Systemic Lupus Erythematosus Flashcards
Which ethnic groups have higher rates of SLE?
People of African, Asian, and Hispanic descent.
What antibodies increase the risk of neonatal lupus?
Typically, the antibodies responsible are anti-SSA (anti-Ro) and anti-SSB (anti-La).
Which is the most concerning cardiac complication that can be seen in infants with neonatal lupus?
Complete congenital heart block, which occurs in 1-3% of affected infants and requires pacemaker placement.
What are the (4) common features of neonatal lupus?
Rash, cytopenias, hepatitis, and bradycardia from congenital heart block.
What is the typical clinical course for infants affected by neonatal lupus who do not have cardiac manifestations?
Most noncardiac features resolve within 6 months as maternal antibodies disappear.
What is the typical clinical course for cardiac abnormalities in infants born with neonatal lupus?
Cardiac manifestations are permanent and can require lifelong pacemaker dependence.
How do patients with SLE typically present, and what are some (3) common manifestations of disease?
Patients typically present with malaise, fever, and/or weight loss. Common manifestations include arthritis (80-90%), rash (70-80%), and nephritis (50-60%).
Is a positive ANA common in pediatric SLE?
Yes. A positive ANA occurs in almost all pediatric patients with SLE (98-9%).
List 6 immunologic laboratory findings one might expect to find in a patient with SLE.
Positive ANA (98-99%), anti-dsDNA (60-70%), anti-Smith (33%), antiphospholipid (50%), anti-U1 RNP (least common antibody), and hypocomplementemia.
The presence of what two antibodies is associated with renal involvement in patients with SLE?
Anti-dsDNA and anti-Smith antibodies.
With what are antiphospholipid antibodies associated?
These antibodies affect pathways of coagulation and increase the risk of miscarriages, thrombocytopenia, livedo reticularis, and/or blood clots in about 25% of patients.
How do C3 and C4 levels correlate with disease activity in patients with SLE?
Complement proteins are consumed during immune complex formation in active SLE, particularly with nephritis. C3 and C4 often decline with active disease and may normalize with successful treatment.
How is a diagnosis of SLE made?
A patient must meet at least 4 of 11 criteria for SLE diagnosis.
What are the 11 possible criteria used to help make a diagnosis of SLE?
Neurologic disorder (seizures or psychosis); Malar rash; Discoid rash; Photosensitive rash; Oral and/or nasal ulcers; Serositis (pleuritis, pericarditis, peritonitis); Renal disorder (proteinuria or cellular casts); Arthritis; Hematologic disorder (hemolytic anemia, leukopenia, lymphopenia, or thrombocytopenia); Immunologic disorder (+ antiphospholipid ab, anti-dsDNA, anti-Smith, or false-positive syphilis test - either RPR or VDRL); presence of ANA.
Describe the relationship between SLE and renal disease.
Renal involvement in SLE is very common and directly affects both morbidity and mortality.
What are the 6 classes of lupus nephritis?
- Minimal mesangial; 2. Mesngial proliferative; 3. Focal; 4. Diffuse segmental/Global; 5. Membranous; 6. Advanced Sclerosing.
What is the most common cause of chorea in the U.S.?
SLE
What laboratory testing should be performed if a patient with SLE presents with CNS dysfunction?
Lumbar puncture (b/c infection and hemorrhage are also possible in these patients). Serum antiribosomal P antibodies and antineuronal antibodies from the CSF can help to diagnose lupus psychosis or lupus cerebritis.
What are the three characteristic skin findings in SLE?
Butterfly/Malar rash, discoid rash (uncommon in childhood SLE), and photosensitivity.
Which two autoimmune diseases are signified by the finding of a malar rash?
A malar rash is usually due to either SLE or dermatomyositis.
How would one distinguish between the malar rash of SLE/dermatomyositis and the similar-appearing rash associated with psoriasis and rosacea?
The malar rash involves the malar eminence but spares the nasolabial folds. The rash of psoriasis/rosacia involves the malar eminence but the nasolabial folds may also be involved.
Which hair finding is common in SLE?
Alopecia
What are the typical characteristics of mucosal lesions associated with SLE?
The lesions/ulcerations are typically painless and most commonly located on the hard palate or in the nares.
What is subacute cutaneous lupus erythematosus?
It is uncommon and is characterized by a vesicular or bullous rash with the presence of anti-SSA and anti-SSB antibodies. This is the same rash and antibody profile seen in neonatal lupus. Some patients go on to develop full SLE, but may don’t and have less severe disease course.
