Download 病案討論 -Systemic Lupus Erythematosus

病案討論
-Systemic Lupus Erythematosus
曾素卿 2006/09
The case

General patient information
Name:林××
Age:40
Gender:female
Education: high
Marital status: married
Occupation: office lady
Admission date:94/04/22
Discharge date:94/04/25
Chart No:61591622

Chief complaint
Progressive lower limb edema and gain of body
weight for about 10 days

Present illness (Ι)
A 40-year-old woman was a case of SLE and
diagnosed at 南門醫院 at age 19. She was under
treatment at 東元醫院 and had received several
cycles of pulse therapy.
She had operation history of endometriosis s/p on
MMH in 1994 and pregnancy with abortion (due to
fever) for two times and an episode of interauterine
death, followed by acute renal failure s/p
hemodialysis for 3 months.

Present illness (Ⅱ)
She sustained from repeated cystitis and PIP in
2001/10 and followed by HPV infected
vaginitis in 2002/7. Type Ⅳ lupus nephritis was told
from renal biopsy at
台大醫院. Mrs.Lin appeared to our clinic in 2004/2/6
with severe Raynaud’s phenomenom and digital
vasculitic purpura which were also present over
elbows, lower limbs and pretibial area.

Present illness (Ⅲ)
She was admitted to received pulse
therapy and cystitis with vaginitis treatment
during 93/05/14-93/05/20. She was admitted
twice to our hospital due to finger and toe tips
vasculitis and malar rash (2004/5, 2004/7) and
herbal medicine induce flare.

Present illness (Ⅳ)
Due to her eager for baby, she refused treatments as pulse
and cytotoxics. However, recurrent lower limb edema with
heavy proteinuria despite prednisolone and azathiprine.
Hydroxychloroquine was withheld due to patient’s fear of
skin hyperpigmentation.

Present illness (Ⅴ)
Her laboratory tests resulted in
dsDNA=31.9>42>95>626>1850>3100>322>235>61>78>
44.5>144>10.9>40
C3/C4=25/10>26/10>38>57>50
ESR=41>75>91>83>72>53>51>57>50>49
alb=2.7>2.5>3.5>2.0 Uprotein=2.77>2.05>5.44>4.7g/day

Present illness (Ⅵ)
aCLIgG=7.8>17.3GPL/ml(N<10),
aCLIgM=3.2>9.8MPL/ml(<7) within 4 months. The
antibodies included Sm(-) RNP(+) SSA(-) SB(-)
cryoglobulin(-).
Marked lower limb edema developed for the past one
week with difficulty performing daily activity.Albumin
level on 4/19 yielded 2.0. She is admitted for albumin
supplement and also pulse therapy.
Past Medical History
Systemic lupus erythematosus
Hyperlipidemia (mixed type)
Severe Raynaud’s phenomenon

Physical Examination
Vital sign:T:36.3 P:98 R:20 BP:120/74
Status:ill-looking
Cons:clear, alert
General:recent weight change(+) fatigue(+)
gain of body weight:3-4 kg/10 days
Skin:rash(+) lumps(+) pruritus(+)
Extremities:bilateral legs pitting edema L’t＞R’t

Dx
1.Systemic lupus erythematosus with lupus
nephritis class Ⅳ with peripheral
vasculitis with flare(hypoalbuminemia,
heavy proteinuria) s/p pulse therapy
2.Acute pharyngitis
3.Hyperlipidemia(mixed type)
4.Severe Raynaud’s phenomenon
5.Insomia

Drug profile
藥物劑量/頻次/使用
方法/時間
4/22
Prednisolone(5 mg)
tab po
4/23
4/24
6# st
Methylprednisolone
(500 mg) 2 vial in
D5W 100 cc iv drip
4# qd
qd
Albumin 25% 1 BT iv
drip
qd
qd
qd
Lasix(20 mg) 1 amp
post albumin iv
qd
qd
qd
Lasix (40 mg) tab po
1#bid 1#q8h 1#q8h
Lipitor(10 mg) po
Brown mixture
15 cc po
4/25
qd
1#qd
1#qd pm
qid
qid
qid
qid

Problem list
1.SLE with lupus nephritis classⅣ with peripheral
vasculitis
2.Hypoalbuminemia
3.Hyperlipidemia(mixed type)
Discussion SLE with lupus nephritis classⅣ with
peripheral vasculitis

