M u s c le p a th o lo g y in ju v e n ile d e rm a to m y o s itis
I n s t i t u t o d e I n f e c t o l o g i a E m l 1 i o R i b a s , S e ç ã o d e A n a t o m i a P a t o l ó g i c a - S ã o P a u l o , B r a s i lL a b o r a t ó r i o d e P a t o l o g i a M o l e c u l a r , D e p a r t a m e n t o d e P a t o l o g i a d a
F a c u l d a d e d e i t l e d i c i n a d a U n i v e r s i d a d e d e S ã o P a u l o ,
I n s t i t u t o A d o l f o L u t z , D i v i s ã o d e .P a t o l o g i a - S ã o P a u l o , B r a z i l
O b je c tiv e : T o s tu d y m u s c le b io p s ie s , u s in g h is to c h e m is try , o n te n c h ild re n w ith in fa n tile d e rm a to m y o s itis . D e s ig n : S e rie s o f te n p a tie n ts (o f w h o m e ig h t p a tie n ts h a d re c e iv e d tre a tm e n t a n d tw o h a d n o t) w e re s u b m itte d to m u s c le b io p s y in o rd e r to d ia g n o s e p o s s ib le in fla m m a to ry m y o p a th y o r to d e te c t re c ~ rre n c e s . P la c e o f d e v e lo p m e n t o f th e s tu d y : P u b lic H e a lth S e rv ic e o f S ã o P a u lo S ta te . P a rtic ip a n ts : c h ild re n w ith c lin ic a i fe a tu re s o f in fla m m a to ry m y o p a th y . In te rv e n tio n : b io p s ie s w e re p e rfo rm e d o n th e v a s tu s la te ra lis u s in g lo c a l a n e s th e tic . H is to c h e m is try w a s p e rfo rm e d a c c o rd in g to s ta n d a rd iz e d m e th o d s . R e s u lts : A rc h ite c tu ra l c h a n g e s o f th e m u s c le fib e rs , n e c ro s is o f v a ria b le in te n s ity a n d a c c e n tu a te d e v id e n c e o f re g e n e ra tio n w e re o b s e rv e d in p a tie n ts w h o h a d n o t re c e iv e d tre a tm e n t (2 c a s e s ) a n d in o n e c a s e w h e re m u s c u la r w e a k n e s s p e rs is te d in s p ite o f c o rtic o s te ro id th e ra p y . N e c ro s is a n d re g e n e ra tio n w e re m in im a l o r a b s e n t in c a s e s tre a te d fo r o n e y e a r o r m o re (4 c a s e s ). In 3 c a s e s w ith c lin ic a i a n d la b o ra to ria l re c u rre n c e s , m u s c le n e c ro s is a n d a rc h ite c tu ra l c h a n g e s w e re d e te c te d . C o n c lu s io n s : It w a s c o n c lu d e d th a t m u s c le b io p s y c o u ld a id in d ia g n o s in g in fa n tile d e rm a to m y o s itis a s w e ll a s in d e te c tin g re c u rre n c e s e v e n in c a s e s w ith o u t c lin ic a i a c tiv ity o f th e d is e a s e .
U N IT E R M S : J u v e n ile d e rm a to m y o s itis . P o ly m y o s itis . M u s c le d is e a s e s . H is to c h e m is try .
IN T R O D U C T IO N
J
u v e n ileg r e a te s t d e r m a to m y o s itisin c id e n c e b e tw e e nis á r a r ef iv e a n dd is e a s ef o u r te e nw ithy e a r sth e o f a g e I.I t is a s y s te m ic d is o r d e r o f u n k n o w n e tio lo g y ,c h a r a c te r iz é d b y n e c r o s is a n d r e g e n e r a tio n o f m u s c le f ib e r s , w ith in f la m m a tio n o f m u s c le , s k in a n d o th e r tis s u e s .
