(systemic sclerosis)

systemic sclerosis

sclerodema


scleroderma
cutaneous sclerosis 2
1. localized cutaneous sclerosis: morphea linear
scleroderma
2. systemic cutaneous sclerosis systemic sclerosis (SSc):

SSc 2
1. limited cutaneous systemic sclerosis (LSSc)


2. diffuse cutaneous systemic sclerosis (DSSc)

systemic sclerosis
1 / 100,000
40-50 ~ 2:1

3
1. edematous inflammatory phase

2. indurative phase
(Raynauds phenomenon)
(digital pitting scar)
3. atrophic phase



salt-pepper appearance

1-2


1-2

SSc
1.
1.1
1.2
2

2.

3.
4.
5.
6.
7.
8.

1.3
1.4
1.5 Raynauds phenomenon
1.6
:
2.1
2.2 : vinyl chloride, adulterated rapeseed oil, L-Tryptophan,
Nicotine
:
:
:
:
:

1.
1.1 :
1.2 (salt and pepper pattern):
1.3 Telangiectasia microcapillary dilatation
1.4 (pitting scar)
1.5 Raynauds phenomenon (digital gangrene)
1.6 (calcinosis cutis CREST syndrome) :
2.
2.1
a. (respiratory rate)
b. course crepitation diffuse interstitial fibrosis
2.2
a. secondary Sjogren syndrome
b. bowel sound
pseudogut obstruction
2.3
3

a. periarticular fibrosis arthritis

b. (myositis)
(muscle fibrosis)
c. tendon friction rub :
2.4
a. arrhythmia cardiac fibrosis
b. pericardial friction rub pericardial effusion pericarditis
c. dilated cardiomyopathy
d. P2 primary secondary pulmonary hypertension
2.5
a. renal crisis
b. renal involvement renal crisis
3. systemic sclerosis American College of
Rheumatology 1 2
97
1. major criterion proximal scleroderma ( MCP joint )
2. minor criteria
a. sclerodactyly ()
b. digital pitting scars fat pad
c. bibasilar pulmonary fibrosis

3
1. : skin
biopsy

2.
a. CBC: MAHA ( renal crisis) nutritional anemia

b. Renal function: urinalysis, creatinine, BUN, electrolyte, albumin, globulin

renal crisis, nephritis
c. Muscle enzymes myositis
4

d. CXR : bibasilar fibrosis, cardiomegaly (option: pulmonary function test,

DLCo BAL active pulmonary involvement)
e. EKG : arrhythmia (option: echocardiography,
pulmonary artery pressure cardiomyopathy pulmonary hypertension)
f. GI study: dysphagia supportive
mechanical obstruction
endoscope
g. Renal biopsy (option) progressive renal failure renal crisis
nephritis ( MCTD, overlapping syndrome)
3. : serology

a. ANA pattern titer

b. Anti-centromere antibody CREST syndrome (option)
c. Anti-Scl-70 anti-topoisomerase I diffuse SSc (option)
d. Anti-U1RNP antibody overlap syndrome MCTD (option)
e. Anti-PM-Scl antibody scleroderma /polymyositis overlap (option)
f. Anti-DNA antibody, anti-Sm antibody (option)

1.

2.

3.

1. :

2. :
a. vibration
b.

c.
10-15

d.

e.
f.
reflux
esophagitis 3
g. 1
h.
1)
2)
3)
4)
5)
6) 3
7)
8)
9) 2 1
3.
3.1 :

1) Colchicine (2):
1 (0.6 .) 2-3

2) Chloroquine 250 mg/day hydroxychloroquine 200-400 mg/day (3)

3) ASA gr I-V (3)
6

4) Prednisolone (< 0.5 mg/kg) edematous phase

indurative phase ( prednisolone 20-30 mg /day
renal crisis ) (2)
5) : topical cream base (1), 10% urea cream
moisturizer (1), (2)
3.2 Raynauds phenomenon digital ulceration
1) low dose ASA (3)
2) calcium channel blocker (2) nifedipine 10-20 . 3

