F???????? RAYNAUD S??????????? ??????????S?????-???????S?????

1
F???????? RAYNAUD S?????????????????????S?????-
???????S?????

• ?????????G??? ???????
• ??????S??????? ??S???????? ?????????

2
F???????? RAYNAUDS

• ?pe?s?d?a a??e??spasµ??,e????µe?a µe ???es?
  st? ???? ? s??????s?a?? f??t?s?, ?a?a?t????eta?
  ap? a??a??? t?? ???µat?? t?? d??µat?? (????t?ta
  -?????s?-e?????t?ta),a?µ?d?e? ?a? p??? t??
  da?t???? t?? ?e????

3
???????? f.Raynaud

• ???t?pa??? (d?? ?????a ????? t?? eµf???s?
  ?p??e?µe??? ??s??)
• ?e?te??pa???

4
????? ?????????T??S F.Raynauds

• S?st?µat??? ?e?µat??? ??s?µata
• S?????de?µ?a, S??, ?????µ??s?t?da/
  ?e?µat?µ??s?t?da,s. Sjogren,??, ???e??t?de?
• ?pa??e?µat??? a?t?a
• ?e???st?? ????st???? µ??a??µ?t??
• F??µa?a
• ß-ap???e?st??, e???taµ???,a?t?s????pt???

5
(No Transcript)
6
??????S? ?ST????S ?? F???????? RAYNAUD
?st????? S?µpt?µata F?s??? e??tas? ???ast???a??
?p???e?µa ??a?µa ??p??sµa F??µa?a ??????µa ????a???e? s.?a?p?a??? s????a ?d??aµ?a ???et?? ?ataß??? ?e??fe????? sf??µ?? F?s?µata ???? st?? ????f?? t?? da?t???? ??ata?a??? t?? t????e?d?? t?? ???????? pt???? ??d?µa t?? da?t???? Ge???? a?µat?? ??? ????sfa????e? ??e?t??f???s? ?e???µ?t??,???, anti-Scl-70
7
S?????????? - ????????S?

• ?. S?S???????? S??????S?
• ?????t?
• ?e?????sµ??? s?????de?µa p.? s.Crest
• ?. ??????S???? S??????S?
• ???f?a
• G?aµµ??? s?????de?µa
• G. ?????????????? S???????

8
Morphea leg
Linear scleroderma
thigh and leg
9
S?S???????? S??????S?? S???????????

• ????s?st?µat??? ??s??
• ?st???????? ?a? ?e?t???????? d?ata?a??? t??
  µ????? a??e???
• ???s? t?? d??µat?? ?a? t?? es?te????? ???????
• ??e???p???s? t?? a??s?p???t???? s?st?µat?? ?a?
  a?t?a??s?a

10
S??????????-??T?G?????

• ?etaß????? d?ata?a?? ???ß?ast??
• ?pe?pa?a???? ????a?????
• ???e?a?? ß??ß? ap?f?a??
• ??e???p???s? a??s???tt???? a?t?a??s?a

11
???t???a ta????µ?s?? S?st?µat???? S??????s??
?at? ACR

• ?e???? ???t????
• S?µµet???? s??????s?, p????s? ?a? d????s? t??
  d??µat?? t?? da?t???? ?a? t?? d??µat?? e???? t??
  ??F ??F a????se??, p?? µp??e? ?a ?ata?aµß??e?
  ?a? ??? t? s?????, t? p??s?p?, t?? t?????? ?a?
  t?? ???µ?.
• ???ss??a ???t???a
• S?????da?t???a
• ????? sta ???a p?? ?ata?e?p??? e?t?p?µa ? ap??e?a
  ?p?d????? ?st?? (?s?a?µ?a)
• ?µf?te??p?e??? ???s? st?? p?e?µ?????? ß?se??
• S?????de?µa ?e???? ? 2 e??ss??a