S y s te m ic m a n if e s ta tio n s s u c h a s f e v e r , le th a r g y a n d g e n e r a l d is c o r n f o r t m a y b e th e f ir s t s y m p to m s o f th e illn e s s ,
A n d re s s fo r c o rre s p o n d e n c e : E d e n ils o n E d u a rd o C a lo re
In s titu to d e In fe c to lo g ia E m ílio R ib a s - S e ç ã o d e A n a to m ia P a to ló g ic a
A v . D r. A rn a ld o , 1 6 5 -P a c a e m b u S ã o P a u lo /S P - B ra s il- C E P 0 1 2 4 6 -9 0 0
w ith m in im a l m u s c le w e a k n e s s . C u ta n e o u s le s io n s a r e f r e q u e n t a n d th e y a p p e a r a t th e o n s e t o r d u r in g th e c o u r s e o f th e d is e a s e . I t c a n b e d if f ic u lt to r e c o g n iz e th e s e le s io n s in b la c k p a tie n ts2
•
I t d if f e r s f r o m a d u lt p o ly m y o s itis a n d d e r m a to m y o s itis in s o m e a s p e c ts . F ir s tly , v a s c u litis o f s m a ll v e s s e ls is f r e q u e n t, le a d in g to u lc e r a tio n s o f th e s k in . S o m e p a tie n ts c a n p r e s e n t c a lc in o s is3 a r id th e g a s tr o in te s tin a l tr a c t, k id n e y a n d m y o c a r d iu m c a n b e in v o lv e d4, 5 , 6 . M o r e o v e r , ju v e n ile d e r m a to m y o s itis is a lw a y s id io p a th ic , w h e r e a s in f la m m a to r y m u s c le d is o r d e r s in a d u lts c a n b e p a r t o f a p a r a n e o p la s tic s y n d r o m e7
•
D ia g n o s is is b a s e d o n c lin ic a i c r ite r ia , e le v a tio n o f s e r u m m u s c le e n z y m e s a n d c o m p a tib le e le c tr o m y o g r a p h y . T h e h is to lo g ic a l m u s c le f e a tu r e s o f th e d is e a s e ( in f la m m a tio n , n e c r o s is a n d r e g e n e r a tio n o f m u s c le f ib e r s a n d p e r if a s c ic u la r a tr o p h y ) a r e c h a r a c te r is tic8
• I ts e v o lu tio n is q u ite v a r ia b le a n d d e p e n d s p a r tia lly o n th e ty p e o f tr e a tm e n t.
C A L O R E , E .E .; C A V A L lE R E , M .J .; P E R E Z , N . M . - M u s c le p a th o lo g y in ju v e n ile
d e rm a to m y o s itis
T h is d is o rd e r m a y b e c la s s ifie d a c c o rd in g to its
e v o lu tio n a s th e a c u te m o n o c y c lic fo rm , a c u te p o ly c y c lic
fo rm o r c o n tin u o u s fo rm9
, 1 0 ,re s p e c tiv e ly if th e re m is s io n is
c o m p le te , p a rtia l w ith re c u rre n c e , o r if th e d is e a s e is
p e rs is te n t in s p ite o f c o rtic o s ~ e ro id th e ra p y . T h is
c la s s ific a tio n d o e s n o t in c lu d e th e re s p o n s e to
im m u n o s u p p re s s iv e s s u c h a s c ic lo p h o s p h a m id e , u s e fu l in
s o m e c a s e s o f d e rm a to m y o s itis .
In th e p re s e n t w o rk , w e d e s c rib e th e h is to lo g ic a l a n d
h is to c h e m ic a l fin d in g s o f s k e le ta l m u s c le b io p s y in 1 0
p a tie n ts w ith ju v e n ile d e rm a to m y o s itis . W e try to c o rre la te
th e s e fin d in g s w ith th e c lin ic a I re s p o n s e to th e tre a tm e n t
a n d th e c lin ic a I re c u rre n c e .
