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CHEST Postgraduate Education Corner

PULMONARY AND CRITICAL CARE PEARLS
A 38-Year-Old Welder With Dyspnea
and Iron Overload
Ariel Modrykamien, MD; Howard Christie, PA-C; Carol Farver, MD;
and Rendell W. Ashton, MD, FCCP
(CHEST 2009; 136:310 313)
38!earo"d #an $resented to t%e $u"#onar!
c"inic &it% a '#ont% %istor! o( $rogressi)e
*and"i+e *urning $ain around %is c%est and s%ar$
sta**ing $ain anterior"!, a"ong &it% s%ortness o(
*reat%- He %ad *een a &e"der (or ./ !ears, #a+ing
iron du#$sters in a $"ant- He noticed s%ortness o(
*reat% &%en c"i#*ing stairs and #oderate
s!#$to#s &%en &a"+ing- He a"so re$orted a dr!
coug%- He denied an! ot%er s!ste#ic s!#$to# and
%ad no ot%er #edica" or
surgica" %istor!- He &as a nons#o+er-
Physical !amination
His )ita" signs &ere as (o""o&s0 1P, ./23.22 ##
Hg4 %eart rate, 58 *eats3#in4 res$irator! rate, .6
*reat%s3 #in4 te#$erature, '8-3784 and arteria"
o9!gen satura tion, '8: &%i"e *reat%ing roo# air- His
$%!sica" e9a# ination &as nor#a" e9ce$t (or ;e"cro
t!$e crac+"es in *ot% "ung *ases-
"a#oratory Findin$s
La*orator! )a"ues at $resentation s%o&ed a %e#at
ocrit o( /2-6: &it% a %e#og"o*in concentration o(
.<-8 g3dL- 8erritin "e)e" &as .,<36 ng3#L =nor#a"
range, .8 to 322 ng3#L>, and iron "e)e" &as .<'
g3dL =nor#a" range, 32 to .62 g3dL>- Trans(errin
saturation &as
<?: =nor#a" range, .. to 6<:>- T%e $atient %ad a
nor#a" @1C and $"ate"et count- His coagu"ation stud
ies, "i)er (unction test, and +idne! (unction test (indings
8ro# t%e Res$irator! Institute =Drs- Modr!+a#ien and As%ton,
and Mr- C%ristie> and De$art#ent o( Anato#ic Pat%o"og! =Dr-
8ar)er>, C"e)e"and C"inic, C"e)e"and, OH-
T%e aut%ors %a)e no con("icts o( interest to disc"ose-
Manuscri$t recei)ed Dece#*er ./, ?2284 re)ision acce$ted
Dece#*er 3., ?228-
Re$roduction o( t%is artic"e is $ro%i*ited &it%out &ritten
$er#ission
(ro# t%e A#erican Co""ege o( C%est P%!sicians =&&&-c%estAourna"-
org3site3#isc3re$rints-9%t#">-
Corres%ondence to& Ariel Modrykamien, MD, Res%iratory 'nsti-
t(te, A)*, Cleveland Clinic, )+** (clid Ave, Cleveland, ,H
--.)+; e-mail& modryka/cc0.or$
D!": 10.1#$8/chest.08%2&54
&ere &it%in nor#a" "i#its- 1"ood g"ucose "e)e"s and
urina"!sis (indings &ere nor#a" as &e""-
Pu"#onar! (unction tests re)ea"ed an 8E;
.
38;C
ratio o( 5<:, an 8E;
.
o( 58: $redicted, and an
8;C o( 8/: $redicted- Di((using ca$acit! o( t%e
"ung (or car*on #ono9ide &as 53: $redicted, and
di((using ca$acit! o( t%e "ung (or car*on #ono9ide
corrected (or %is a")eo"ar )enti"ation &as .2/:
$redicted-
A *ronc%osco$! &as $er(or#ed s%o&ing a nor#a"
air&a!- A *ronc%ia" &as%ing and trans*ronc%ia" *i
o$sies o( t%e rig%t #idd"e "o*e re)ea"ed a*undant
%e#osiderin"aden #acro$%ages- Cu"tures (or *acte
ria" and (unga" in(ections, )iruses, and A81 &ere a""
negati)e- Seru# #ar+ers (or autoi##une diseases,
suc% as antinuc"ear anti*od!, r%eu#atoid (actor, SM,
RNP, SSA, SS1, centro#ere, sc"eroder#a IgB, and
Co. anti*odies, &ere negati)e- Mar+ers (or
)ascu"itides, suc% as c!to$"as#ic antineutro$%i"
c!to$"as#ic anti*od!, $erinuc"ear antineutro$%i"
c!to$"as#ic anti*od!, and g"o #eru"ar *ase#ent
#e#*rane anti*od!, &ere negati)e as &e""- T%e $atient
under&ent a )ideoassisted t%oraco sco$ic surger!
