SYSTEMIC SCLEROSIS

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SYSTEMIC SCLEROSIS

• ??' ????? ????-?????
• ????? ???????????
• ???? ????? ??? ?????

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SYSTEMIC SCLEROSIS (SSc)SCLERODERMA

• ???? ?????? ?? ??????? ?????????? ?? ?????.
• ???????? ?"? ????? ??? ????? ???????? ?? ????
  ????? ??? ?"? ?????? ?????? ????????? ?? ??? ??
  ??? ?????? ???????

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SYSTEMIC SCLEROSIS (SSc) PATHOGENESIS
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SYSTEMIC SCLEROSIS (SSc)??? ????? ???????
???????
?????? (LIMITED) ????? ??? ??????, ????? ??????
???????? ?? ?????? (?? ??????? ????????)
????? (DIFFUSE) ????? ???? ????? ?? ??????
????????? ?? ?????? ??"? ????
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????? RAYNAUD

• ?????? ?-95 ????? SSc.
• ????? ?????? ????????? ????????
  (VASOCONSTRICTION) ?? ????? ??????? ?????? ??????
  ???? ?? ???? ????.
• ????? ???-?????.
• ?????? ???? ???? ??? ????? ???? ?? ???? ??????
  ??????????.

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????? RAYNAUD ??? I ??????? ?? ???????
????????, ?? ?? ??????? ????
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????? RAYNAUD ??? II ???????? ?? ???????
????????
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????? RAYNAUD ??? III ??? ????????
??????????.

• ????? ?? ???? ???? ?????
• ???? ?????? ?? ???-??? ??????.
• ????? ?????? RAYNAUD ???????, ????? ??? ??????

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????? RAYNAUD

• ????? RAYNAUD ???????
• 15-4 ?????????? ??????.
• ?????? ???? ?????.
• ????? ???? ?????.
• ????? ?????? ? 30 ???????.
• ??? ???? ??? ????? ????.
• ????? ???????.
• ??? ??????? ?????? ???? ??? ?? ???????.
• ??? ???????.
• ????? RAYNAUD ?????
• ??????? ???? ???? ????? ?? ???? ??? ????.

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????? RAYNAUD ????? NAIL FOLD CAPILAROSCOPY
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????? RAYNAUD ????? NAIL FOLD CAPILAROSCOPY
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????? RAYNAUD ????? ???????
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????? RAYNAUD ????? ???????
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SSc ???? ?????????? RAYNAUD ?????? ??
??????? ?????? ?-SSc

• Diffuse SSc - ???' ?????? ?? ???
• Limited SSc ???' ????

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CREST Limited SSc

• Calcinosis
• Raynauds phenomenon
• Esophagus
• Sclerodactyly
• Telnagictasis

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SSc ???? ????????? ??????

• ???? ?? ????
• ????? ?? ?????? ????
• ????????

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SSc ???? ?????
???? ??????

• ?????? ????????
• FATIGUE (??????)
• ???
• ????????
• ???????

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SSc ???? ????? ???? ?"???????"

• ????? ???
• ????? ????? ?????? ??????
• ??????? ????? (???????????) ?? ???????

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• SSc ???? ?????
• ????? ??? ?????
• ????? ?????
• ???? "????"
• ????? ????? ???

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• SSc ???? ?????
• ????? ??? ????
• ??????? ??????????

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SSc ???? ?????
TELANGIECTASIA
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SSc ???? ????? CALCINOSIS
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SSc ??????? ?????? ???????????? ??????

• ???? ??????
• ??? ?????
• ????? ??? ???? ??' ??????

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SSc ??????? ?????? ??????? ????? ??????

• ????
• ????? ????????? ?????

?????, ????, ????? ?????
????? ?-INTAKE ?????
????? ?????
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SSc ??????? ?????? ??????? ????? ??????

• ??? ??
• ????? ????????? ???? ???
• PSEUDOOBSTRUCTION
• BACTERIAL OVERGROWTH
• MALABSORPTION

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SSc ??????? ?????? ???????????? ??????

• ??? ??
• ?????? ??????
• ??????
• DIVERTICULOSIS

????? ??????? ???????? ??????
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SSc ??????? ?????? ??????? ????? ??????

• ??????? ???
• PRIMARY BILIARY CIRHOSIS
• ?????
• Limited SSc

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SSc ??????? ?????? ????????????

• ??????? ??????

• PULMONARY ARTERIAL HYPERTENSION
• ???? ???? ?- limited SSc

• INTERSTITIAL LUNG DISEASE
• (ILD)
• ???? ???? ?- diffuse SSc
• ????? ?????? ???????? ????? ??????

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ILD ?????? ???????