3) digital gangrene pentoxyphilline (3) 2-3 tab/day

calcium channel blocker
4)
3.3
1) H2-blocker: gastroesophageal reflux cimetidine
800 2 400 mg 4 Ranitidine 150 . 2
(2)
2) Proton pump inhibitor H2 blocker
H2 blocker omiprazole 20 . 2 (2)
3) Domperidone : 1 3
(2)
4) Prokinetic drugs: (2)
5) : tetracyclin (250 mg) 2 cap qid metronidazole (200 mg) 2
tab tid norfloxacin (400 mg) 1 tab bid 5 bacterial
overgrowth
3.4 :
active alveolitis bronchoalveolar larvage (BAL) /
high resolution CT scan (HRCT)
a. Active alveolitis:
1) Prednisolone (1mg/kg) 1-2 (2)
2) prednisolone (10 mg/day) cyclophosphamide 2 mg / kg
1
b. primary pulmonary hypertension:
7

3.5 :
a. active myocarditis prednisolone 1-2 mg/kg/day (2)
b. Arrhythmia, massive pericardial effusion

3.6 scleroderma renal crisis :

1) ACEI (1) captopril (25mg) 1tab 8 ., enalapril (5) 1tab 12 .

2) prednisolone 10 mg/day
3) supportive
4) creatinine > 2mg/dl
5) dialysis
3.7
a) analgesic drug paracetamol, ASA (prn)
b) NSAIDS 2

c) Prednisolone 0.5-1 mg/kg/day active myositis

3.8
3.9
3.10
a. esophageal stricture
b. cardiac temponade

Rating

1.
RCT
2
- RCT

-
-
-
monitor

3
-

- (

monitor
4.
-
-

Recommend

Recommended Treatment in Systemic Sclerosis

Nonphamacologic measure
- Skin:
moisturizing agent
/
- Raynaud :

vasoconstictive agent

- GI :

-
-
-

10


Skin

Raynaud

GI

Pulmonary

Renal
Joint
Sicca
symptoms

D-penicillamine
Colchicine
CQ*
Cyclosporine, ATG,
-INF
Ca channel blocker
- blocker
topical NTG *
IV prostaglandin analog
Oral prostaglandin analog
Pentoxyphyline
ASA, Antiplatelet agent
H2 blocker/PPI
Prokinetic agent
Antibiotic*
Prednisolone*
Immunosupp. Agent**
- Cyclophosphamide
- Azathioprine
- Ca channel blocker***
- IV prostacyclin***
- ACEI*
- Antihypertensive agent**
- Analgesic*
- NSAID**
- Artificial tear
- Artificial saliva
- Pllocarpine

Rate

4
2 or 3
3
* vasculitis
4
2
2 or 3
3
2 or 3
3
3
3
2
2
2
1, 2
2
2 or 3
2 or 3
3
1
2
2
2
1, 2
2
3

* Ca channel block
reflux

GI symptoms
* bacterial overgrowth
*Vasculitis, myopathy, pericarditis
**pulmonary involvement
***Pulmonary HT

*Renal crisis
** ACEI control BP
* arthralgia/arthritis
** arthritis
Keratoconjunctivitis sicca
Xerostomia
Xerostomia

11


* Hx & PE
- BP
- Urine
-

** Investigation
CBC, Blood smear, U/A
BUN/Cr, CXR, EKR

GI
-H2 blocker PPI
-Prokinetic drug
-antibiotic

* 1
** 2

Arthritis
-Paracetamol
-NSAIDs

Skin
-Colchicine
-Chloroquin
-ASA
-prednisolone

F/U

internist

rheumatologist

Raynaud

-ASA
-Calcium channel

internist
Pulmonary (active alveolitis ): Prednisolone
: prednisolone + Cyclophos
blocker
.
phamide
Renal crisis : ACEI
: Dialysis
: Supportive
Myositis : prednisolone
Active myocarditis : prednisolone

- 2

Other dis.

F/U

rheumatologist

12

1.
2.
3.
4.
5.
6.
7.
8.
9.

digital gangrene
2 1
2
CBC, Blood smear
U/A, BUN/Cr, Electrolyte, Albumin, Globulin
CXR
EKG

13