12
?????t? s?????de?µ?a

• S?????de?µ?a p??s?p??, ???µ??, ?????, ???? t??ß??
  te???t??
• ?a?e?a eµf???s? (s?ed?? ta?t??????lt6µ??e?)
  s?????de?µ?a? µet? t?? eµf???s? fa???µ????
  Raynaud
• Sp?a????? p??sß??? p?e?µ??e?, ?a?d??,
  ?ast?e?te????, ?ef???
• Tet??? a?t?p??????? a?t?s?µata,a???t???
  a?t??e?t??µe??d?a??
• ?a?? p?????s? 10et?? ep?ß??s? 40-60.T??at?? ap?
  p?e?µ????? ???s? ? ?ef???? ???s? (?p??tas? µe
  ep?de???s? t?? ?ef????? ?e?t?????a?)
• ?? a?ast??e?? t?? µetat?ept???? e???µ??
  p???aµß????? t? ?ef???? ???s? ?a? ße?t?????? t??
  ep?ß??s?

13
(No Transcript)
14
(No Transcript)
15
?e?????sµ??? s?????de?µ?a

• S?????de?µ?a p??s?p??, pe??fe????? ????? (??t?
  ap? a????e?/???ata)
• ???? e?????? µet? t?? eµf???s? t?? Raynaud ?
  s?????de?µ?a eµfa???eta? µet? ap? gt6 µ??e?
  fa???µ???? Raynaud
• Sp?a????? p??sß??? µet? ap? ?????a
• ??e?µ????? ?p??tas?
• CRESTcalcinosis,telangiectasias,fa???µe??
  Raynaud,d?s????s?a ??s?f????, s?????da?t???a
• Tet??? a?t??e?t??µe??d?a?? a?t?s?µata
• ?a??te?? p?????s? ep?ß??s?? 70 sta 10 ?????a

16
CREST
17
CREST
18
CREST
19
CREST
20
CREST
21
S??????????????G?OS????? ????G????S T??S???????S

• ??tas? de?µat???? ß?aß??
• ?e??µ??? ???? ?a? ??pt??? p?e?µ????
• ?aµ???te?? spe??aµat??? d????s?
• ?e???a?d?t?da - ??e?t???a?d????af???? d?ata?a???

22
S?????????? -????????S

• ?.?????S?S ??OS?
• ?????t? s?????de?µa
• ??t?a ?a??t??
• Sta p??ta (1-3) ?????a t?? ??s??
• ??????? ß??a?, d?sp???a,µ? µ??s???? ???? st??
  ß?se??
• ??t?????af?aµ? d?a???st???
• ?????a ????? ?????
  ???e??t?da
• HRCT
• ?????a µe??s?????????a?
  ???µes?? ???s?
• ?????????e??d??? ??p??µa (BAL)
• ???gt2.2 ? ??s???f??a gt3,2 ??de???
  ???e??t?da?
• ?????a
• Te?ape?a?????f?sfaµ?d? ???t???ste??e?d?

23
(No Transcript)
24
S?????????? -????????S

• ?.??e?µ????? ?p??tas?
• ?e?????sµ??? s?????de?µa
• ??sp???a
• ?e?t???????? d???µas?e? (DL CO )
• ????a?d?????f?µa?pe?t??f?a ?? ?????a?
• ?p?a ??s?? a?ast??e?? d?a????
• Te?ape?a
  Ca
• ?a??? ??s?? µp?se?t???
• 
  p??sta???????

25
(No Transcript)
26
S??????????-??F???

• ?????t? s?????de?µa (sta p??ta 2-4 ?????a)
• ?ef???? ???s? a?f??d?a eµf???s? a?t???a???
  ?p??tas??, ????????a? ?ef???? a?ep???e?a
• ?????? d?se??(gt10 mg p?e?d????????) µp??e? ?a
  ep?ta?????? t? ?ef???? ???s?
• ??ast??e??
  t?? µetat?ept????
• Te?ape?a e???µ?? t??
  a??e??te?s????
• ??µ????a?s?

27
S?????????? - ??????

• ?e???a?d?a?? ????
• ???s? t?? µ???a?d??? a????µ?a, ??pt?s? t??
  ?a?d?a??? s?sta?t???t?ta?

