythematosus in Adult
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