S:
progression lower limb edema and gain of body
weight for about 10 days

O:
1.Gain of body weight:3-4 kg/10 days
2.Extremities:bilateral legs pitting edema L’t＞R’t
Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis
O:
3.Lab data(一)
unit
Normal range
4/19
4/22
4/23
Hb
gm/dL
11.0-16.0
13.5
12.3
12.0
WBC
10ˆ3/
ul
4.00-10.00
9.10
8.60
7.00
RBC
10ˆ6/
ul
4.20-5.50
3.99
3.83
Ht
％
34.0-50.0
35.9
34.6
MCV
fl
80.0-98.0
90.0
90.3
Platelet
10ˆ3/
ul
140-450
211
Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis
O:
3.Lab data(二)
unit
Normal range
4/19
Anti-dsDNA IU/ml
negative:<10
equivocal:1015
positive:>15
44.3
E.S.R.1hr
0-12
85
mm/HR
Creatinine MG/DL
0.5-1.1
4/23
4/24
0.3
0.4
Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis
O:
Lab data(三)
24 hrs urine (1900 ml)
Protein
452 MG/DL= 8.58 g/day
Creatinine H 59.7 MG/DL=ClCr=196 ml/min
Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis
Assessment:
1.This patient is belong to WHO grade IV,
[diffuse proliferative lupus nephritis (DPLN)
affecting >50% of glomeruli] aggressive
immunosuppression is recommended.
If untreated, develop end-stage renal disease
(ESRD) within 2 years.

Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis
Assessment:
2.This patients with dangerous proliferative forms of
glomerular damage and proteinuria (>500 mg per 24 h)
therefore, aggressive immunosuppression is indicated
(usually systemic glucocorticoids plus a cytotoxic drug)
but proteinuria is less likely to improve on lupus nephritis
immunosuppressive therapies. Lupus nephritis tends to be
an ongoing disease, with flares requiring re-treatment over
many years.

Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis
Assessment:
3.Treat with monthly intravenous
cyclophosphamide,500 to 1000 mg/m2 body
surface area for 6 months,along with high-dose
corticosteroids (usually initially pulse
methylprednisolone,1000 mg/d for 3
days,followed by prednisone,40 to 60 mg/d,for the
first month), may be benefit for this patient.

Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis
Assessment:
4. Due to her eager for baby, this patient
refused treatments.
Therefore consider other drugs like
azathioprine or mycophenolate.

Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis

Assessment:
5. Azathioprine (a purine antagonist) added to
glucocorticoids probably reduces the number of SLE flares
and the maintenance glucocorticoid requirement; however,
this approach requires several months to be effective, and
cyclophosphamide is effective in a higher proportion of
patients. Daily oral azathioprine may have fewer adverse
effects than daily oral cyclophosphamide;
Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis
Assessment:
6.A recent prospective study in Chinese patients
with lupus nephritis comparing daily oral
mycophenolate plus prednisolone for 12 months to
daily oral cyclophosphamide plus prednisolone for
6 months followed by oral daily azathioprine plus
prednisolone showed good improvement in 80%
of patients in both groups at 1 year of follow-up
and fewer adverse effects with mycophenolate.

Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis

Assessment:
7.Pregnancy and lupus:
this patient should avoid becoming pregnant
before disease stable.
Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis

Plan:
1.Recommended drug treatment:
pulse therapy :systemic glucocorticoids(0.5~2mg/kg per
day orally or 1000 mg of methylprednisolone sodium
succinate intravenously daily) for 3 days.
maintenance therapy: combine corticosteroids(5~10
mg/day)and immunosuppressive drugs[azathioprine (1~2
mg/kg/d) or mycophenolate(500~1500 mg/d)].
Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis
Plan:
2.Goal:
There is no cure for SLE, and complete sustained
remissions are rare. Therefore, the physician should plan to
control acute, severe flares then develop maintenance
strategies that suppress symptoms to an acceptable level
and prevent organ damage.

Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis

Plan:
3.Monitoring parameters:
It is useful to follow tests that indicate the status of organ
involvement known to be present during SLE flares. These
might include hemoglobin levels, platelet counts,
urinalysis, and serum levels of creatinine or albumin.
There is great interest in identification of additional
markers of disease activity. Candidates include levels of
anti-dsDNA antibodies, several components of
complement (C'3 is most widely available), activated
complement products, soluble interleukin (IL)2, and
urinary monocyte chemotactic protein 1.
Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis
Plan:
4.Patient education
· Stress the importance of compliance with drug regimen
and follow-up visits.
· When starting treatment with prednisone, inform
patients about weight control, low-fat diet, and
exercise.
· Advise patients about proper use and side effect
profiles of other medications used in treating SLE.
· Counsel the patient about the importance of developing
a social support system that will provide feedback
about lupus self-management behaviors, problem solving,
and alternate solution planning.

