II.

Systemic Connective tissue diseases

(collagen-vascular diseases)
I.
II.
III.
IV.
V.
VI.

SLE
Scleroderma
Vasculitis
MCTD and overlap syndrome
Antiphospholipid syndrome
Polymyositis and dermatomyositis

II. Systemic Connective tissue diseases

(collagen-vascular diseases)

SLE (Systemic Lupus Erythematosus)

autoantibody

,

ACR

SLE: etiology

Genetic: HLA DR2, DR3

autoantibody

B-cell, T-cell, NK cell, macrophage

Hormone: female > male

SLE ACR criteria (> 4 items)

1.
2.
3.
4.
5.
6.

7.
8.
9.

Malar rash
Discoid rash
Photosensitivity
Oral ulcer
Nonerosive arthritis
Serositis:
: protein > 0.5 g/d 3+
CNS: epilepsy, psychosis
Hematology: , WBC < 4,000,
lymphocyte < 1,500, Plt < 100,000

10.

Immunology
1.
2.
3.
4.

11.

LE cell
dsDNA
Anti-sm
False positive VDRL

ANA

SLE: Clinical manifestation

1.

2.

: 37.8 C , , , ,

85% : :
RA
avascular necrosis
steroids

3. : 80%
1.

Acute Cutaneous LE: butterfly malar rash

2.

cutaneous LE discoid lupus (DLE)

Subacute cutaneous LE (SCLE):

50% Anti-Ro(SSA)
SCLE
60%

4. Lupus Nephritis: 70%

HTN, UA: RBC,WBC, cast

ARF, CRF
Proteinuria > 1g/ 24 hrs.

5. Hematology: 77%

Anemia
Coombstest 40-50%
WBC 2,500-3,500/ml3: granulocyte, lymphocyte
steroids WBC 15,000-25,000/ ml3

6. 20-25% :

(grand mal status epilepticus)

: depressive disorder, overt psychosis:
schizophrenia

5% Steroid psychosis: dose dependent > 40 mg/d:

5 2-3

, CVA

7. 20-22%:

Pleuritis

8. CVS: 17-28%

Pericarditis Pleuritis

9. GI 5-20%: N, V, anorexia, dyspepsia

SLE

Pregnancy:

Steroids

Drug induced Lupus:

Hydralazine, procainamide
Quinidine, chlropomazine, isoniazid
, , . ,
Anti-histone

SLE: Laboratory investigation

Diagnosis: Anti-dsDNA 50%, Anti-Sm 30%

Follow-up/ prognosis:

CBC: thrombocytopenia
UA: Proteinuria > 1 g/d
Scr
Alb/ Glb:
ESR/ CRP:
Anti-dsDNA + C4 20-25 wk C3
Biopsy:

SLE: Prognosis

Control factors: , , , ,

2-10%

. . , ,

30 prognosis 50

SLE Treatment

Mild SLE

Local corticosteroids
Chloroquine 250 mg/d
NSAIDs: analgesic

Moderate SLE: serositis, fever,UA cast, proteinuria < 1

g/d

NSAIDs
AZA 1-2 mg/kg/d
Prednisolone 15-30 mg/d

SLE

Severe SLE: severe and rapidly

High dose predisolone 1-2 mg/kg/d oral or

dexamethasone IV 200-500 mg/d titer
Immunosuppresive therapy:

Azathiprine
Cyclophosphamide

Maintainance therapy

Low dose prednisolone, AZA, cyclosporine, CQ

SLE Complications

Infection : fever, high PMN or band, high CRP

Avascular necrosis: from steriods
Raynauds phenomenon, APL,