• Presenting symptom- ???? ????? ?????
• ????? ???????? ??????? ????? ?????? ?????-
  Velcro-like, ??? ???? ???-????? ??

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ILD ?????? ???

• ????? ??? ?????? ????? ?????? ????????
• ???? ????? ???? ?????????????? ?????? ????????

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ILD ?????? ???

• ????? ?????? ????
• ?????? ?????? ????? ???? ??????.
• ????? ????? ????? (FVC)
• ????? ????? ???????? (DLCO).

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ILD ?????? ???

• HIGH RESOLUTION CT (HRCT)
• ????? ????? ?????? ??????? ???????? ?? ?????.
• ?????? ?????? ????? ????
• GROUND GLASS ????? ?- ALVEOLITIS ?????.

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ILD ?????? ???

• BRONCHOALVEOLAR LAVAGE
• ????? ???? ?????
• ???? ????? ?? ???????????, ???????????? ?? CD8-T
  cells
• ??????? ???? GOLD STANDARD

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ILD ????

• ACTIVE ALVEOLITIS

INTERSTITIAL FIBROSIS
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PULMONARY ARTERIAL HYPERTENSION

• ???? ?????
• ??????? ???????? ???????? ??????? ??"? ????
  ?????.
• ???? ????? ?????
• ??????

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PULMONARY ARTERIAL HYPERTENSION

• ????? ??????
• 2P ?????
• ????? ??-????? ?? ????? (???? ?????)

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PULMONARY ARTERIAL HYPERTENSION

• ?????? ???
• ??? ???? ???? ?? ??? ????
• RVH
• right axis deviation

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PULMONARY ARTERIAL HYPERTENSION

• ?????? ???
• ???? ???
• ????? ??? ??? ???? ?????.
• ????? ??? ?? ????????

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PULMONARY ARTERIAL HYPERTENSION

• ?????? ???
• SIX MINUTE WALK
• DOPLER ECHOCARDIOGRAPHY - ????? ????? ????? ??
  ???? ?????? ?????
• ?????? ???? ????? ?????? ????? ????????, ???
  ????? ????? ?????.
• ????? ???? ?????? ????? ?? ??? ?? ?????

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SSc??????? ??????? ??????

• ASPIRATION PNEUMONIA ????? ?????? ?????? ????
• ENDOBRONCHEAL TELANGIECTASIA ?? ??? ?????.
• BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIA
  (BOOP)
• ????? ????????
• ?????? ?????? ?? ?????? ???? ?-ILD

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SSc??????? ??????

• ???? ?????? ???????
• ????? ????????? ????
• ????????? ???? ????? ?????
• ?????????
• ??? ???? ???? ????
  

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SSc ??????? ??????

• PERICARDITIS - ?????, ???? ???????????
• ?????? ??? ?????? ????? ????????? ????
• ???? ???? ??? ?? ??? ???? ??? ?? ?????
• PATCHY MYOCARDIAL FIBROSIS - ???? ?????
  ???????-???????? ????? ?????? ?????????
• ?? ????? ?? (????? - ????? ???????? ??????, ??
  ??????)

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SSc ??????? ??????

• ?????? ?????? SCLERODERMA RENAL CRISIS
• ACCELERATED HYPERTENSION ?/?? RAPIDLY PROGRESSIVE
  RENAL FAILURE
• 10 ????? SSc (???? diffuse )
• ?-4 ????? ????????

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SSc??????? ??????

• SCLERODERMA RENAL CRISIS - ????? ?????
• ??????? ????? ?? ????, ???????
  ???????
• FRICTION RUB ??? ??????
• ????? ???? ??? ??????
• ?????? ??????? ?????? anti-RNA Polymerase III
• ????? ???? ?????????????????

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SSc??????? ??????

• SCLERODERMA RENAL CRISIS ????? ??????
• ?????? ????????? ?-MALIGNANT HYPERTENSION
• ??? ??? ?? gt 150/90
• ?????? ???? ???????? ?????
• NORMOTENSIVE RENAL CRISIS

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SSc??????? ??????

• SCLERODERMA RENAL CRISIS ?????? ?????
• ????????? ?????
• ???????????
• ??????? ???????????
• MICROANGIOPATHIC HEMOLYTIC ANEMIA ??
• ?????????????? -(????? ?-NORMOTENSIVE
  SCLERODERMA RENAL CRISIS )

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SSc????? ?????? ??????? ?????????

• ????? ?????? ??????? ???? ???? ??????
• ENTRAPMENT NEUROPATHIES
• ?????? ?-50 ???????
• MALE IMPOTENCE

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SSc?????
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SSc????? ?????