M A T E R I A L A N D M E T H O D S
T e n c a s e s o f ju v e n ile d e rm a to m y o s itis (2 b o y s a n d 8
g irls , a g e d fro m 3 V 2to 1 4 y e a rs ) w e re s u b m itte d to m u s c le
b io p s y . D e rm a to m y o s itis w a s d ia g n o s e d b a s e d o n c lin ic a I
c rite ria : c u ta n e o u s e rith e m a o r h e lio tro p e a s s o c ia te d to
p ro x im a l m u s c le w e a k n e s s o f v a ria b le in te n s ity . In o n e o f
th e c a s e s (c a s e n u m b e r 1 0 ) c u ta n e o u s le s io n s w e re n o t
o b s e rv e d . H o w e v e r, in fla m m a to ry m y o p a th y a s s o c ia te d
w ith p e rifa s c ic u la r a tro p h y , w h ic h is c h a ra c te ris tic in
d e rm a to m y o s itis , w a s o b s e rv e d b y m u s c le b io p s y .
T h e b io p s y w a s p e rfo rm e d o n th e v a s tu s la te ra lis . T h e
s a m p le s w e re fro z e n in n -h e x a n e p re v io u s ly c o o le d in liq u id
n itro g e n a n d th e s e c tio n s w e re c u t in a c ry o s ta t. H is to lo g ic a l
a n d h is to c h e m ic a l te c h n iq u e s w e re p e rfo rm e d a c c o rd in g to
D u b o w itz ,1 9 8 511 •
P a tie n ts w e re s u b d iv id e d in to 3 g ro u p s (T a b le 1 ):
G ro u p I:c a s e s th a t h a d n o t re c e iv e d tre a tm e n t (c a s e s
1 a n d 2 ) o r w ith p e rs is te n c e o f th e m u s c u la r w e a k n e s s in
s p ite o f c o rtic o s te ro id th e ra p y (c a s e 3 , tre a te d fo r 1 0
m o n th s );
G ro u p 1 1 : c a s e s th a t w e re tre a te d fo r m o re th a n o n e
y e a r a n d w ith g o o d c lin ic a I re s p o n s e (c a s e s 4 , 5 , 6 , 7 );
G ro u p 1 1 1 :c a s e s th a t w e re tre a te d a n d p re s e n te d g o o d
c lin ic a I re s p o n s e , b u t w ith c lin ic a I o r la b o ra to ria l
re c u rre n c e (in c re a s e in m u s c le s e ru m e n z y m e s ) a t th e tim e
o f th e b io p s y (c a s e s 8 , 9 a n d 1 0 ).
T h e re s u lts o f th e h is to c h e m ic a l s tu d y w e re g ra d e d
(fo r e a c h h is to c h e m ic a l re a c tio n ) a t th re e le v e Is : (+ ) ra re
o c c u rre n c e o f p o s itiv e fib e rs ; (+ + ) m o d e ra te n u m b e r o f
p o s itiv e fib e rs ; (+ + + ) g re a t n u m b e r o f p o s itiv e fib e rs .
R E S U L T S
G ro u p I: T h e m o n o n u c le a r c e llu la r re s p o n s e w a s
m o d e ra te o r in te n s e . It in v a d e d s o m e m u s c le c e lls (F ig 1 ).
T h e a c id p h o s p h a ta s e re a c tio n s h o w e d v a ria b le g ra d e s o f
n e c ro s is , a n d th e a lk a lin e p h o s p h a ta s e re a c tio n d is p la y e d
m o d e ra te to in te n s e fe a tu re s o f re g e n e ra tio n . T h e o x id a tiv e
e n z y m e s , p a rtic u la rly th e n ic o tin a m id e a d e n in e
-d in u c le o ti-d e te tra z o liu m re d u c ta s e (N A D H - T R ) re a c tio n ,
s h o w e d m a rk e d a rc h ite c tu ra l c h a n g e s in m u s c le fib e rs , s u c h
a s w h o rle d , m o th -e a te n a n d ta rg e t fib e rs (F ig . 2 ). F o c a l
v a s c u litis w a s o b s e rv e d in c a s e 3 .
