SLE

Lupus is a chronic inflammatory disease that occurs when your body's immune system attacks
your own tissues and organs. Inflammation caused by lupus can affect many different body
systems including your joints, skin, kidneys, blood cells, brain, heart and lungs.
Lupus can be difficult to diagnose because its signs and symptoms often mimic those of other
ailments. The most distinctive sign of lupus a facial rash that resembles the wings of a
butterfly unfolding across both cheeks occurs in many but not all cases of lupus.
Symptoms:

Fatigue and fever

Joint pain, stiffness and swelling
Butterfly-shaped rash on the face that covers the cheeks and bridge of the nose
Skin lesions that appear or worsen with sun exposure
Fingers and toes that turn white or blue when exposed to cold or during stressful periods
(Raynaud's phenomenon)
Shortness of breath
Chest pain
Dry eyes
Headaches, confusion, memory loss

Musculoskeletal symptoms
Joint pain is one of the most common reasons for the initial clinical presentation in patients with
SLE. Arthralgia, myalgia, and frank arthritis may involve the small joints of the hands, wrists,
and knees. In contrast to rheumatoid arthritis, SLE arthritis or arthralgia may be asymmetrical,
with pain that is disproportionate to swelling.
Dermatologic symptoms
Cutaneous manifestations of SLE comprise 3 American College of Rheumatology (ACR) lupus
diagnostic criteria and other clues to a potential diagnosis of lupus.
The first criterion is malar rash, which is characterized by an erythematous rash over the cheeks
and nasal bridge. It lasts from days to weeks and is occasionally painful or pruritic.
The second feature is photosensitivity, which may be elicited from patients who are asked if they
have any unusual rash or symptom exacerbation after sun exposure, with anticipated duration of
approximately 2 days in classic cases.
The third feature may be discoid rash. Discoid lesions often also develop in sun-exposed areas
but are plaquelike in character, with follicular plugging and scarring. They may be part of
systemic lupus or may represent discoid lupus without organ involvement, which is a separate
diagnostic entity.

Alopecia is an often less specific cutaneous feature of SLE. It often affects the temporal regions
or creates a patchy pattern of hair loss.
Criteria Diagnostic
The American College of Rheumatology last updated the diagnostic criteria for SLE in 1997.
The most current criteria are listed below.[52, 53]
Table 1. American College of Rheumatology Diagnostic Criteria (Open Table in a new window)
Criterion

Definition

1. Malar rash

Fixed erythema, flat or raised, over the malar eminences, tending to spare the
nasolabial folds

2. Discoid rash

Erythematous raised patches with adherent keratotic scaling and follicular

plugging (Atrophic scarring may occur in older lesions)

3. Photosensitivity

Skin rash as a result of unusual reaction to sunlight, by patient history or physician

observation

4. Oral ulcers

Oral or nasopharyngeal ulceration, usually painless, observed by a physician

5. Arthritis

Nonerosive arthritis involving 2 peripheral joints, characterized by tenderness,

swelling, or effusion

6. Serositis

(A) Pleuritis: Convincing history of pleuritic pain or rub heard by a physician or

evidence of pleural effusion or
(B) Pericarditis: Documented by ECG or rub or evidence of pericardial effusion

7. Renal disorder

(A) Persistent proteinuria >0.5 g/d or >3+ if quantitation not performed or

(B) Cellular casts: May be red blood cell, hemoglobin, granular, tubular, or mixed

8. Neurologic disorder (A) Seizures: In the absence of offending drugs or known metabolic derangements
(eg, uremia, ketoacidosis, electrolyte imbalance) or
(B) Psychosis: In the absence of offending drugs or known metabolic
derangements (eg, uremia, ketoacidosis, electrolyte imbalance)