28
S?????????? - G?S??????????

• ???sß???eta? ???? ? ?ast?e?te????? s????a?
• ??s?f????
• ??te??

29
S?????????? -????????SO????

• ???90
• ??d??? a?t?s?µata
• ?at? t?p??s?µe??s?? l(Scl70) 30- 50 dcSSc
• ?????te??? ???d???? ?ef????? ?a? p?e?µ??????
  s?µµet????
• ?at? ?e?t??µe??d??? (50 -90LSSc)
• ?????te??? ???d???? p?e?µ?????? ?p??tas??

30
S??????????

• ???????() ???????S????()
• ???ß? te???t?? 70 5
• ???µes?? ???s? p?e?µ???? 70 35
• ??e?µ????? ?p??tas? 5 25
• ?ef???? ???s? 20 1
• ACA 5 50 - 90
• Anti-Scl-70 30 10
• ??????? d????e?a ap? µ??e? ?????a
• f.Raynaud
• ??tas? s??????s?? p??? ap? t???
  ??t? ap? t???
• a????e?,???ata ?a? a????e?,
  ???ata
• ???µ? ???
  ???µ?
• ??????s? ?a?? (10et?? ep?ß??s? ?a??te??
  (10et??
• 40-60)
  ep?ß??s? 70)

31
S???????????-T???????

• ??????
• ?e? ?p???e?
• S????O??????
• F.Raynaud?efed?p???,p?a??s???,t?p????
  ??t??d?,p??sta??a?d???,p??sta???????.?p?f???
  ???es?? se ???? ?ap??sµat??
• ??ap?e?st???
• ???e??t?da?????f?sfaµ?d? ???t???e?d?
• ???µes?? ???s?IFN-??
• ??e?µ????? ?p??tas?p??sta???????
• ?ef??? a?ast??e?? t?? µetat?ept???? e???µ?? t??
  a??e??te?s???? (?-???)
• ???s?e?et??? ?S?F (COX 2)

32
S??d??µ? Sjogren

• F?e?µ???d?? d????s? e???????? ad????
• ????st?µ?a- ????f?a?µ?a
• ?? d?aß??t??? a????t?da
• Fa???µe?? Raynaud

33
?????a e??ss???? s?e??????? ad??a
34
Shirmer test
35
Keratoconjuctivitis sicca
36
F?e?µ???de?? µ??s?t?de?

• ????µ??s?t?da
• ?e?µat?µ??s?t?da
• ???s?t?da e? e???e?st??
• ???s?t?da ??s?µ?t?? s??det???? ?st??
• S??
• S?st?µat??? s?????de?µa
• S. Sjogren
• ?e?µat?e?d?? a????t?da

37
?a?a?t???st???

• ????µ??? CPK
• S?µµet???? µ???? ad??aµ?a ?e?t??µe????
• ??????µa de?µat?µ??s?t?da?
• ???pa??t???? t?p?? ??G
• ?a?a?t???st??? e???µata µ. ß????a?  

38
A???????s? µ????? ?s??o?

• 0 ap??s?a s?sp?se??
• 1 as?e???-pa??d??? s?spas?
• 2 s?spas? ep? e???det???s?? t?? ßa??t?ta?
• 3 ????s? ??a?t? t?? ßa??t?ta?
• 4 ? µ. ?s???, a??? s?spas? ?a? ??a?t?
  a?t?st?se??
• 5 f?s???????? µ. ?s???

39
????? ad??aµ?a / ?e?t??????? ad??aµ?as?µa?t???
t? ?st?????

• ?e?t??????? ad??aµ?a - ?st?????
• Ge???e?µ??? a?s??µa ad??aµ?a?
• ??sp???a st?? ??p?s?, a???a???e?, pa?a?s??s?e?,
  spast???t?ta
• ????? ad??aµ?a - ?st?????
• ?d??aµ?a p?a?µat?p???s?? e??as???
• ???ßasµa s???a?, ?t???sµa, ??e?s?
• ??s??µa ß?????/d?s?aµ??a? se ????
• S?????? ap??s?a µ?a?????