G ro u p 1 1 :m ild n e c ro s is a n d re g e n e ra tio n w e re s e e n
w ith th e a c id a n d a lk a lin e p h o s p h a ta s e re a c tio n s . T h e
in te rm y o fib rilla r n e tw o rk o f m u s c le fib e rs p re s e n te d d is c re te .
c h a n g e s w ith th e N A D H - T R re a c tio n .
F ig u r e 1 - M o n o n u c le a r in v a s io n o f n e c r o t ic m u s c le c e lls .
H e m a t o x y lin - E o s in s t a in ( X 4 0 0 ) .
F ig u r e 2 - A r c h it e c t u r a l c h a n g e s in m u s c ie f ib e r s o b s e r v e d b y t h e
N A D H - T R r e a c t io n ( X 4 0 0 ) .
C A L O R E , E . E . ; C A V A L lE R E , M . J . ; P E R E Z , N . M . - M u s c le p a t h o lo g y in ju v e n ile d e r m a t o m y o s it is
la b ia 1
C lin ic a i a n d h is to p a th o lo g ic a l d a ta .
G ro u p C a s e A g e / T im e o f d is e a s e / N u m b e r S e x T im e o f tre a tm e n t
E v o lu tio n N e c ro s is V a s c u litis In fa rc t R e g e n e ra tio n C e n tra l A rc h ite c tu ra l (a c id p h o s p h ) (a lk a lin e p h o s p h ) n u c le i C h a n g e s
% fib re s
A tro p h y
1 1
1 1 1
8
1 0 1 4 Y
F
2 Y d is e a s e
2 y tre a te d
G o o d re s p o n s e
H e lio tro p e
In te n s e w e a k n e s s
R e s p ira to ry fa ilu re
+ +
+++
+++
+++
+++
+
+
+++
3 0 %
1 0 %
2 0 %
+
+
+ +
n o t s e le c tiv e
p e rifa s c ic u la r
p re d o m in a n tly
p e rifa s c ic u la r
G r o u p 1 1 1 : M o d e r a te to in te n s e d e g r e e s o f n e c r o s is
w e r e s e e n , a s w e ll a s a m o d e r a te g r a d e o f r e g e n e r a tio n . O n e
c a s e s h o w e d la r g e r e g io n s o f m u s c u la r in f a r c t. C h a n g e s in
th e in te r m y o f ib r illa r n e tw o r k w e r e o f m o d e r a te in te n s ity .
V a s c u litis w a s o b s e r v e d in th e th r e e c a s e s ( F ig . 3 ) .
E x c lu s iv e ly p e r if a s c ic u la r a tr o p h y w a s o b s e r v e d in a
m in o r ity o f c a s e s ( F ig . 4 ) . H o w e v e r w id e s p r e a d m u s c le f ib e r
a tr o p h y ( in c lu d in g p e r if a s c ic u la r a tr o p h y ) w a s s e e n in 8
o u t o f th e 1 0 c a s e s .
T h e r e s u lts a r e s u m m a r iz e d in T a b le I .
D IS C U S S IO N
T h e h is to p a th o lo g ic a l f e a tu r e s o f s k e le ta l m u s c le in
ju v e n ile d e r m a to m y o s itis a r e e s s e n tiã lly th e s a m e a s th o s e
s e e n in a d u lt d e r m a to m y o s itis . T h e s e f e a tu r e s in c lu d e :
in f la m m a tio n w ith m u s c le f ib e r n e c r o s is , v a s c u litis ,
m u s c u la r in f a r c ts a n d p e r if a s c ic u la r a tr o p h yl2 .
T h e d e g r e e o f in f la m m a tio n is v a r ia b le . I n s o m e c a s e s
it is s c a r c e , b e in g m o r e in te n s e in th e p e r im y s iu m a n d le s s
in te n s e in th e e n d o m y s iu m . T h e in f la m m a to r y p r o c e s s
c o m p r is e s h ig h p e r c e n ta g e s o f B ly m p h o c y te s a n d T
s u p p r e s s o r Iy m p h o c y te s 1 3 .T h e in te n s ity o f th e m u s c le f ib e r
n e c r o s is is v a r ia b le a n d th e n e c r o tic f ib e r s a r e f r e q u e n tly
f ille d w ith m a c r o p h a g e s . A r o u n d th e v e s s e ls th e
in f la m m a to r y p r o c e s s m a y b e e x u b e r a n t a n d tr u e v a s c u litis
m a y b e s e e n , p o s s ib ly w ith f ib r in o id n e c r o s is o f th e s m a ll
v e s s e ls .