&it% rig%t u$$er, #idd"e, and "o&er "o*e &edge
*io$sies =8ig ., ?> due to t%e "ac+ o( diagnosis-
8igure .- Pu"#onar! $arenc%!#a &it% di((use *ro&n $ig#ent
in)o")ing 52 to 82: o( t%oracosco$ic *io$s!, centered around t%e
s#a"" air&a!s =%e#ato9!"ineosin, origina" .?-/>-
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310 Postgraduate Education Corner
8igure ?- 1ro&n %e#osiderin $ig#ent is $resent $redo#inant"!
&it%in a")eo"ar #acro$%ages surrounding t%e *ronc%o)ascu"ar
area o( t%e *io$s! =%e#ato9!"ineosin, origina" ?22>-
8igure 3- CT scan o( t%e c%est s%o&ing di((use centri"o*u"ar
groundg"ass nodu"ar o$acities-
Radiolo$y Findin$s
C%est radiogra$%! &as nor#a", and a %ig%
reso"ution CT scan o( t%e c%est s%o&ed *i"atera"
di((use centri "o*u"ar groundg"ass nodu"ar o$acities
=8ig 3>-
What is the dia$nosis1
www.chestjournal.org CHEST / 136 / 1 / JUL! "##$ 311
Dia$nosis& Welder2s siderosis 3%ne(moconiosis
siderotica4
Discussion
@e"derDs siderosis or $neu#oconiosis siderotica
&as origina""! c%aracteriEed *! Doig and McLaug%
"in as a "ung disease caused *! t%e in%a"ation o( iron
dust- In t%eir re$ort, t%e aut%ors descri*ed .<
e"ectric arc &e"ders &%o re#ained in a good state o(
%ea"t% des$ite t%e diagnosis o( siderosis- T%e! con
c"uded t%at t%is entit! &as not associated &it% res$i
rator! s!#$to#s or (unctiona" i#$air#ent-
Pneu#oconiosis can *e c"assi(ied as (i*rotic or
non(i*rotic, de$ending on t%e $resence o( (i*rosis in
t%e "ung *io$s! s$eci#en- 8i*rotic $neu#oconioses
usua""! $resent &it% s!#$to#s suc% as coug% and
d!s$nea, di((erentiating it (ro# non(i*rotic (or#s-
Si"icosis, coa" &or+er $neu#oconiosis, as*estosis,
ta"cosis, and *er!""iosis are e9a#$"es o( (i*rotic $neu
#oconiosis, &%ereas *aritosis, stannosis, and sidero
sis are non(i*rotic (or#s-
@e"ding in)o")es #i9tures o( #eta"s suc% as iron,
#anganese, si"ica, c%ro#iu#, nic+e", and ot%ers- In
situations &%ere t%e )enti"ation is $oor or t%e &e"d
ing acti)it! is $er(or#ed in con(ined s$aces, t%e
$ro*a*i"it! (or t%e de)e"o$#ent o( "ung disease
increases- T%e acFuired disease de$ends on t%e
s$eci(ic (u#e e9$osure, t%e coating o( t%e rods, t%e
&e"ding o$eration $er(or#ed, and t%e #eta" &e"ded-
T%ere(ore, it is essentia" to distinguis% G&e"dersD
$neu#oconiosis,H &%ic% re(ers to t%e e((ect o( in%a"
ing %eterogeneous dusts generated *! &e"ding, (ro#
G&e"dersD siderosis,H &%ic% is t%e e((ect o( in%a"ed
iron o9ide $artic"es a"one-
En)iron#enta" "e)e"s o( (u#es and $artic"es are
t%oug%t to *e direct"! re"ated to t%e de)e"o$#ent o(
"ung disease- Hence t%e Occu$ationa" Sa(et! and
Hea"t% Ad#inistration o( t%e US De$art#ent o(
La*or u$dated t%e $er#issi*"e e9$osure "i#it esta*
"is%ed (or &e"ding (u#es to / #g3#
3
- Ho&e)er,
des$ite t%is u$dated regu"ation, t%ere are sti"" re$orts
o( acute "ung diseases in &e"ders e9$osed to "e)e"s as
"o& as 6-3 #g3#
3
-
1ecause &e"dersD siderosis is a non(i*rotic $neu
#oconiosis, it &as origina""! t%oug%t not to *e