• RISK STRATIFICATION IN SSc
• ??????? ?????? HLA-DR53a???? ?-ILD
• ??????? ??????? ??? ???????? ????? ???????? ??
  ???? ????

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SSc????? ?????

• RISK STRATIFICATION IN SSc - ????
• ??????? ?????????
• Anti-centromere Ab limited SSc
• hypertension
  pulmonary
• ???? ???
  ???
• 2. Anti- topoisomerase 1 (Scl 70) diffuse SSc

Interstitial lung disease Scleroderma renal crisis
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SSc????? ?????

• ?????
• ????? ???? - Modified Health Assessment
  Questionnaire (HAQ)
• ??? - Skin Scoring System ??????
• ????? ???? ??? ??
• ????? ?????? ???? (???????)
• ??? ??
• HRCT
• ?? ??? ??
• ?????

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SSc????? ?????

• ?????
• ????? ???????? ????? ?? ?????? ?????
• Soluble ICAM
• Collagen propeptide
• Soluble IL-2 receptor
• Soluble E-selectin
• Thrombomodulin
• von-Willebrant factor

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SSc????? ?????

• ?????
• ???? ????? ????? ?????? ???????? ?????????? ?-SSc
  
• Immune cell activation
• Vascular damage
• fibrogenesis

????? ????? ???? ????? ????? ?????????????? ????
????? ????? ??????????? ??? ???? ????? ?????
??????? ??? ??
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SSc????? ?????

• ?????
• ????? ????? ????
• ????? ?????? ?? ???? ????
• ??????? ?????? ????? ????? ??????? ?? ???????????
• ???? ?? ???? ??????? ???? ??????
• ????? ????? ??????

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SSc????? ?????

• ?????
• 2. ????? ???????????
• Penicillamine
• Colchicine
• Interferon?
• Interferon ?

?????? ?? ????? ?????? ?????
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SSc????? ?????

• ?????
• 3. ????? ???? ????
• ???????????????? ????? ???? ????? ??? ????
  ????? ?????????? ???? ?????? ????
• ????? gt 15 mg/d ???? ?????? ?????
  ?-scleroderma renal crisis

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SSc????? ?????

• ?????
• 3. ????? ???? ???? - ????
• METHOTREXATE
• CHLORAMBUCIL
• CYCLOSPORINE
• ANTI-THYMOCYTE GLOBULIN
• EXTRACORPORAL PHOTOPHERESIS
• 5 FLUOROURACIL

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SSc????? ?????

• ?????
• 3. ????? ???? ????- ????
• - CYCLOPHOSPHAMIDE ?????? ?- FIBROSING ALVEOLITIS
• ???? ???? ??????? ?? ??????? ????? ?????? ???????
  ???????????? ?? ??????? ?????? ?????.

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SSc????? ?????

• ?????
• 3. ????? ???? ????- ????
• HIGH DOSE IMMUNOABLATIVE THERAPY AND AUTOLOGOUS
  HEMATOPOEITIC STEM CELL TRANSPLANTATION
• ???? ?????? ???? ??????
• ?????? ???????? ?? ?? ???????
• ????? ????? ?????

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SSc????? ?????

• ?????
• 4. ????? ??????? ??? ?? ?????? RAYNAUD
• ????? ???????? ?????? ???? ????? ????
• ??????? ?????? ???????? ??????? ???-??
• ????? ????? ????? ????????????

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SSc????? ?????

• ?????
• 4. ????? ??????? ??? ?? ?????? RAYNAUD - ????
• ?????? ??????? ??? ?? ????? ????? ????,
• ????? ???????? ?-ANGIOTENSIN
• SILDENAFIL (VIAGRA)
• SSRI
• ????? ???????? ?-ENDOTHELIN

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SSc????? ?????

• ?????
• 4. ????? ??????? ??? ?? ?????? RAYNAUD - ????
• 5. PROSTACYCLIN ?????????
• IV ILIOPROST
• IV EPOPROSTERNOL
• IV ALPROSTADIL (PGE1)

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SSc????? ?????

• ?????
• 4. ????? RAYNAUD
• ????? ???????
• Stellate ganglion blockage
• Cervical sympathectomy
• Digital sympathectomy

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SSc????? ?????

• ?????
• 4. pulmonary Hypertension
• ????
• ????? ?????????????
• ????? ??????? ???-??
• IV EPOPROSTENOL- ????? ????? ????? ?????,
  ??????? ????? ????? ??????? ??-?????
  ?? ?????, ??????? ????? ???????? ???? ??? ????????

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SSc????? ?????

• ?????
• SCLERODERMA RENAL CRISIS
• ????? ?????
• ????? ACE ????? ??????
• ????? ?
• CLONIDINE
• MINOXIDIL
• ???????