S tu d ie s o f th e m ic r o v a s c u la tu r e o f s k e le ta l m u s c le o f
p a tie n ts w ith d e r m a to m y o s itis s h o w e d p r o m in e n t c a p illa r y
d a m a g e a n d c a p illa r y d e p le tio n th a t w e r e n o t s e e n in o th e r
in f la m m a to r y m y o p a th ie s , s u c h a s p o ly m y o s itis l4
• A r e a s o f
in f a r c ts d u e to v a s c u litis m a y a ls o p o s s ib ly b e s e e n .
C A L O R E , E .E .; C A V A L lE R E , M .J .; P E R E Z , N . M . - M u s c le p a th o lo g y in ju v e n ile d e rm a to m y o s itis
F ig u re 3 - V a s c u litis o f a s m a ll v e s s e l. lh e w a ll is in filtra te d b y m o n o n u c le a r c e lls . H e m a to x y lin -E o s in s ta in (X 4 0 0 ).
P e rifa s c ic u la r a tro p h y is a d is tin c t, c h a ra c te ris tic
p h e n o m e n o n o f d e rm a to m y o s itis , e v e n if ra s h is a b s e n t,
a lth o u g h it c a n o c c u r in o th e r c o n d itio n s s u c h a s
s c le ro d e rm al2
•
In th e p re s e n t s e rie s o f p a tie n ts , th e in te n s ity o f
n e c ro s is h a s a re la tio n s h ip to th e a c tiv ity o f th e d is e a s e :
c a s e s th a t h a d n o t re c e iv e d tre a tm e n t a n d c a s e s w ith
re c u rre n c e h a d m o re n e c ro s is th a n c a s e s w ith a g o o d
c lin ic a I re s p o n s e . V a s c u litis w a s o b s e rv e d in c a s e s w ith
c lin ic a I o r la b o ra to ria l re c u rre n c e a n d in th e c a s e w h e re
m u s c u la r w e a k n e s s p e rs is te d in s p ite o f c o rtic o s te ro id
th e ra p y . T h e c lin ic a I s ig n s o f th e p a tie n ts w ith re c u rre n c e
w e re v a ria b le : o n e p a tie n t p re s e n te d o n ly h e lio tro p e ;
a n o th e r s h o w e d a n in c re a s e in s e ru m e n z y m e s (w ith o u t
a n y e v id e n c e o f m u s c u la r w e a k n e s s ) a n d a th ird p a tie n t
p re s e n te d in te n s e m u s c u la r w e a k n e s s a n d re s p ira to ry
fa ilu re .
F ig u re 4 - P e rifa s c ic u la r a tro p h y . H e m a to x y lin -E o s in s ta in (X 4 0 0 ).
A tro p h y o f m u s c le c e lls w a s a fre q u e n t fin d in g (8 /
1 0 ), b u t w a s e x c lu s iv e ly p e rifa s c ic u la r in o n ly tw o c a s e s ,
w ith o u t a c lin ic a I re la tio n s h ip to th e e v o lu tio n o f th e d is e a s e .
C h a n g e s in m u s c le fib e r a rc h ite c tu re b y o x id a tiv e
e n z y m e s , w e re p ro m in e n t in th e tw o c a s e s th a t h a d n o t
re c e iv e d tre a tm e n t, in th e c a s e w ith o u t a g o o d c lin ic a I
re s p o n s e to tre a tm e n t a n d in re c u rre n t c a s e s . T h e s e c h a n g e s
c o u ld b e d u e to is c h e m ia (c a u s e d b y th e v a s c u la r
in v o lv e m e n t) o r d u e to th e p rim a ry m u s c le in fla m m a tio n .