associated &it% res$irator! s!#$to#s- Ne)ert%e
"ess, so#e aut%ors descri*ed s!#$to#atic disease
&it% interstitia" (i*rosis in arc &e"ders- It is
t%oug%t t%at t%ese cases #ig%t %a)e occurred
secondar! to conco#itant in%a"ation o( si"icates
and iron =si"icosiderosis>-
So#e re$orts %a)e descri*ed t%e occurrence o(
&e"dersD siderosis associated &it% s!ste#ic iron o)er
"oad- T%ree cases o( &e"dersD siderosis &it% e"e)ated
(erritin, seru# iron "e)e", and seru# trans(erring
saturation %a)e *een re$orted- None o( t%ese $a
tients %ad %e#oc%ro#atosis, t%a"asse#ia, or sider
o*"astic ane#ia, and t%ere &as no %istor! o( #u"ti$"e
*"ood trans(usions, iron ingestion, or a"co%o" con
su#$tion- T%e aut%ors conc"uded t%at iron o)er"oad
see#ed "i+e"! to *e due to occu$ationa" e9$osure-
C%est CT i#aging in $atients &it% $neu#oconio
sis siderotica de#onstrates s#a"" nodu"es t%at are
#ore $ro#inent in t%e #idd"e t%ird o( t%e "ung-
T%ese nodu"es indicate radio$aFue accu#u"ation o(
iron $artic"es in #acro$%ages aggregated a"ong t%e
$eri*ronc%o)ascu"ar *und"e- T%ese c%anges are usu
a""! re)ersi*"e and #a! co#$"ete"! reso")e a(ter
cessation o( e9$osure-
It is i#$ortant to di((erentiate &e"dersD siderosis
(ro# t&o ot%er entities0 idio$at%ic $u"#onar! %e#o
siderosis and #eta" (u#e (e)er-
Idio$at%ic $u"#onar! %e#osiderosis is a rare in
terstitia" "ung disease o( un+no&n etio"og! t%at oc
curs $re(erentia""! in c%i"dren- Its c"inica" $resenta
tion )aries (ro# an acute and (u"#inant (or# &it%
di((use a")eo"ar %e#orr%age to a #ore su*acute
$resentation &it% coug%, d!s$nea, re$etiti)e %e#o$
t!sis, and ane#ia- S$utu# and 1AL ("uid e9a#ina
tion s%o& #u"ti$"e %e#osiderin"aden #acro$%ages,
and t%e "ung *io$s! con(ir#s a *"and a")eo"ar %e#
orr%age &it% )aria*"es degrees o( (i*rosis- Meta"
(u#e (e)er, or GMonda! #orning (e)er,H is a se"(
"i#iting in%a"ation (e)er attri*uted to a nu#*er o(
#eta" o9ide (u#es- It $resents &it% (e)er, %eadac%e,
(atigue, and d!s$nea &it%in 3 to .2 % a(ter t%e
e9$osure to %eated #eta"s, inc"uding ga")aniEed
stee", Einc o9ide, #agnesiu# o9ide, and c%ro#iu#-
Its diagnosis is *ased on c"inica" sus$icion, and
$re)enti)e strategies are ai#ed at reducing (u#e
e9$osure-
Clinical Co(rse
Our $atient &it% &e"dersD siderosis $resented &it%
s!#$to#s o( c%est $ain, se)ere s%ortness o( *reat%,
and coug%- A"t%oug% $atients &it% non(i*rotic $neu
#oconiosis usua""! $resent &it%out res$irator! s!#$
to#s, on occasion, t%e! #a! *e s!#$to#atic- T%e
$resence o( iron o)er"oad &as t%oug%t to *e re"ated
to t%e in%a"ation o( iron dust *ecause %e did not %a)e
a %istor! (or ot%er sources o( endogenous or e9oge
nous iron o)er"oad- @e reco##ended t%e $atient to
&ear res$irator! $rotection =res$irator> and encour
aged %i# to &or+ in areas &it% an e((ecti)e )enti"a
tion s!ste#- @e a"so reco##ended t%at a"" &e"ders
in %is $"ant *e screened (or seru# (erritin, tota" iron
"e)e", and iron *inding ca$acit!-
312 Postgraduate Education Corner