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SUPPORT FOR YOU FROM THE
PULMONARY FIBROSIS FOUNDATION
The Pulmonary Fibrosis Foundation mobilizes people and resources to provide access to high quality care and leads research for a cure so people with pulmonary fibrosis will live longer, healthier lives.
To learn more about how the PFF can help support you, contact the PFF Patient Communication Center at 844.TalkPFF (844.825.5733) or [email protected], or visit the PFF online at pulmonaryfibrosis.org.
SPONSORED BY:
© 2020 PULMONARY FIBROSIS FOUNDATION. ALL RIGHTS RESERVED.
RH
EU
MA
TO
LO
GY
PO
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IDE
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FRIENDSOF THE FOUNDATION
A multidisciplinary discussion (MDD) for patients suspected to have CTD-ILD has many benefits and is crucial to the diagnostic process. MDD can increase diagnostic confidence by refining a provisional diagnosis, enhance interobserver agreement on the diagnosis, and increase diagnostic precision. It is important that CTD patients with evidence of respiratory disease are referred to a pulmonologist. In addition to rheumatology and pulmonary, other areas of specialty that are often involved in MDD for CTD-ILD include thoracic radiology, and pathology. Patients benefit from improved care coordination when MDD is utilized.
Understanding Connective Tissue Disease-Related Interstitial Lung Disease
Rheumatology
Thoracic Radiology Pathology
Pulmonary
Respiratory Therapy
*HRCT images courtesy of Gregory P. Cosgrove, MD
Mitto S, Fell CD. Semin Respir Crit Care Med 2014;35:249.
Organizing pneumonia (dense ground glass)
Reticulation (lines)
Ground glass
Traction bronchiectasis
Acknowledgements Elizabeth Volkmann, MD, MS University of California, Los Angeles Medical Center Los Angeles, CA
TA
BL
E 1
: D
IAG
NO
ST
IC C
LU
ES
TH
AT
SU
GG
ES
T IL
D IN
TH
E S
ET
TIN
G O
F A
PR
EV
IOU
SLY
DIA
GN
OS
ED
CT
D
TA
BL
E 2
: PR
EV
AL
EN
CE
AN
D R
ISK
FA
CT
OR
S F
OR
ILD
BA
SE
D O
N T
HE
UN
DE
RLY
ING
CT
D
Sy
ste
mic
sc
lero
sis
Rh
eu
ma
toid
art
hri
tis
Sjo
gre
n’s
sy
nd
rom
e
My
osi
tis
Sy
ste
mic
lup
us
e
ryth
em
ato
sus
Mix
ed
co
nn
ec
tiv
e
tiss
ue
dis
ea
se
Pre
va
len
ce
o
f IL
D
>65
%
30
-76
%
25%
23
-65
%
3-1
3%
18-6
6%
Ris
k fa
cto
rs o
n h
isto
ry
an
d p
hy
sic
al
Mal
e se
x A
fric
an A
mer
ican
race
D
iffu
se c
uta
neo
us
scle
rosi
s
Mal
e se
x O
lder
ag
e C
igar
ette
sm
oki
ng
Mal
e se
x O
lder
ag
e C
igar
ette
sm
oki
ng
Am
yop
ath
ic d
erm
ato
myo
sitis
O
lder
ag
e A
rth
ritis
No
t wel
l defi
ned
Old
er a
ge
Ray
nau
d p
hen
om
eno
n
Dys
ph
agia
No
n-s
pe
cific
inte
rstit
ial p
neu
mo
nia
(NS
IP) i
s th
e
mo
st c
om
mo
n H
RC
T p
atte
rn s
een
in lu
ng
dis
ease
as
soci
ate
d w
ith C
TD
, alth
ou
gh
oth
er in
ters
titia
l p
atte
rns,
su
ch a
s u
sual
inte
rstit
ial p
neu
mo
nia
, ar
e p
oss
ible
. Th
e N
SIP
pat
tern
is m
ore
co
mm
on
ly
rela
ted
to C
TD
than
idio
pat
hic
NS
IP. D
istin
ctiv
e ch
arac
teri
stic
s se
en in
NS
IP a
re s
ymm
etri
c,
bib
asila
r, g
rou
nd
gla
ss, r
etic
ula
tion
, tra
ctio
n
bro
nch
iect
asis
(dila
tion
an
d d
isto
rtio
n o
f th
e
bro
nch
i or b
ron
chio
les
in a
reas
of fi
bro
sis)
, an
d s
ub
ple
ura
l sp
arin
g.1
1 Ref
eren
ces:
Cap
ob
ian
co J
, et a
l. R
adio
gra
ph
ics
201
2; 3
2: 3
3-5
0.
Ah
uja
J, e
t al.
Rad
iol C
lin N
Am
201
6; 5
4: 1
015
-10
31.
Mu
elle
r-M
ang
et a
l. R
adio
gra
ph
ics
20
07
; 27
: 59
5-6
15.
Han
sell
et a
l. R
adio
log
y 2
00
8; 2
46
; 69
7.
Ris
k fa
cto
rs b
ase
d o
n
lab
ora
tory
stu
die
s
Scl
-70
an
tibo
dy
PM
/Scl
an
tibo
dy
RF
an
tibo
dy
CC
P a
ntib
od
y
AN
A a
ntib
od
y R
F a
ntib
od
y
Jo-1
(PL1
) an
tibo
dy
PL
7 a
ntib
od
y P
L12
an
tibo
dy
MD
A5
an
tibo
dy
dsD
NA
an
tibo
dy
An
ti-S
mith
an
tibo
dy
*
Sy
mp
tom
s
Ear
ly-
Fatig
ue/
Dys
pn
ea
on
exe
rtio
n
Lat
e-
Dys
pn
ea a
t res
t
Man
y p
atie
nts
are
as
ymp
tom
atic
in
early
ILD
Sym
pto
ms
may
b
e n
on
spec
ific
Cer
tain
CT
Ds
hav
e sp
ecifi
c ris
k fa
cto
rs fo
r IL
D a
s d
escr
ibed
in
Tab
le 2
.
Clu
es
Tip
s
Sig
ns
Cra
ckle
s o
n
ausc
ulta
tion
Dig
ital c
lub
bin
g
Oxy
gen
des
atu
ratio
n
with
exe
rcis
e o
r at r
est
Ear
ly in
co
urs
e o
f IL
D,
PF
Ts m
ay b
e n
orm
al,
or t
her
e m
ay b
e an
is
ola
ted
dec
reas
e in
th
e D
LCO
NS
IP is
the
m
ost
co
mm
on
ra
dio
gra
ph
ic p
atte
rn
of I
LD
in C
TD
Ra
dio
gra
ph
ic
Hig
h-r
eso
lutio
n c
hes
t CT
fin
din
gs
con
sist
ent w
ith IL
D
Ret
icu
latio
n (p
erip
her
al “l
ines
” in
the
sub
ple
ura
l sp
ace)
Gro
un
d g
lass
(haz
y) o
pac
ities
an
d/o
r Tra
ctio
n b
ron
chie
ctas
is
(dila
tion
of t
he
airw
ays
larg
er th
an
the
adja
cen
t blo
od
ves
sel)
Ho
ney
com
b c
han
ge
(per
iph
eral
, su
bp
leu
ral “
cyst
s” in
row
s)
Ph
ysi
olo
gic
Res
tric
tive
pat
tern
o
n P
FTs
- L
ow
FV
C
- Lo
w T
LC
Diff
usi
on
Imp
airm
ent
- Lo
w D
LCO
