Systemic Lupus Erythematous Flashcards
What can be defined as an inflammatory autoimmune disorder characterized by autoantibodies to nuclear atigens?
Systemic lupus erythematous
What are many of SLE symptoms secondary to what 2 things?
- trapping of antigen-antibody complexes in capillaries of visceral structures
- autoantibody-mediated destruction of host cells
What is the clinical course of SLE marked by?
spontaneous remission and relapses
What 3 factors influence the incidence of SLE?
- gender
- race
- genetic inheritance
About __% of SLE patients are women and __% are men
85
15
When do the majority of cases begin in women?
After menarche and before menopause
SLE occurs in 1:____ white women and 1:_____ black women
1: 1000
1: 250
* More common in black women
If one identical twin has SLE there is a __-__% chance the other twin will as well
25-70
If a mother has SLE what are her daughter’s risks of developing the disease? What are her son’s?
1 in 40
1 in 250
What disorders are seen in asymptomatic relatives of those with SLE?
Rheumatic diseases (aggregation of serologic abnormalities)
Before making a diagnosis of SLE it is imperative to ascertain the symptoms are not secondary to what?
drug induced
What 3 drugs are considered to induce this type of lupus?
- procainamide
- hydralazine
- isoniazid
What are the 4 features that separate drug-induced lupus from SLE?
1) the sex ratio is nearly equal
2) nephritis and CNS symptoms typically aren’t present
3) hypocomplementemia and antibodies to double-stranded DNA are absent
4) the clinical features and lab abnormalities usually revert to normal after the drug is withdrawn
When should the diagnosis of SLE be suspected?
In patients having a multisystem disease with a positive test for antinuclear antibodies
What are 8 differential diagnoses for SLE?
- Rheumatoid arthritis
- Systemic vasculitis
- Scleroderma
- Inflammatory myopathies
- Viral hepatitis
- Sarcoidosis
- Acute drug reactions
- Drug-induced lupus
The diagnosis of SLE can be made with reasonable probability if _ or the following 11 criteria are found.
- Malar rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis
- Serositis
- Kidney disease
- Neurologic disease
- Hematologic disease
- Immunologic abnormalitis
- Positive ANA in serum
4
What are the systemic features of SLE?
- Fever
- Anorexia
- Malaise (generalized feeling of discomfort)
- Weight loss
What are the cutaneous manifestations of SLE?
- Malar (butterfly) rash
- Panniculitis: (inflammation of subcutaneous fat)
- Discoid lupus
- Fingertip lesions
- Periungual erythema
- Nail fold infarcts
- Splinter hemorrhages
- Alopecia (hair loss)
- Raynaud phenomenon
- Mucous membrane lesions
Raynaud phenomenon is present in __% of patient’s with SLE
20
When do mucous membrane lesions tend to occur?
during periods of exacerbation
Joint symptoms occur in over __% of patients and are often the _____ manifestation
90
earliest
Arthritis associated with SLE leads to what?
reversible swan-neck deformity
What are the ocular manifestations of SLE?
- Conjunctivitis (pink eye)
- Photophobia
- Transient or permanent monocular blindness
- Blurred vision
What do cotton-wool spots on the retina represent?
Degeneration of nerve fibers due to occlusion of retinal blood vessels
What are the pulmonary manifestations of SLE?
- Pleurisy
- Pleural effusion
- Bronchiopneumonia
- Pneumonitis
- Interstitial lung disease (rare)
- Restrictive lung disease
- Alveolar hemorrhage (rare)
What layer covering the heart is affected in the majority of patients?
pericardium
Heart failure associated with SLE may result from what?
- myocarditis
- hypertension
What are the neurological complications of SLE?
- Psychosis (delusions and hallucinations are very common)
- Cognitive impairments
- Seizures
- Peripheral and cranial neuropathies
- Transverse myelitis
- Stroke
What 5 things can lead to SLE flare-ups?
- Stress (most common cause)
- Exposure to the sun
- Infections
- Surgery
- Pregnancy
Antinuclear antibody (ANA) tests are positive in virtually all SLE patients, but are also positive in what 4 nonlupus conditions?
- RA
- autoimmune thyroid disease
- sclerodoma
- Sjögren’s Syndrome
What are the 2 ways in which ANA is tested?
- Immunofluorescence (IFR)
- Enzyme Immunoassay
The IFR test is considered positive for lupus if the blood sample to diluting sample is ____ than 1:40
greater
What are the 3 types of antiphospholipid antibodies?
- Syphilis False Positive
- Lupus Anticoagulant
- Anti-Cardiolipin
Ultimately SLE lab studies should include data from what 3 parameters?
- renal
- hematological
- immunological
All SLE patients should wear SPF__ or greater when going outside
55
What do skin lesions associated with SLE often respond to?
local corticosteroid use
What are minor joint symptoms usually treated with?
rest and NSAIDs
What specific drug assists with treating the rashes, joint symptoms, and severity of symptoms?
Hydroxychloroquine (an anti-malarial drug)
What complex symptoms usually require systemic corticosteroids (prednisone)?
- glomerulonephritis
- hemolytic anemia
- pericarditis / myocarditis
- alveolar hemorrhage
- CNS involvement
- thrombotic thrombocytopenic purpura
When are immunosuppressants used?
when the SLE is resistant to corticosteroid treatment
What is the treatment of choice for pateints with the antiphospholipid syndrome?
anticoagulants (Warfarin)
The 5 year survival rate is __%
The 10 year survival rate is __%
90
85
True or False
SLE follows a relapsing and remitting course.
True
What are the leading cause of death in the early years?
What are the leading cause of death in the later years?
infections
accelerated atherosclerosis
Patients with SLE have an incidence rate _ times higher than normal people for a myocardial infarction.
5
True or False
SLE patients have a higher risk of developing malignancy.
True
Appropriate diagnosis and management of SLE requires the active participation of what HCP?
A rheumatologist
When should you admit a person with suspected lupus?
When they show signs of:
- Rapidly progressive glomerulonephritis - Pulmonary hemorrhage - Transverse myelitis - Severe infection - Other severe manifestations
