[medical radiology] systemic vasculitis || respiratory tract vasculitis

Respiratory Tract Vasculitis

M. Habib Bouhaouala, Ridha Charfi, Meher Abouda,Nadia Mehiri, Bechir Louzir, and Lotfi Hendaoui

Contents

References .......................................................................... 442

Abstract

The respiratory airway and pulmonary parenchymaare frequently involved in vasculitis particularly insmall-vessel vasculitis associated with antineutro-phil cytoplasmic antibodies. The diagnosis is basedon anamnesis and physical examination butremains largely dependent on high-resolution CTand angiography. The confirmation of diagnosisdepends on tissue biopsy. Conventional chestradiography is usually not very contributive.High-resolution CT is more sensitive in showingthe spectrum of thoracic manifestations but it isnonspecific. MRI and MR angiography can pro-vide information on vessel wall thickening in theearly stage and on luminal changes. PET/CT is agood indicator of inflammation and helpful forfollow-up.

Upper and lower respiratory airway and pulmonaryparenchyma are frequently involved in vasculitis andsometimes represent one of the major ‘‘target organs’’such as in small-vessel vasculitis associated withantineutrophil cytoplasmic antibodies (Wegener’sgranulomatosis, Churg–Strauss syndrome and polyar-teritis nodosa) (Chakravarty 1997; Castaner et al.2010). The diagnosis is based on anamnesis and phys-ical examination but remains largely dependent onhigh-resolution CT and angiography. The confirmationof diagnosis depends on tissue biopsy.

The possibility of vasculitis must be systematicallyevoked in the presence of the following clinical signs:– Pulmonary signs such as nonproductive coughing,

shortness of breath, and wheezing.

M. Habib Bouhaouala (&) � R. CharfiDepartment of Medical Imaging,Medical School of Tunis,Interior Security Forces Teaching Hospital,2070, La Marsa, Tunisiae-mail: [email protected]

M. AboudaDepartment of Pneumology,Medical School of Tunis,Interior Security Forces Teaching Hospital,2070, La Marsa, Tunisia

N. Mehiri � B. LouzirDepartment of Pneumology and Allergology,Medical School of Tunis,La Rabta Teaching Hospital,Tunis, Tunisia

L. HendaouiDepartment of Imaging,Medical School of Tunis,Mongi Slim Teaching Hospital,2070, La Marsa, Tunisia

L. Hendaoui et al. (eds.), Systemic Vasculitis, Medical Radiology. Diagnostic Imaging,DOI: 10.1007/174_2011_242, � Springer-Verlag Berlin Heidelberg 2012

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436 M. Habib Bouhaouala et al.

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Respiratory Tract Vasculitis 437

Ta

ble

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hem

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ifes

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on):

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ral

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ated

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en,

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pher

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rom

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edi

seas

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–14%

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leur

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emen

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hem

ost

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mon

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acic

man

ifes

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on):

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ral

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sion

(not

very

abun

dant

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late

ral

in50

%of

the

case

s,an

dof

ten

asso

ciat

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ith

peri

card

itis

),pl

eura

lfi

bros

isS

eoet

al.

2000

;E

ngel

keet

al.

2002

;D

avie

s20

05;

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iton

ean

dS

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2008

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hung

etal

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gero

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and

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ease

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eval

ence

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ffus

ely

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ters

titi

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ith

area

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chov

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les

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rlob

ular

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bron

chio

lar

infl

amm

atio

nM

aybe

rry

etal

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iton

ean

dS

alva

rani

2008

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tis

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gin

volv

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ffus

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ters

titi

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lmon

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ural

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ent:

poss

ible

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dier

1994

(con

tinu

ed)


Ta

ble

1(c

onti

nued

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culi

tis

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ease

Fin

ding

s

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rode

rma

Wom

en,

30–5

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ars

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gin

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emen

t:pu

lmon

ary

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osis

(the

mos

tco

mm

onra

diog

raph

icfi

ndin

g:20

–65%

)in

itia

lly

afi

nere

ticu

lar

patt

ern

then

area

sof

grou

nd-g

lass

atte

nuat

ion,

poor

lyde

fine

dsu

bple

ural

nodu

les,

reti

cula

rpa

tter

nof

atte

nuat

ion,

hone

ycom

bing

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dtr

acti

onbr

onch

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ith

usua

lly

alo

wer

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ean

dpe

riph

eral

pred

omin

ance

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lmon

ary

hype

rten

sion

(10%

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leur

alin

volv

emen

t:un

com

mon

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dier

1994

;M

aybe

rry

etal

.20

00;

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iton

ean

dS

alva

rani

2008

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ymyo

siti

san

dde

rmat

omyo

siti

sL

ung

invo

lvem

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ndar

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phar

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ew

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ess

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mon

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phic

find

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omin

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gin

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nes

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ha

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bron

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lan

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ural

dist

ribu

tion

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ally

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zing

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agm

atic

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atio

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duce

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es,

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lar

atel

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sis

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berr

yet

al.

