FOR RECO

GN

ITION

OF

ACUTE RH

EUM

ATIC FEVER (ARF)

RED FLAG

TOOL

RHD

Australia | T: 0

8 8

922 8

196 | E: in

fo@rh

dau

stralia.org.au | W

: ww

w.rh

dau

stralia.org.au

• 5-

14 y

ears

old

- G

AS p

har

yngi

tis

is c

omm

on in

th

is a

ge g

rou

p

(How

ever

, AR

F h

as a

lso

bee

n s

een

in

pat

ien

ts in

to t

hei

r m

id

fort

ies

and

sh

ould

not

be

rule

d o

ut)

• Ab

orig

inal

peo

ple

an

d T

orre

s St

rait

Isl

and

ers

livi

ng

in r

ura

l or

rem

ote

sett

ings

are

kn

own

to

be

at h

igh

ris

k

• Oth

er A

bor

igin

al p

eop

le a

nd

Tor

res

Stra

it I

slan

der

s livi

ng

in

urb

an s

etti

ngs

, M

aori

s an

d P

acifi

c Is

lan

der

s, a

nd

pot

enti

ally

im

mig

ran

ts f

rom

dev

elop

ing

cou

ntr

ies,

may

als

o b

e at

hig

h r

isk

• ARF is a n

otifiab

le cond

ition in

WA, N

T and

QLD

. RHD

is not

curren

tly notifi

able an

ywh

ere in Au

stralia.

• W

hilst in

hosp

ital, the p

atient sh

ould

be registered

in

centralised

and

local ARF/RHD

registers. Please contact th

e Rh

eum

atic Heart D

isease Control Program

in you

r state/territory.

For compreh

ensive in

formation

on ARF an

d RH

D p

lease refer to:

• RH

DAu

stralia (ARF/RHD

writin

g group

), Nation

al Heart

Foun

dation

of Australia an

d th

e Cardiac Society of Au

stralia and

N

ew Zealan

d. Au

stralian gu

idelin

e for preven

tion, d

iagnosis

and

man

agemen

t of acute rh

eum

atic fever and

rheu

matic h

eart d

isease (2nd

edition

). 2012

• RH

DAu

stralia (ARF/RHD

writin

g group

), Nation

al Heart

Foun

dation

of Australia an

d th

e Cardiac Society of Au

stralia and

N

ew Zealan

d. Au

stralian gu

idelin

e for preven

tion, d

iagnosis

and

man

agemen

t of acute rh

eum

atic fever and

rheu

matic h

eart d

isease (2nd

edition

) Qu

ick reference gu

ides. 20

12

Available from

: RHDAu

stralia

Red flag 1:

• An

illness cau

sed b

y an au

toimm

un

e respon

se to a b

acterial infection

with

group

A streptococcu

s (G

AS infection

)

• ARF cau

ses an acu

te infl

amm

atory respon

se that

affects the h

eart, joints, b

rain an

d skin

. This

infl

amm

atory respon

se leaves no lastin

g dam

age to th

e joints, b

rain an

d skin

, how

ever the

dam

age to the h

eart (specifi

cally the m

itral and

aortic valves) m

ay remain

once th

e episod

e of ARF h

as resolved.

This is kn

own

as rheu

matic h

eart disease (RH

D)

• Is

gu

ided

by

the

2012

Au

stra

lian

cri

teri

a fo

r th

e d

iagn

osis

of

acu

te r

heu

mat

ic f

ever

• Cl

inic

al f

eatu

res

are

div

ided

in

to M

AJO

R an

d M

INOR

man

ifes

tati

ons

• M

AJO

R m

anif

esta

tion

s ar

e cl

inic

al f

eatu

res

that

m

ake

the

dia

gnos

is m

ore

like

ly

• M

INOR

man

ifes

tati

ons

are

con

sid

ered

to

be

sugg

esti

ve,

bu

t n

ot s

uffi

cien

t on

th

eir

own

for

a

defi

nit

e d

iagn

osis

of

acu

te r

heu

mat

ic f

ever

.

Acknow

ledg

men

ts: Orig

inal con

cept b

y Lisa Pa

nton

Revised

/ad

apted

by R

HDAu

stralia

, April 20

12

Red f

lag

2:Re

d f

lag 3

: Red

flag 7:

Rh

eum

atic H

eart D

isease Au

stralia

is an

initia

tive of Men

zies School of H

ealth

Research

, Jam

es Cook U

niversity a

nd

Ba

ker IDI.