9.
Hematologic (A) Hemolytic anemia: With reticulocytosis or
disorder
(B) Leukopenia: < 4000/mm3 total on 2 occasions or
(C) Lymphopenia: < 1500/mm3 on 2 occasions or
(D) Thrombocytopenia: < 100,000/mm3 in the absence of offending drugs
10.
Immunologic (A) Anti-DNA: Antibody to native DNA in abnormal titer or
disorder
(B) Anti-Sm: Presence of antibody to Sm nuclear antigen or
(C) Positive finding of antiphospholipid antibodies based on (1) an abnormal
serum level of IgG or IgM anticardiolipin antibodies, (2) a positive test result for
lupus anticoagulant using a standard method, or (3) a false-positive serologic test
for syphilis known to be positive for at least 6 months and confirmed by
Treponema pallidum immobilization or fluorescent treponemal antibody
absorption tests
11.
Antinuclear An abnormal titer of antinuclear antibody by immunofluorescence or an
antibody
equivalent assay at any point in time and in the absence of drugs known to be
associated with drug-induced lupus syndrome
SLE can be diagnosed if any 4 or more of the 11 criteria are present, serially
or simultaneously, during any interval of observation.

Causes
Lupus occurs when your immune system attacks healthy tissue in your body. It's likely that lupus
results from a combination of your genetics and your environment. It appears that people with an
inherited predisposition for lupus may develop the disease when they come into contact with
something in the environment that can trigger lupus. The cause for lupus in most cases, however,
is unknown. Some potential triggers include:

Sunlight. Exposure to the sun may bring on lupus skin lesions or trigger an internal
response in susceptible people.
Medications. Lupus can be triggered by certain types of anti-seizure medications, blood
pressure medications and antibiotics. People who have drug-induced lupus usually see
their symptoms go away when they stop taking the medication.

Treatments and drugs

Treatment for lupus depends on your signs and symptoms. Determining whether your signs and
symptoms should be treated and what medications to use requires a careful discussion of the
benefits and risks with your doctor. As your signs and symptoms flare and subside, you and your
doctor may find that you'll need to change medications or dosages. The medications most
commonly used to control lupus include:

Nonsteroidal anti-inflammatory drugs (NSAIDs). Over-the-counter NSAIDs, such as

naproxen (Aleve) and ibuprofen (Advil, Motrin, others), may be used to treat pain,
swelling and fever associated with lupus. Stronger NSAIDs are available by prescription.
Side effects of NSAIDs include stomach bleeding, kidney problems and an increased risk
of heart problems.
Antimalarial drugs. Antimalarials may work through numerous proposed mechanisms
in SLE, mediating subtle immunomodulation without causing overt immunosuppression.
They are useful in preventing and treating lupus skin rashes, constitutional symptoms,
arthralgias, and arthritis. They also help to prevent lupus flares and have been associated
with reduced morbidity and mortality in SLE patients followed in observational trials.
Medications commonly used to treat malaria, such as hydroxychloroquine (Plaquenil),
also can help control lupus. Side effects can include stomach upset and, very rarely,
damage to the retina of the eye.

Corticosteroids. Prednisone and other types of corticosteroids can counter the

inflammation of lupus, but often produce long-term side effects including weight gain,
easy bruising, thinning bones (osteoporosis), high blood pressure, diabetes and increased
risk of infection. The risk of side effects increases with higher doses and longer term
therapy.
Immune suppressants. Drugs that suppress the immune system may be helpful in
serious cases of lupus. Examples include cyclophosphamide (Cytoxan), azathioprine
(Imuran, Azasan), mycophenolate (Cellcept), leflunomide (Arava) and methotrexate
(Trexall). Potential side effects may include an increased risk of infection, liver damage,
decreased fertility and an increased risk of cancer. A newer medication, belimumab
(Benlysta) also reduces lupus symptoms in some people. Side effects include nausea,
diarrhea and fever.
Immunomodulators. These agents restore the potential to minimize self-immunity.
Belimumab (Benlysta), Rituximab (Rituxan)