2000

;P

ipit

one

and

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vara

ni20

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ylos

ing

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dylo

sis

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gin

volv

emen

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orsp

angl

edop

acit

ies

asso

ciat

edw

ith

irre

gula

rca

vita

ryim

ages

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dier

1994

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plas

tic

Lym

phom

atoi

dgr

anul

omat

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gin

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l-de

fine

dm

asse

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ith

orw

itho

utca

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tion

(up

to80

%)

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tipl

e,bi

late

ral

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les

loca

ted

pred

omin

antl

yin

the

mid

dle

and

low

erlo

bes

Seo

etal

.20

00;

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iton

eet

al.

2008

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ign

lym

phoc

ytic

angi

itis

and

gran

ulom

atos

is

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gin

volv

emen

t:bi

late

ral

(80%

)or

isol

ated

nodu

lar

orin

filt

rati

vear

eas

Seo

etal

.20

00;

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iton

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al.

2008

Mis

cell

aneo

usIn

fect

ious

vasc

ulit

isB

acte

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(pyo

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ith

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holo

gica

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ase

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sis:

gran

ulom

atou

sva

scul

itis

(em

boli

zati

onof

the

smal

lpu

lmon

ary

vess

els

bypa

rasi

teeg

gs)

Myc

osis

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berr

yet

al.

2000

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ung

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ther

than

angi

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ase,

usua

lly

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ater

al(7

5%)

wit

hup

per

zone

pred

omin

ance

Lar

geop

acit

y(2

–6cm

),m

ulti

ple

nodu

les,

area

sof

cons

olid

atio

n,at

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tasi

s,m

ucoi

dim

pact

ion,

reti

culo

nodu

lar

infi

ltra

tes

and

cavi

tati

onS

eoet

al.

2000

(con

tinu

ed)


– Inflammatory involvement of the bronchial treeleading to obstruction of the large airways andbronchial stenosis with obstructive parenchymalcollapse (Chakravarty 1997).

– Diffuse pulmonary hemorrhage often associatedwith primary small vasculitis (Wegener granulo-matosis and microscopic polyangiitis) and com-bining hemoptysis, dyspnea, and decreasinghemoglobin level (Chakravarty 1997; Jayne 2009;Castaneret al. 2010).

– Extrathoracic signs such as general signs (low-gradefever, polymyalgias, polyarthralgias, fatigue,weight loss), temporal headache, visual loss, jawclaudication, uveitis, arthritis, chronic refractorysinusitis or rhinorrhea, acute glomerulonephritis orrenal failure, mononeuritis multiplex, multisystemicdisease, and palpable purpura (Castaner et al. 2010).Conventional chest radiography is usually per-

formed for the initial assessment of pulmonaryvasculitis, but frequently it is not very contributive.High-resolution CT is more sensitive in showingthe spectrum of thoracic manifestations but it isnonspecific (Jayne 2009; Castaner et al. 2010).MRI/magnetic resonance angiography can provideinformation on vessel wall thickening in the earlystage and on luminal changes (narrowing, aneurys-mal dilatation, and occlusion in advanced fibroticstages). PETCT is a good indicator of inflammationand helpful for follow-up (Castaner et al. 2010).The imaging findings are extremely variable andnonspecific. They include (Brillet and Brauner2007; Castaner et al. 2010; Chung et al. 2010):– Cavitary nodules in connection with infection or

parenchymatous infarction;– Micronodules, nodules, and masses predominat-

ing on the periphery and in the upper and midlung territory;

– Interstitial pulmonary changes: diffuse centrilob-ular ground-glass small areas and reticular, retic-ulonodular, or miliairy pattern (uncommon);

– Alveolar opacities• Nonsystematized alveolar opacities in connec-

tion with diffuse pulmonary hemorrhage pre-dominating in the perihilar and basal regions;

• Unifocal or multifocal alveolar opacities ofvariable natures;

– Tracheobronchial involvement;– Vessel wall thickening;T

ab

le1

(con

tinu

ed)

Vas

culi

tis

Dis

ease

Fin

ding

s

Dru

g-an

dfo

reig

n-m

ater

ial-

indu

ced

pulm

onar

yva

scul

itis

Dru

gs(g

emci

tabi

ne,

prop

ylth

iour

acil

,et

c.)

and

crac

kco

cain

e:pu

lmon

ary

capi

llar

itis

and

diff

use

pulm

onar

yhe

mor

rhag

eF

orei

gnm

ater

ial

(tal

c,ce

llul

ose,

mal

tose

,et

c.):

fore

ign

body

angi

ocen

tric

gran

ulom

atou

sre

acti

onap

pear

ing

onth

ech

est

X-r

ayas

smal

lno

dule

san

don

CT

asce

ntri

lobu

lar

smal

lno

dule

sor

vasc

ular

tree

-in-

bud

opac

itie

sw

ith

poss

ible

evol

utio

nto

fibr

osis

Chu

nget

al.

