*

*


233 (26-9-2011)

STILL (ADULT STILLS
DISEASE)





SJOGREN

/
( Takayasu)

-

- BEHCET

1




-
, (3-6) . ,

( 1-3 )
- (DMARDs)
.
- DMARD
*
.
DMARD
(, , , ,
)
-
.
.
-

* :
. RF / -CCP ( )
. /
. ( ,
)

1
-

7.5mg/

+
-

(7.5 20 mg , 25 mg)
(5mg/)
:
, , , , ,
/
* 3-6
.
.
3

2 : -TNF ()
-
- 1 +

--TNF (infliximab, adalimumab, etanercept, certolizumab, golimumab)
- batacept
- nakinra
- ocilizumab

3
2 ,

rituximab .

1:
. .. HbSAg,
rituximab TNF .
2:

3 .

.
3:
.

 STILL (ADULT
SET STILLS DISEASE)

1 *

per os (0.5-1 mg/kg) / (

)

: 7.5.-20 mg/

:
20mg/
2.5mg/kg/
Azathioprine 2-3mg/kg/

2 *


:
1. Anakinra, 100mg

2. Infliximab, 3-5mg/kg/

3. Tocilizumab, 8mg/kg IV 2

4. Etanercept 50mg

5. Adalimumab 40mg 2
*
.



.
1
-
: :
- 3 ,

- <3 ,
2
--TNF
: -TNF :

4 .

:
-

BASDAI 4 ( 0-10)

.
1
7,5mg/
-

: 7,5mg/ 2

: 4 3gr/
<4 ,
+

2
-

-TNF

.
1
-

2
--TNF
: -TNF 2

6


.
1
2 anti-TNF ()
B.
1 7.5mg-20mg/w ( ,
)
Cyclosporine () 2.5 -3.5 mg/kg/d

Leflunomide () 20mg/d

2000 mg/d
: p.os
2 anti-TNF ()
. :

. -
1
-

PUVA/UVB (psoralen plus ultraviolet A)

2.5-3.5 mg/kgld

7.5-15 mg/w

20 mg/d
2 anti-TNF ()
E.
1 :
-
-
2 : anti-TNF ()
: anti-TNF
, antiTNF
7


.
anti-TNF (infliximab & Adalimumab)

:
( )
.

.
1 :
2000-3000 mg /d
+
: budenoside prednisolone p.o
2 : anti-TNF


. +
. , , :
20-40 mg/d
:
.
Chlamydia trachomatis ,
.

 ()

.
. /
- (7.5-10mg)
- (200-400 mg/)
- (7.5-15mg/) + (5mg/)

.
1
- ( )
per os (200-400mg/)
2
- (7.5mg/)
- (2-3mg/)

3
- 50mg/
- 100mg/
.
1
- (20mg/)
2
- (2mg/KgB)
. ( /
)
.
( : ISN IIIa, IIIa/c, IVa, IVa/c, V*)

(ISN IIIa, IIIa/c, IVa, IVa/c)
10

-
6 IV (1gr),
(1mg/KgB) per os *
+
6 IV (750-1000mg/m2)
+
1. ( )
2. MESNA (2-Mercaptoethanesulfonate sodium)
( )
* , 1 mg/kg

1
-

(2-3mg/KgB)

2
-

(2-3kg/)

Rituximab

(V)
per os (5mg/KgB)

.
. * ( , ,
, )
. ( , Libman Sacks,
, , )
. ( , <50000/mm3,
WBC<1000/mm3 )
. ()
. ( , ,
, shrinking lung)
. ( , )
. (,
)
11

-
- (1mg/KgB)
(1gr/ 3 )
/
-iv (750-1000mg/m2)
6

Rituximab
*+, , ( )

.
-
- 80mg

-Rituximab

12

 SJOGREN

.

1
-
- 5mg ( 6-8 )
2
-per os N-acetylcysteine

1
-
- 5mg ( 6)
2
- 0.05% 12
/ x 6
.

-
-

1
- (200mg/)

- 7,5mg/
+
- (7.5mg/)
2
Rituximab IV 1000mg x 2 15 6 8 10

RAYNAUD
-

- (0.5mg/kg )
-rituximab IV 1000mg x 2 15 .
6


.
/
13

- (10-15gr/Kg/)

- (0,5 1,0 mg/kg / )
- (2mg/kg /)
.

- (0,5 -1,0 mg/kg /)

- (0,5-1,0 g/m2 / )
6
(2mg/kg . )

- (3-12g )
.

- -

-, (0,5-1,0mg/kg /)
- (2mg/kg /)
.
. (, )
- (1gr 3 )
- (0,5-1,0 mg/kg / )
- (0,5 1,0 g/m2 /)
- (2mg/kg / )
.

- (0,5-1,0 mg/kg )
-Rituximab (375mg/m2 , 4 )

- MALT

-
CHOP (cyclophosphamide, vincristine, doxorubicin, prednisolone) + rituximab

14



. Rynands

(... 120 mg )
.
: IIprost i.v. 0.5-2ng/kg/min
5-7
1: bosentan
.