Fun

ded

by th

e Austra

lian

Govern

men

t Dep

artm

ent of H

ealth

an

d Ag

eing

Revised/a

dop

ted b

y RHD

Austra

lia 20

13

MAJ

OR

man

ifes

tati

ons

for

hig

h r

isk

grou

ps

MIN

OR

man

ifes

tati

ons

for

hig

h r

isk

grou

ps

Card

itis

(in

cludin

g su

bcl

inic

al

evid

ence

of

rheu

mat

ic v

alvu

liti

s on

ec

hoc

ardio

gram

)

Mon

oart

hra

lgia

Poin

ts f

or d

iagn

osis

:

• M

ay b

e as

ymp

tom

atic

• M

ay p

rese

nt

as a

mu

rmu

r, c

hes

t pai

n

(rar

e)

• M

ost

com

mon

for

m:

valv

uliti

s (a

orti

c an

d/o

r m

itra

l va

lve)

• Se

vere

car

dit

is m

ay p

rese

nt

as h

eart

fa

ilu

re

Poin

ts f

or d

iagn

osis

:

• Pa

in in

joi

nt

mov

emen

t

• N

o ev

iden

ce o

f h

eat

or

swel

lin

g in

th

e jo

int

Poly

arth

riti

s or

ase

pti

c m

onoa

rth

riti

s or

pol

yart

hra

lgia

Feve

r

Poin

ts f

or d

iagn

osis

:

• M

ost

com

mon

pre

sen

tin

g AR

F sy

mp

tom

• Ca

n o

ccu

r in

on

e jo

int

or m

ult

iple

jo

ints

• As

ymm

etri

cal,

mig

rato

ry

• Af

fect

s la

rge

join

ts:

knee

s, a

nkl

es

Less

com

mon

: sh

ould

ers,

hip

s

• M

ost

com

mon

in

ch

ild

ren

/ad

oles

cen

ts

(can

pre

sen

t in

ad

ult

s)

• Re

spon

ds

to N

SAID

s w

ith

in 3

day

s

*ARF

shou

ld a

lways

be

con

sid

ered

a

s a

dif

fere

nti

al d

iag

nos

is f

or

pati

ents

pre

sen

tin

g w

ith

art

hri

tis

in

hig

h r

isk

pop

ula

tion

s

Poin

ts f

or d

iagn

osis

:

• H

isto

ry o

r pre

sen

tati

on w

ith

fe

ver ≥

38°

Cels

ius

Chor

eaRa

ised

ESR

/CRP

Poin

ts f

or d

iagn

osis

:

• Je

rky,

un

coor

din

ated

mov

emen

ts:

han

ds,

fee

t, t

ongu

e, f

ace

• Oth

er s

ign

s: m

ilkm

aid

s gr

ip,

spoo

nin

g, p

ron

ator

sig

n

• M

ovem

ents

dis

app

ear

du

rin

g sl

eep

• M

ay a

ffec

t on

ly o

ne

sid

e (h

emic

hor

ea)

• Pr

edom

inan

tly

affe

cts

adol

esce

nt

fem

ales

• Co

mm

on p

rese

nta

tion

in

Ab

orig

inal

an

d T

orre

s St

rait

Isl

and

er p

eop

le t

hat

pre

sen

t w

ith

ARF

Poin

ts f

or d

iagn

osis

:

• ES

R ≥

30 m

m/h

or

• CR

P ≥

30 m

g/L

Eryt

hem

a M

argi

nat

um

ECG

ch

anges

Poin

ts f

or d

iagn

osis

:

• Ex

trem

ely

rare

• Pr

esen

tati

on i

s h

igh

ly s

pec

ific

for

ARF

• Ra

sh:

bri

ght

pin

k m

acu

les

or p

apu

les

• Oft

en m

ista

ken

for

rin

gwor

m

• Occ

urs

mai

nly

on

tru

nk,

ext

rem

itie

s (n

ever

on

fac

e)

• Ra

sh m

ay p

ersi

st/r

ecu

r fo

r w

eeks

/m

onth

s af

ter

an e

pis

ode

of A

RF

Poin

ts f

or d

iagn

osis

:

• Pr

olon

ged

PR

inte

rval

an

d

oth

er r

hyt

hm

abn

orm

alit

ies

Subcu

tan

eous

nod

ule

s

Poin

ts f

or d

iagn

osis

:

• Fi

rm,

rou

nd

, pai

nle

ss n

odu

les

un

der

th

e sk

in w

hic

h m

ove

free

ly

• ½

–2

cm in

siz

e

• Ap

pea

rs o

n b

ony

join

ts:

elb

ow,

wri

st,

knee

s, a

nkl

es

• Ex

trem

ely

rare

• St

ron

gly

asso

ciat

ed w

ith

car

dit

is

Red f

lag

4:

Red f

lag 5

:

Inve

stig

atio

ns

in s

usp

ecte

d A

RF:

• Al

l pat

ien

ts w

ith

su

spec

ted

or

con

firm

ed A

RF s

hou

ld u

nd

ergo

ec

hoc

ard

iogr

aph

y to

con

firm

or

refu

te t

he

dia

gnos

is o

f rh

eum

atic

ca

rdit

is

• Th

e fo

llow

ing

inve

stig

atio

ns

are

reco

mm

ended

for

all c

ases

• W

hit

e b

lood

cel

l co

un

t

• Er

yth

rocy

te s

edim

enta

tion

rat

e (E

SR)

• C

Reac

tive

Pro

tein

(CR

P)

• B

lood

cu

ltu

res,

if

feb

rile

• El

ectr

ocar

dio

gram

( i

f p

rolo

nge

d P

-R

inte

rval

or

oth

er r

hyt

hm

ab

nor

mal

ity,

rep

eat

in 2

wee

ks a

nd

aga

in a

t 2

mon

ths,

if

still

abn

orm

al)

• Ch

est

X-ra

y, i

f cl

inic

al o

r ec

hoc

ard

iogr

aph

ic e

vid

ence

of

card

itis

• Ec

hoc

ard

iogr

am (

con

sid

er r

epea

tin

g af

ter

1 m

onth

, if

neg

ativ

e)

• Th

roat

sw

ab (

pre

fera

bly

bef

ore

givi

ng

anti

bio

tics

); c

ult

ure

for

gro

up

A

stre

pto

cocc

us

• An

tist

rep

toco

ccal

ser

olog

y: b

oth

ASO

an

d a

nti

-D

Nas

e B

tit

res,

(if

av

aila

ble

) re

pea

t 10

-14

day

s la

ter

if fi

rst

test

not

con

firm

ator

y

• Te

sts

for

alte

rnat

ive

dia

gnos

es,

dep

endin

g on

clin

ical

fea

ture

s

• Re

pea

ted

blo

od c

ult

ure

s, i

f p

ossi

ble

en

doc

ard

itis

• Jo

int

asp

irat

e (m

icro

scop

y an

d c

ult

ure

) fo

r p

ossi

ble

sep

tic

arth

riti

s

• Co

pp

er,

ceru

lop

lasm

in,

anti

nu

clea

r an

tib

ody,

dru

g sc

reen

for

ch

orei

form

mov

emen

ts

• Se

rolo

gy a

nd

au

toim

mu

ne

mar

kers

for

arb

ovir

al,

auto

imm

un

e or

re

acti

ve a

rth

riti

s

Red f

lag 6

:

Def

init

e in

itia

l ep

isod

e of

AR

F

2 M

AJOR

or 1 M

AJOR

and

2 M

INOR

+ evid

ence

of

a p

rece

din

g G

AS in

fect

ion

Def

init

e re

curr

ent

epis

ode

of

ARF

in a

pat

ien

t w

ith

kn

own

pas

t AR

F or

RH

D

2 M

AJOR

or 1 M

AJOR

and

1 M

INOR

or 3 M

INOR

man

ifes

tati

ons

+ evid

ence

of

a p

rece

din

g G

AS in

fect

ion

Prob

able

ARF

(f

irst

epis

ode

or r

ecurr

ence

)

A cl

inic

al p

rese

nta

tion

th

at f

alls

sh

ort

by

eith

er o

ne

MAJO

R or

on

e M

INOR

man

ifes

tati

on,

or t

he

abse

nce

of

stre

pto

cocc

al s

erol

ogy

resu

lts,

bu

t on

e in

wh

ich

ARF

is

con

sid

ered

th

e m

ost

like

ly d

iagn

osis

. Su

ch c

ases

sh

ould

be

furt

her

cat

egor

ised

acc

ord

ing

to t

he

leve

l of

co

nfi

den

ce w

ith

wh

ich

th

e d

iagn

osis

is

mad

e:

• H

igh

ly-su

spec

ted

ARF

• U

nce

rtai

n A

RF