Discussion SLE with lupus nephritis classⅣ with peripheral
vasculitis

Plan:
5.Institute measures to prevent steroid-induced
osteoporosis.
.Vitamin D and calcium supplementation
. Weight-bearing exercise
.Bisphosphonates or HRT; unless otherwise
contraindicated, alendronate and HRT can both be used.
Discussion Hypoalbuminemia
S/O:
1.Lab data

unit
Normal
range
Albumin GM/DL 3.5-5.0
4/19
4/22
4/23
2.0
1.9
2.2
Discussion Hypoalbuminemia
 Assessment:
1.Hypoalbuminemia,a characteristic feature of the
nephrotic syndrome, results from augmentation of
both urinary albumin lossess and the catabolic rate
of albumin.
2.This is a temporary measure, since the infused
albumin only transiently increase serum albumin
and is promptly excreted in the urine.
Discussion Hypoalbuminemia

Plan:
1.Recommended drug treatment:
25% albumin 1BT iv drip, then
furosemide(40mg) 1 amp for 3 days.
Discussion Hypoalbuminemia

Plan:
2.Goal:
treatment of the underlying disorder and an
adequate protein diet combined with
intervention aimed at reducing protein
excretion may be beneficial.
Discussion Hypoalbuminemia

Plan:
3.Monitoring parameters:
periodic measurement of serum albumin
levels.
4.Diet:
an adequate protein diet.
Discussion Hyperlipidemia(mixed
type)
S/O:
1.Lab data

unit
Total
MG/DL
Cholesterol
Triglyceride MG/DL
Normal
range
130-230
4/23
35-165
335
321
Discussion Hyperlipidemia(mixed
type)

Assessmrent:
1.Lipid abnormalities including TC and TG have been
described in this patients.
2.This patients are at an increased risk for atherosclerotic
cardiovascular disease. Since the lipid abnormalities seen
in nephrotic syndrome are associated with accelerated
atherosclerosis.
Discussion Hyperlipidemia(mixed
type)

Plan:
1.Recommended drug treatment:
Atorvastatin 10 mg qd am(without regard to
time of day and with food if desired)
2.Acquire other lipoprotein level eg. LDL,
HDL.
Discussion Hyperlipidemia(mixed
type)

Plan:
2.Goal:
a. Reduce the risk of atherosclerosis in patient with SLE.
b. NCEP( National Cholesterol Education Program)-ATPⅢ
suggested TC level<200 mg/dl and TG<150 mg/dl.
Discussion Hyperlipidemia(mixed
type)

Plan:
3.Monitoring parameters:
Lipid levels after 2-4 wks; LFTs, CPK.
It is recommended that liver function
tests(LFTs) be performed prior to and at 12
wks following both the initiation of therapy
and elevation in dose, and periodically (eg.
semiannually) thereafter.
Discussion Hyperlipidemia(mixed
type)

Plan:
4.Patient education:
Dietary therapy and lifestyle modifications should
be tried for 3 months.
Nondrug and drug therapy should be initiated
simultaneously. Increasing physical activity will
aid in the treatment of hyperlipidemia and
improve cardiovascular health.
謝謝聆聽,敬請指教!!
1.WHO has classified lupus nephritis as six grades, the patient belong to
grade ?
(d) a.Ⅰ(no histologic changes) b.Ⅱ(proliferative changes confined to the
mesangium) c.Ⅲ(proliferative changes in tufts of 10 to 50% of
glomeruli) d Ⅳ(diffuse proliferative affecting>50% of glomeruli)
2.Which drug has become the standard drug used for controlling life(a) threatening active lupus nephritis, particularly in patients whose renal
biopsies show WHO grades III, IV, and V proliferative or
membranoproliferative forms of nephritis?
a.cyclophosphamide b.azathioprne c.mycophenolate
3.Which one is side effect of cyclophosphamide?
(e) a. high rate of irreversible ovarian or testicular failure b. nausea c.
malaise d. alopecia e.以上皆是
4.Wnen flare, anti-ds DNA and serum C3 will be?
(c) a. high anti-ds DNA , high C3 b. low anti-ds DNA , low C3 c.high antids DNA , low C3 d. high anti-ds DNA , high C3.
5.使用statins藥物時,要監測何值?
(d) a. lipid level b. liver function tests c. CPK d.以上皆是
Document at least four of the following American
College of Rheumatology classification criteria











Positive ANA
Malar rash
Discoid lupus
Photosensitivity
Oral ulcers
Arthritis
Serositis
Renal disorder
Neurologic disorder
Hematologic disorder
Immunologic disorder
azathioprine
mycophenolate
prednisolone
懷孕分級
D
C
C
1.If the benefits from use in pregnant women may be
acceptable despite the risk (e.g., if the drug is needed in a
life-threatening situation or for a serious disease for which
safer drugs cannot be used or are ineffective).
2.Maternal SLE should be controlled with
prednisone/prednisolone at the
lowest effective doses for the shortest time required.
懷孕分級
statins
fibrate
cholestyramine
ezetimibe

X
C
C
C
Atherosclerosis is a process that occurs over
time, and discontinuing lipid-lowering
agents during pregnancy is not likely to
adversely affect the overall outcome of
hypercholesterolemia treatment