In s u m m a ry , w e p re s e n t th e m u s c le p a th o lo g y , u s in g
h is to c h e m is try , o f 1 0 p a tie n ts w ith in fa n tile
d e rm a to m y o s itis . A lth o u g h th e n u m b e r o f c a s e s is s m a ll
(b e c a u s e it is a ra re d is e a s e ) th e p re s e n t d a ta s u g g e s t th a t
b e s id e s b e in g u s e fu l fo r d ia g n o s in g in fa n tile
d e rm a to m y o s itis , m u s c le b io p s y m a y in a d d itio n d e te c t
re c u rre n c e s e v e n in s o m e p a tie n ts w ith o u t o b v io u s s ig n s o f
a c tiv ity o f th e d is e a s e .
O b je tiv o : e s tu d a r b ió p s ia s m u s c u la re s a tra v é s d e h is to q u ím iç a e m d e z c ria n ç a s c o m .d e rm a to m io s ite in fa n til. D e s e n h o : S é rie d e d e z p a c ie n te s (o ito d o s q u a is re c e b e ra m tra ta m e n to e d o is q u e n ã o o re c e b e ra m ) fo ra m s u b m e tid o s à b ió p s ia p a ra d ia g n ó s tic o d e p o s s ív e l m io p a tia in fla m a tó ria , o u p a ra a d e te c ç ã o d e re c o rrê n c ia s d a s m e s m a s . L o c a l: R e d e P ú b lic a d e S a ú d e d o E s ta d o d e S . P a u lo . P a rtic ip a n te s : c ria n ç a s q u e s u p o s ta m e n te a p re s e n ta v a m m io p a tia in fla m a tó ria . In te rv e n ç ã o : a s b ió p s ia s m u s c u la re s fo ra m re a liz a d a s n o m ú s c u lo v a s to la te ra l u tiliz a n d o a n e s te s ia lo c a l. F o i re a liz a d a h is to q u ím ic a d e a c o rd o c o m m é to d o s p a d ro n iz a d o s . R e s u lta d o s : F o ra m o b s e rv a d a s a lte ra ç õ e s d a a rq u ite tu ra in te rn a d a s fib ra s m u s c u la re s , g ra u s v a ria d o s d e n e c ro s e e s in a is d e re g e n e ra ç ã o n a s b ió p s ia s d e d o is p a c ie n te s q u e n ã o re c e b e ra m tra ta m e n to e e m u m c a s o d e p e rs is tê n c ia d a fra q u e z a m u s c u la r a p e s a r d e c o rtic o te ra p ia . N o s c a s o s c o m b o a re s p o s ta a o tra ta m e n to , fib ra s e m n e c ro s e o u re g e n e ra ç ã o fo ra m o b s e rv a d a s ra ra m e n te . E m trê s c a s o s c o m re c Q rrê n c ia c lín ic a o u la b o ra to ria l d a d o e n ç a fo ra m o b s e rv a d a s d e fo rm a a b u n d a n te fib ra s m u s c u la re s n e c ró tic a s e o u tra s fib ra s n ã o n e c ró tic a s c o m a lte ra ç õ e s d a a rq u ite tu ra in te rn a . C o n c lu s õ e s : C o n c lu i-s e a p a rtir d o o b s e rv a d o n o p re s e n te tra b a lh o q u e a b ió p s ia m u s c u la r a lé m d e a u x ilia r n o d ia g n ó s tic o d e d e rm a to m io s ite in fa n til, p o d e ta m b é m a u x ilia r n a d e te c ç ã o d e re c o rrê n c ia s , m e s m o n a a u s ê n c ia d e s in a is c lín ic o s fra n c o s d a d o e n ç a .
C A L O R E , E .E .; C A V A L lE R E , M .J .; P E R E Z , N . M . - M u s c le p a th o lo g y in ju v e n ile d e rm a to m y o s itis
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