Must have 4/11 criteria for classification, not diagnosis.
1. Malar rash
2. Discoid rash
4. Oral ulcers
7. Renal disease
8. Low blood counts
9. Neurological disorder
11. Immunological disorder
Fixed erythema which can be flat or raised over the malar eminences, tending to spare the nasolabial folds.
Butterfly rash that is hallmark of lupus.
Erythematous raised patches with adherent keratotic scaling and follicular plugging.
Atrophic scarring may occur in older lesions. The scarring destroys the hair follicles if the rash is on the head which results in balding.
Hands look mangled in lupus, however there is no erosion of the joints even though they look deformed.
Affects the kidneys and can cause kidney failure.
Often detected in urinalysis.
Lupus Hematologic Manifestations
Can cause leukopenia (50%)
Autoimmune hemolytic anemia (10%)
Inflammation of the serous tissues of the body, such as the linings of the lungs (pleura), the linings of the heart (pericardium), and the inner lining of the abdomen (peritoneum).
Lupus Neurological Symptoms
Cognitive dysfunction is common, and often is a main complaint.
Seizures and psychosis occur in less than 10% of cases.
Lupus - Other Features
Profound and debilitating fatigue
Fever, weight loss, lymphadenopathy
A condition where vasospasm results in white fingers, which then turn blue, and then turn to bright red as they rewarm.
The inflammation of a blood vessel or blood vessels. Arteries and veins can be affected. It is primarily caused by leukocyte migration and resultant damage to the tissue.
Highly specific for the diagnosis of lupus ( >95%)
(Detected on the ENA profile)
Anti-Double Stranded DNA Antibodies
Highly specific for diagnosis of lupus (95%)
SOAP BRAIN MD
Pneumonic for remembering the lupus classification criteria
Blood counts low
Genetics and Lupus
They are important. Concordance studies show that monozygotic twins have a 25-50% concordance for systemic lupus.
Men with Klinefelter's syndrome (XXY) have an increased risk of SLE, suggesting that it might be involved with the X-chromosome.
Genetics are important, but are not sufficient in themselves to cause SLE.
Accelerating Factors: UV Light
UV light can cause flares of the disease by damaging cells and inducing apoptosis. When this occurs, self-antigens become exposed. People with lupus have poor clearance of apoptotic debris in SLE.
Acute Serum Sickness
Excess antigen overwhelms the removal system.
Gets deposited in joints.
Waste Disposal Hypothesis
SLE patients have a defective clearance of debris from dying cells. Because of this, nuclear antigens are "seen" by the immune system. Genetically predispositioned patients make antibodies against self antigens.
Results in a positive ANA or anti-dsDNA
General Management of Lupus
Education and regular rheumatology followups.
Avoid accelerating factors such as sun, sulfa antibiotics, certain vaccinations, and pregnancy planning.
Preventing cardiovascular disease.
Treatment of Lupus
Monoclonal antibody that targets B lymphocyte stimulation.
Has been shown to have a decrease in disease flares.
First new drug to be approved for SLE in decades.
Prognosis of Lupus
20 year survival is >80%
Causes of death are usually active SLE, infections, accelerated atherosclerosis.
Renal failure occurs in 10% of individuals affected.