40
????? ad??aµ?a

• ??????? e??tas?
• S?????? ap??s?a
• ?t??f?a? (a????? st?d?a)
• ?????? e?a?s??s?a? e?t?? ap?
• ???µ?de?? µ??s?t?de?, f??µa?a, ?p??e??e?e?d?sµ??,
  µetaß?????? µ??p??e?e?
• ?a???s?a ?µ????? ?s????
• ?e?t??µe??????
• Oµ??? ???? (de?t?e?d?)
• ?????a
• ???µ??
• ?a???????te?/tet?a?ef?????
• ???sa??????
• ?e??fe??????

41

• ????? ?a?a?t???st??? ??/??
• ?. ad??aµ?a (s?µµet???? e????)
• ?. at??f?a s?????? µ??? sta p??????µ??a st?d?a
• ?. e?a?s??s?a
• 25-50 , ?p?a
• ?a?a?t???st??? ??
• ??????µa
• ????µ??? s????t?ta ?a????e?a?
• ?a?a?t???st??? ?. e? e???e?st??
• ?p?a ?CPK (ltX5-6)
• ?e??fe???? p??sß???
• ?e???pa??t???? ß??ße?
• ??????µ???? ??d?e?
• ?st???a ?e?ape?a?

42

• ???a ????? ?a?a?t???st??? ??-??
• ????a????t?da
• F. Raynaud
• ???µes? p?e?µ???p??e?a
• ??? () ?80
• ??d??? a?t?a?t?s?µata (anti-Jo-1, anti-Mi-2)
• ???s?t?da ??s?µ?t?? s??det???? ?st??
• s?????? ??????? ?p?a

43
S?s??t?s? µe ?e?p?as?e?

• ?e?µat?µ??s?t?da saf?? a???µ??? ep?pt?s?
  ?e?p?as???
• ????µ??s?t?da p??a??
• ??d? ?e?p?as???
• O????e?, p?e?µ??a?, µast??, G?S, ?on-HL
• ????µ???? ???d????
• 3 p??ta ?t?
• ??????µ????
• ??e?e???s?
• ?ate?????µe?? a?????a t?? ?????a? p??d?a?es????
  pa?a???t??
• ??a??? et?s??? ta p??ta ? 3 ?t?

44
?e?µat?µ??s?t?da
45
?e?µat?µ??s?t?da
46
?e?µat?µ??s?t?daasßest?p???se??
47
F?e?µ???de?? µ??s?t?de?-p??s????s?

• ??e?t??µ?????f?µa (90 pa????????)
• ????µ??? d?ast????t?ta st?? e?s?d? t?? ße?????,
  a?t?µat? d?ast????t?ta
• ?aµ??? ?????, µ????? d????e?a? p???fas???
  d??aµ???
• ??G ßas??? st? ?? µ??pa??t???? ap?
  ?e???pa??t???? ?? (ALS, pe??fe????
  p????e???p??e?a, myasthenia gravis)
• ?a??µ??a e???µata se µ??p??e?e?
• ???s?t?de?
• ???µ?de??
• ???????
• ?etaß??????

48
F?e?µ???de?? µ??s?t?de?-p??s????s?

• ?????a µ???
• ?????s?, e?f???s?, a?a?????s?, f?e?µ???d??
  d????s?
• ???CD8 e?d?µ????
• ?? ? CD4 pe??µ????-pe??a??e?a?? d????s?
• ?????a e? e???e?st?? ? ????e?sta (?70)
• ?p? ?p???a? µetaß?????? µ??p??e?a? ? ?????
  µ??pa?e???
• ??e?t?????? µ????s??p??
• ????µ???? ??e????

49
?????a µ???
????µ??s?t?da
?e?µat?µ??s?t?da
50
????µ??s?t?da-de?µat?µ??s?t?da ??af???? st??
pa?????e?a
?e?µat?µ??s?t?da ???ß? epa??µe?? ap? t?
s?µp????µa (MAC) CD4 ?e p??sß??? t?? a??e???
st?? pe??f??e?a t?? µ????? deµat???
????µ??s?t?daCD8 T cell epa??µe?? ß??ß?