SLE 2 Flashcards
Patients with dangerous proliferative forms of glomerular damage (ISN Ill and IV) usually have microscopic hematuria and proteinuria
(>500 mg per 24 h)
20% of individuals with lupus diffuse proliferative ‘
glomerulonephritis (DPGN) die or develop ESRD within
10 years of dx
Approximately 20% of SLE patients with proteinuria (usually nephrotic) have ____changes without
proliferative changes on renal biopsy
membranous glomerular
For most people with lupus nephritis, ____ becomes important after several years of disease
accelerated atherosclerosis
- most common manifestation of diffuse CNS lupus
- difficulties with memory and reasoning
Cognitive dysfunction
When excruciating, they often indicate SLE flare
Headaches
caused by focal occlusion or by embolization from carotid artery plaque or from fibrinous vegetations
of Libman-Sacks endocarditis
Ischemia in the brain
are primarily manifestations of accelerated atherosclerosis
myocardial infarctions
The increased risk for vascular events is 3-to tenfold overall, and is highest in women aged
<49 years
- most common pulmonary
manifestation of SLE - when mild, may respond to treatment with nonsteroidal
anti-inflammatory drugs (NSAIDs); when more. severe, patients require a brief course of glucocorticoid therapy
Pleuritis w/ or without pleural effusion
Life-threatening pulmonary manifestations include
- interstitial inflammation leading to fibrosis,
- shrinking lung syndrome,
- intraalveolar hemorrhage
- most frequent cardiac manifestation;
- usually responds to anti-inflammatory therapy and infrequently leads to tamponade
Pericarditis
More serious cardiac manifestations
- myocarditis
>fibrinous endocarditis of Libman-Sacks
most frequent hematologic manifestation of SLE
Anemia
can be rapid in onset and severe, requiring high-dose glucocorticoid therapy, which is effective in most patients
Hemolysis
common and almost always consists of npnopenia, not granulocytopenia; lymphopenia rarely predisposes to infections and by itself usually does not require therapy
Leukopenia
- a recurring problem
thrombocytopenia
If the platelet counts are ___ and abnormal bleeding is ___, therapy may not be required
- > 40000/uL
- absent
SLE flare
- Nausea,
- vomiting,
- diarrhea
d/t autoimmune peritonitis or intestinal vasculitis
Diffuse abdominal pain
Gastrointestinal Manifestation that is common when active
Increase in AST and ALT
Intestinal vasculitis — LIFE THREATENING; may lead to:
- ischemia
- bleeding
- sepsis
5 EYE PROBLEMS ASSOCIATED WITH LUPUS
- Dry eye disease
- eyelid disease
- retinal disease
- scleral disease
- nerve disease
MOST IMPORTANT to detect; positive in >95% of patients, usually at the onset of symptoms
ANA
SPECIFIC for SLE TEST
High titer IgG antibodies to dsDNA
Also specific test and assist in diagnosis
Antibodies to Sm
not used for diagnosis, but indicates increased risks for neonatal lupus, sicca syndrome, and SCLE
Anti —Ro/ SS-A
not specific for SLE ; if (+) — increased risk for venous or arterial clotting & thrombocytopenia
aPL
Mainstay Tx for SLE
Analgesics and antimalarials
analgesics/anti-inflammatories for arthritis/arthralgias
NSAIDs
Often reduce dermatitis, arthritis and fatigue
Antimalarial (hydrochloroquine, chloroquine and quinacrine)
- reduces accrual of tissue damage, including renal damage over time
- Because of potential retinal toxicity, patients receiving antimalarials should undergo ophthalmologic examinations annually
Hydroxychloroquine
- MAINSTAY FOR SEVERE SLE Proliferative Form of Lupus Nephritis
- High doses are recommended for much shorter period
Systemic glucocorticoids
In patients whose renal biopsies show ISN grade Ill or IV disease, early
treatment with combinations of ____ reduces progression to ESRD and death
glucocorticoids and cyclophosphamide
multiple cellular crescents and/or fibrinoid necrosis on renal biopsy, or rapidly
progressive glomerulonephritis
Severe nephritis
High-dose cyclophosphamide (500-1000 mg/m2 body surface area given monthly IV for
6 months, followed by azathioprine or mycophenolate
maintenance)
Cyclophosphamide and mycophenolate responses begin ____after treatment is initiated, whereas glucocorticoid responses may
begin within ____
- 3—16 weeks
- 24 h
- For maintenance therapy, ____ probably are similar in efficacy and toxicity
- both are safer than cyclophosphamide
- mycophenolate
- azathioprine
common effect of high-dose cyclophosphamide therapy
- ovarian failure
- can be reduced by treatment with a gonadotropin-releasing hormone agonist prior to each monthly cyclophosphamide dose
he number of SLE flares is reduced by maintenance therapy with
- mycophenolate mofetil (1.5—2 g daily) or
- azathioprine (1—2.5 mg/kg per day)
- Both cyclophosphamide and mycophenolate mofetil are potentially
- atients should be off either medication for ___ before attempting to conceive
- can be used if necessary to control active SLE in patients who are pregnant
- teratogenic
- at least 3 months
- Azithioprine
- Good improvement occurs in 80% of lupus nephritis patients receiving either cyclophosphamide or mycophenolate at
- Most patients with SLE of any type should be treated with ___ since it prevents damage in skin and kidney and reduces overall damage scores
- Px w/ proteinuria >500mg daily should receive ___, as they reduce the chance for ESRD
- 1-2 years of follow-up
- hydroxychloroquine
- ACE inhibitors or ARBs
- presence of cellular or fibrotic crescents in glomeruli with proliferative glomerulonephritis
- indicates a worse prognosis than in patients without this feature
- high-dose cyclophosphamide as the induction therapy of choice
Crescentic Lupus Nephritis
- Most SLE patients with it have proliferative changes and should be treated for proliferative disease
- Treatment for this group is less well define
Membranous Lupus Nephritis
- In SLE patients with antiphospholipid antibodies and prior fetal losses, tx with ___ has been shown in prospective controlled trials to increase significantly the proportion of live births.
- Aspirin alone may be used, although most consider it less effective
heparin (usually low-molecular-weight) plus low-dose aspirin
Patients with SLE who have venous or arterial clotting and/or repeated fetal losses and at least two positive tests for antiphospholipid antibodies have APS and should be managed with
long-term anticoagulation
POOR PROGNOSIS (50% mortality in 10 years)
- High serum creatinine levels >1.4 mg/dL
- Hypertension
- Nephrotic syndrome (24hr urine protein excretion >2.6g)
- Anemia (Hgb <12.4 g/dL)
- Hypoalbuminemia
- Hypocomplementemia
- Male sex
