DERMATO/POLYMYOSITIS

DR. DANK kATALIN

Systemic manifestations of PM/DM:
ene!a"
#$ati%&e' ma"aise' (ei)* "oss' Rayna&+ fenomenon
M&sc&"oske"eta"
#M&sc"e (eakness' ,ain' ten+e!ness' at!o,*y
#A!t*!a")ias an+ a!t*!itis
-&taneo&s
#ott!on.s ,a,&"es' *e"iot!o, !as*' !as*es o/e! t*e ante!io! neck 01 si)n2' an+
+ist!i3&tion of a s*a(" 0s*a(" )i!"2
#Ro&)*enin)' c!ackin)' an+ sca"in) of t*e fin)e!s 0mec*anic.s *an+s2
#1asc&"itis (it* infa!cts an+ &"ce!s' s&3c&taneo&s ca"cifications
#Nai"fo"+ ca,i""a!y c*an)es
ast!ointestina"
#Dys,*a)ia' !ef"&4' an+ +ysmoti"y of t*e 3o(e"
#Nasa" !e)&!)itation
P&"mona!y
#1enti"ato!y ins&ficiency an+ ate"ectasis f!om m&cs"e (eakness' as,i!ation
#Pne&monia
#Inte!stitia" fi3!osis
-a!+iac
#Myoca!+itis (it* a!!*yt*mias an+ con)esti/e fai"&i!e
#-o! ,&"mona"e
-!ite!ia fo! t*e +ia)nosis o+ PM/DM
5. M&sc"e (eakness#&s&a""y ,!o4ima" an+ symmet!ica"
6. Positi/e m&sc"e 3io,sia
7. E"ect!omyo)!a,*ic t!ia+ of s*o!t' sma""' ,o"y,*asic moto! &nits' fi3!i""ation'
,ositi/e s*a!, (a/es an+ inse!tiona" i!!ita3i"ity' an+ 3i8a!!e' *i)* f!e%&ency
!e,etiti/e +ic*a!)es
9. Raise+ -K acti/ity
:. Any one of t*e c*acta!estic +e!mato"o)ic feat&!es of !as* of +e!matomyositis
A' *e"iot!o, !as* 0!e+#,&!,"e e+emato&s e!yt*ema on t*e &,,e! ,a",e3!a2
;' ott!on.s si)n 0!e+#,&!,"e ke!atotic' at!o,*ic e!yt*ema' o! mac&"es on t*e
e4tenso! s&!fe of fin)e! <oints' e!yt*ema on t*e e4tenso! s&!face of e4t!emity
<oints2
-"assification of inf"ammato!y myo,at*y
Clinical groups
!o&, 5: ,!ima!y i+io,atic ,o"ymyositis
!o&, 6: ,!ima!y i+io,at*ic +e!matomyositis
!o&, 7: +e!matomyositis 0o! ,o"ymyositis2 associate+ (it* neo,"asia
!o&, 9: c*i"+*oo+ +e!matomyositis 0o! ,o"ymyositis2
!o&, :: ,o"ymyositis o! +e!matomyositis associate+ (it* ot*e! connecti/e tiss&e +isease
!o&, =: inc"&sion 3o+y myositis
Serological groups
A&toanti3o+y anti)en
Anti#synt*etase 5 of : kno(n amaminoacy"#tRNA synt*etase
Anti#SRP ,!oteins of si)na" !eco)nition ,a!tica"
Anti#Mi#6 +i!ecte+ a)ainst a 66>kDa n&c"ea! ,!otein
NI? c"assification of IIM &ti"i8in) t*e MSA stat&s an+ imm&no)enetics
DR(:6 associate+:
5. aminoacy"#tRNA synt*etase
6. SRP
7.
