November 19, 2015 - Lupus

Question 1

Classification Criteria

Answer 1

Must have 4/11 criteria for classification, not diagnosis.

1. Malar rash
2. Discoid rash
3. Photosensitivity
4. Oral ulcers
5. Arthritis
6. Serositis
7. Renal disease
8. Low blood counts
9. Neurological disorder
10. ANA
11. Immunological disorder

Question 2

Malar Rash

Answer 2

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.

Question 3

Discoid Rash

Answer 3

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.

Question 4

Jaccoud’s Arthropathy

Answer 4

Hands look mangled in lupus, however there is no erosion of the joints even though they look deformed.

Question 5

Lupus Nephritis

Answer 5

Affects the kidneys and can cause kidney failure.

Often detected in urinalysis.

Question 6

Lupus Hematologic Manifestations

Answer 6

Can cause leukopenia (50%)

Lymphopenia (85%)

Thrombocytopenia (15%)

Autoimmune hemolytic anemia (10%)

Question 7

Lupus Serositis

Answer 7

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).

Question 8

Lupus Neurological Symptoms

Answer 8

Cognitive dysfunction is common, and often is a main complaint.

Seizures and psychosis occur in less than 10% of cases.

Question 9

Lupus - Other Features

Answer 9

Profound and debilitating fatigue

Fever, weight loss, lymphadenopathy

Raynaud’s phenomenon

Vasculitis

Question 10

Raynaud’s Phenomenon

Answer 10

A condition where vasospasm results in white fingers, which then turn blue, and then turn to bright red as they rewarm.

Question 11

Vasculitis

Answer 11

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.

Question 12

Anti-Sm

Answer 12

Highly specific for the diagnosis of lupus ( >95%)

(Detected on the ENA profile)

Question 13

Anti-Double Stranded DNA Antibodies

Answer 13

Highly specific for diagnosis of lupus (95%)

Question 14

SOAP BRAIN MD

Answer 14

Pneumonic for remembering the lupus classification criteria

Serositis

Oral ulcers

Arthritis

Photosensitivity

Blood counts low

Renal disease

ANA

Immunological disorder

Neurological disorder

Malar rash

Discoid rash

Question 15

Genetics and Lupus

Answer 15

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.

Question 16

Accelerating Factors: UV Light

Answer 16

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.

Question 17

Acute Serum Sickness

Answer 17

Excess antigen overwhelms the removal system.

Gets deposited in joints.

Question 18

Waste Disposal Hypothesis

Answer 18

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

Question 19

General Management of Lupus

Answer 19

Education and regular rheumatology followups.

Avoid accelerating factors such as sun, sulfa antibiotics, certain vaccinations, and pregnancy planning.

Preventing cardiovascular disease.

Preventing osteoporosis.

Question 20

Treatment of Lupus

Answer 20

NSAIDs

Antimalarial drugs

Corticosteroids

Immunosuppressive drugs

Question 21

Belimumab

Answer 21

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.

Question 22

Prognosis of Lupus

Answer 22

20 year survival is >80%

Causes of death are usually active SLE, infections, accelerated atherosclerosis.

Renal failure occurs in 10% of individuals affected.