Ch 55 Systemic Lupus erythematosus Flashcards
SLE is a disease of unknown etiology in which tissues and cells are damaged by autoantibodies and immune complexes directed at one or more components of cell nuclei
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lupus improves in 1/3 of women
1/3 unchanged
1/3 worsen
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during pregnancy, major morbidity 1:20 chance of life threatening event. generally these are due to
renal impairment, myocarditis, or serositis
complications associated with preeclampsia and antiphospholipid antibody syndrome are worrisome
pregnancy outcome in SLE improved if:
- lupus activity has been quiescent for 6 months
- no active renal involvement manifest by proteinuria or renal dysfunction
- superimposed preeclampsia does not develop
- no evidence of antiphospholipid antibody activity.
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neonatal lupus is unusal syndrome characteraized by skin lesions - lupus dermatitis
and variable degree of hematological and systemic derangements and occasionally congenital heart block
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recurrence rate for neonatal cutaneous lupus is
25%
anti -SS-A (Ro)
and anti -SS-B (La)
antibodies may lead to diffuse fetal myocarditis and fibrosis in region between atrioventricular node and bundle of His causing
CONGENITAL HEART BLOCK.
HOWEVER; INFANTS OF WOMEN WITH ANTIBODIES TO SS-A AND SS-B ANTIGENS, INCIDENCE OF ARRHYTHMIA IS ONLY 3%
OF THOSE AFFECTED, CARDIAC LESION IS PERMANET AND A PACEMAKER IS GENERALLY NECESSARY.
RECURRENCE RISK FOR CONGENTIAL HEART BLOCK IS 10-15%
CLINICAL MANIFESTATION OF LUPUS
fatigue, malaise, fever, weight loss - 95%
arthralgias, myalgias, polyarthritis, myopathy - 95%
anemia, hemolysis, leukopenia, thrombocytopenia, lupus anticoagulant - 85%
malar (butterfly) rash, discoid rash, photo sensitivity, oral ulcers, alopecia, skin rashes - 80%
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diagnosis of systemic lupus erythematosus
4 or more of the following 11:
identification of antinuclear antibodies (ANA) is best screening tool; however, a positive test not specific for lupus. antibodies to double stranded DNA (dsDNA) and to Sm ( smith ) antigens are relatively specific for lupus.
lupus versus preeclampsia - eclampsia
preeclampsia is common in women with lupus and superimposed preeclampsia is encountered even more often in those with lupus nephropathy.
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management of SLE:
1. frequent hematological evaluation and assessment of renal and hepatic functions are suggested to detect changes in disease activity during pregnancy and puerperium.
prognosis is worsened with lupus flare, significant proteinuria, renal impairment, and asscoiated hypertension and/or development of preeclampsia.
unless HTN develops, there is evidence for fetal compromise or growth restriction, pregnancy is allowed to progress to term.
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