DR(:7 associate+
5. Mi#6
Imm&ne a3no!ma"ities in PM/DM
Myositis specific autoantibodies (MSA)
@ni%&e feat&!e
Anti)en#+!i/en
A!ise mont* 3efo!e t*e oneset of myositis
-o!!e"ate in tite! (it* +isease acti/ity
Disa,,ea! afte! ,!o"on)e+ com,ete !emission
One MSA in one ,atient
Anti-synthetase autoantibodies
anti#Ao#5 0anti#*isti+y"#t#RNA synt*etase
anti#PL#B 0anti#t*!eony"#tRNA synt*etase2
anti#PL#56 0anti#a"any"# tRNA synt*etase2
anti#OA 0anti#iso"e&cy"# tRNA synt*etase2
anti#EA 0anti#)"ycy"#tRNA synt*etase2
Pat*o"o)ic c*an)es of m&sc"e in +e!matomyositis
Muscular histopathology
,e!i/asc&"a!' ,e!ifascic&"a! inf"ammation'
at*!o,*y'
/asc&"a! +ama)e' isc*aemia'
se)mentia" fi3e! nec!osis
M&sc&"a! cyto,at*o"o)y
*i)*e! ,e!centa)e of ; "ym,*ocytes an+ -D9C "ym,*ocytes
Pe!i,*e!a" cyto,*ato"o)y
+ec!ease+ -D7C an+ TLISAC monon&c"ea! ce""s'
inc!ease+ -D6>C an+ -D6>CDRCce""s
Anti#Mi#6 syn+!ome
Clinical manifestations
-"assic +e!matomyositis (it* 1 si)n' s*a(" si)n
Onset of myositis
Rate: ac&ta
Se/e!ity: mi"+
Season: &nkno(n
Response to therapy
oo+' !as* may ,e!sist
Prognosis : oo+' :#y s&!/i/a" !ate of nea!"y 5>>D
HLA associations: DRB' DRE:7' DFA5G>6>5
Frequency in myositis: :#5>D
Antisynt*etase syn+!ome
-"inica" manifestations
A!t*!itis' inte!stitia" "&n) +isease' fe/e!' mec*anic.s *an+s' Rayna&+.s
fenomenon
Oneset of myositis
Rate: ac&te
Se/e!ity: se/e!e
Season: s,!in)
Response to therapy: mo+e!ate' f"a!es (it* ta,e!in) t*e!a,y
Prognosis: ,oo!' :#y s&!/i/a" of B>D
HLA associations: DR7' DR(:6 DFA5G>:>5
Frequency in myostitis: 6>#6:D
Immune mechanisms in inflammatory myopathies
De!matomyositis
- antibody or immune-complex-mediated respones
against a vascular-endothelial component
- deposition of the complement membrane complex in or
around microvascular endothelium
- focal capillary depletion
- perifascicular atrophy
Po"ymyositis an+ I;M
- endomysial inflammatory exudate containing large
numbers of C!" # cells
- all if the invaded fibers express $%A class Iag
- $%A class-I-restricted cytotoxic # cell mediated
response against surface ag expressed by muscle fibers
&athologic changes of muscle in polymyositis
M&sc&"a! *isto,at*o"o)y
$ascic&"a!/en+omysia" inf"ammation' (it*o&t ,e!ifasc&"a! at!o,*y
M&sc&"a! cyto,at*o"o)y
?i)*e! ,e!centa)e of T "ym,*ocytes -DHC "ym,*ocytes an+ acti/ate+
T ce""s
Eit* minima" ; "ym,*ocyte in/o"/ement
Pe!i,*e!a" cyto,at*o"o)y
Inc!ease+ -D7CDRC monon&c"ea! ce""s' an+ -D7CDRC/DRC !atio
T!eatment of myositis
$ist#"ine t*e!a,y
'lucocorticoid( )-* mg+,g+d )-- month
Secon+#"ine t*e!a,y
A.othioroprine( )-* mg+,g+d
Methotrexate( /01-*1 mg+2ee,
Cyclophosphamide( *-- mg+,g+d
$ydroxycloro3uine( *44-544 mg+d
Cyclosporin A( --1 mg+,g+d
Intravenous immune globuline( )g+,g+d * days+month
Mec*anism of action if I1I
)6 7c receptor bloc,ade in the reticuloendothelial system
*6 increase in suppressore activity
-6 inhibition of autoreactive 8 cells that carry the autoreactive clones of anti-
idiotypic antibodies
56 modulation of cyto,ine production
16 neuralie complement0 this preventing the formation of membrane attac,
complex