.
2: sildenafil -


. ( )
1
( standard . , , , )
:
:
:
Bosentan tabs
: 62.5mg x 2 (1 ) 125x2

Sildenafil tabs 50mg x 3

Sitaxentan tabs
: 100mg/
:
Bosentan Sitaxentan Sildenafil
IV:
Epoprostenol IV
: 2ng/kg/min
.
7,5mg/
+
10-20mg/
. -
per os 20mg ( - )
+
* 6 12
Azathioprine 2.5mg/kg/
15

* :
i.v. (600mg/m2/),

per os: 1-2mg/kg/day

. -
(-),
Iloprost i.v. 0.5-2ng/kg/min
.
-
-
-Octreotide

16

/

1
per os (1mg/kg)
+
per os im (17,5-25 mg/)
Azathioprine per os (2-3mg/Kg/)
(3mg/Kg/)

+
- (IVIG) i.v. (0.4g/kg/ 5 ) 3-6
,
/ . IVIG

2
Cyclophosphamide
Rituximab (1gr x 2 15/6 )
: ,
,
.

(INCLUSION BODY MYOSITIS)

17


Takayasu)

1.
-per os (1mg/Kg, 60mg) (
)
2.
1
-: 20-25mg/
-:
2mg/kg/
2
- ( )
3
--TNF *

18

 ()

1. (i) (
):
40-60mg/

32-48mg/
(ii) :
IV 500mg-1gr , 3
(iii) :
60mg/
2.

3. ,
D
.
4. ( )

5.

.

19

 -
( Wegener, , Churg-Strauss,
, )

[ ,
<500mol/L (5.6mg/dl)/ ]
1.
1. (1mg/Kg, 60mg)
+
(per os 2mg/kg/day iv)

Per os 2mg/kg/day

i.v. 15mg/kg 2-3

2. ( )
3. MESNA (2-Mercaptoethanesulfonate sodium)
4. Pneumocystis jiroveci ( Pneumocystis carinii)
trimethoprim/sulphamethoxazole (800/160mg
400/80mg ),
2.

(2mg/kg/day), (20-25mg/)*
trimethoprim/sulphamethoxazole (800/160 mg
)
Wegener
, (Mupirocin)
S. Aureus

B. ANCA-
( )
(2025mg/ )

. ( >500 mol/L
5.65 mg/dl)
+ (. 1)
.
Rituximab (375 mg/m2/ 4 )
. ( )
(
)
20

. C.
(-
)
. .
( 2
),

21



per os 15-20mg/

:

22

 - BEHCET
.
+ .
(2mg/KgB) -TNF

.
.

(2mg/KgB) . /
infliximab (5 mg/Kg).

. *
, , , -TNF .

. *
(1mg/KgB)
(1gr/ 3 ), /
6 iv
2
(750-1000mg/m ), /
-TNF .

. - *
(,
, , / -TNF ).
.

* .

: -TNF .
infliximab

23



1.
1
( )

40mg
( )
2


2.
) (
<6mg/dL (0,36mmol/l)
( ),
.
Febuxostat

)
Anakinra
3.

0,5-1mg/ ( ). :

:

: (
)

(
)

24

 (-)

-1
( )
-2

-
( )
+ Tramadol 1-2g/kg
- ( )

- ( )

.
.
( )
100mg/, 2 .
per os,
.

()
1
, 4g/

2



.
.
( )

25



.
1.
1
Amitriptyline
Fluoxetine
Duloxetine
Milacipran
Moclobemide
2
Pregabalin
Tropisetron
Pramipexole
2. ..
3. ..
4. Tramadol

.
1.
Amitriptyline
Fluoxetine
Duloxetine
Milnacipran
Moclobemide

26


1)
(DXA, Tscore
-2,5) , ,

:
)
)

,
, , , , , Denosumab ,
(1-84 )
, , , ,
Denosumab,
, Denosumab

.
(evidence based)



, ,
.
1000-1200mg Ca+2 600-800IU
Vit +D

) , , 5 mg IV
) Strontiun Ranelate
) SERMs ( ):
:
Ca (
Ca )
) Denosumab
) :

27

) : -

.
.
) :

.

2)
(DXA, Tscore (-1) (2,5) , ,

()
()

( ) ..
, , ,
,
Ca Ca
.. Crohn,
( ,
, Parkinson, , ,, )
: (
, ) ,



( , ,

() 10
FRAX (http://www.shef.ac.uk/FRAX) =>3%
=>20% (, ,
, , )
: 2)
1)

3)
(DXA T-score
-2,5) ) ,
,
.
28


, , , ,
, , Denosumab , (1-84)

, , , , ,
, , (1-84)
Denosumab, ,
, , Denosumab

.
(evidence based)


, ,
.


1000-1200mg Ca+2 600-800IU Vit
+D

) , , IV 5mg/ ,

) Strodiun Ranelate
) Denosumab
) SERMs ( ):
(
/
Ca )
) (
)
) , (1-84)*
(**) ,
,
(*)
(**) 1
() (>LSC [
])

) :
1)
2)

29

.

) : -

.
.

30


> 50

O (T-score<-2.5),


FRAX (http://www.shef.ac.uk/FRAX) =>3%
=>20% (, ,
, , )
:
, ,

. >50
.
- 1000-1200mg Ca+2 600-800IU
Vit +D ( )
1. :
) <7,5mg/
) >7,5mg/,
2. :
) <7,5mg/
) >7,5mg/
3. :
) <5mg/ 1 , .
) >=5mg/ 1 >
1,

*
3

31

. <50

1. <50
. 1-3 :
i) 5mg/
ii) 7,5mg/
) >3 :

2.
. 1-3
. >3 :
i) <7,5mg/
ii) 7,5mg/

. <50

32