2010

Nec

roti

zing

sarc

oid

gran

ulom

atos

is50

year

s

Tra

chea

lin

volv

emen

t:ra

rely

ofth

edi

stal

trac

hea

orse

vere

caus

ing

airw

ayob

stru

ctio

nL

ung

invo

lvem

ent

Mul

tipl

e,un

ilat

eral

,or

bila

tera

lno

dule

sin

asu

bple

ural

and

peri

bron

chov

ascu

lar

dist

ribu

tion

,so

met

imes

asso

ciat

edw

ith

ill-

defi

ned

opac

itie

sS

olit

ary

nodu

le(2

5%)

Exc

avat

ion

ofth

epa

renc

hym

atou

sle

sion

s:ra

re(u

nlik

ecl

assi

csa

rcoi

dosi

s)M

edia

stin

alin

volv

emen

t:hi

lar

lym

phad

enop

athy

(unu

sual

unli

kecl

assi

csa

rcoi

dosi

s)S

eoet

al.

2000

;P

ipit

one

etal

.20

08

AN

CA

anti

-neu

trop

hil

cyto

plas

mic

anti

bodi

es,

CT

Com

pute

dto

mog

raph

y


– Pulmonary hypertension.Some negative signs such as the absence of pleural

effusion, adenomegaly, heart enlargement or venoushypertension pulmonary are essential to note (Brilletand Brauner 2007).

According to radiological findings, Seo et al. haveclassified thoracic vasculitis findings into three cate-gories (Seo et al. 2000):1. A localized nodular and patchy opacities which are

frequent in the angiitis–granulomatosis group,including Wegener granulomatosis, allergic angi-itis and granulomatosis, necrotizing sarcoid gran-ulomatosis, lymphomatoid granulomatosis, andbronchocentric granulomatosis.

2. A diffuse pulmonary hemorrhage due to capillaritisrealizing a diffuse air-space consolidation whichmay be seen in polyarteritis nodosa, systemic lupuserythematosus, Goodpasture syndrome, mixedcryoglobulinemia, Behçet syndrome, Wegenergranulomatosis, and Henoch-Schönlein purpura.

3. A large pulmonary artery aneurysm or stenosis asin Takayasu arteritis or Behçet syndrome.The main respiratory system findings during the

most important types of vasculitis are summarized inTable 1.

References

Brillet PY, Brauner M (2007) Imagerie pulmonaire dans lesvascularites associées aux ANCA. Presse Med 36:907–912

Castaner E, Alguersuari A, Gallardo X et al (2010) When tosuspect pulmonary vasculitis: radiologic and clinical clues.Radiographics 30:33–53

Chakravarty K (1997) Vasculitis by organ systems. BaillieresClin Rheum 2:357–393

Chung MP, Yi CA, Lee HY, Han J, Lee KS (2010) Imaging ofpulmonary vasculitis. Radiology 2:322–341

Cordier JF (1994) Vascularites pulmonaires. EMC (ElsevierMasson SAS, Paris), Pneumologie 6-024-D-10

Davies DJ (2005) Small vessel vasculitis. Cardiovasc Pathol14:335–346

Engelke C, Schaefer-Prokop C, Schirg E et al (2002) High-resolution CT and CT angiography of peripheral pulmonaryvascular disorders. Radiographics 22:739–764

Guillevin L, Pagnoux C (2006) Principales vascularitesnécrosantes systémiques. EMC (Elsevier Masson SAS,Paris), Appareil locomoteur, 14-245-F-10

Hamzaoui K, Hamzaoui A (2005) Manifestations thoraciquesdes maladies de Behçet et de Takayasu. Rev Mal Respir22:999–1019

Hiller N, Lieberman S, Chajek-Shaul T, Bar-Ziv J, Shaham D(2004) Thoracic manifestations of Behçet disease at CT.Radiographics 24:801–808

Jayne D (2009) The diagnosis of vasculitis. Best Pract Res ClinRheum 23:445–453

Khasnis A, Langford CA (2009) Update on vasculitis. J AllergyClin Immunol 123:1226–1236

Mayberry JP, Primack SL, Müller NL (2000) Thoracicmanifestations of systemic autoimmune diseases: radio-graphic and high-resolution CT findings. Radiographics20:1623–1635

Pipitone N, Salvarani C (2008) Role of imaging in vasculitisand connective tissue diseases. Best Pract Res Clin Rheum6:1075–1091

Pipitone N, Versari A, Salvarani C (2008) Role of imagingstudies in the diagnosis and follow-up of large-vesselvasculitis: an update. Rheumatology (Oxford) 47:403–408

Ponge T, JH Barrier (1999) Maladie de Horton. EMC (ElsevierMasson SAS, Paris), Akos Encyclopédie Pratique deMédecine 5-0365, Angéiologie, 19-1810

Puechal X (2007) Vascularites associées aux anticorps antic-ytoplasme des polynucléaires. Rev Rhum 74:824–832

Quéméneur T, Hachulla E, Lambert M, et al (2007) Manifes-tations pulmonaires de la maladie de Takayasu. EMC(Elsevier Masson SAS, Paris), Pneumologie, 6-024-D-20

Rybojad M, Brudy L (2001) Maladie de Kawasaki. EMC(Elsevier Masson SAS, Paris), Dermatologie, 98-515-A-10

Seo JB, Im JG, Chung JW (2000) Pulmonary vasculitis: thespectrum of radiological findings. Br J Radiol 73:1224–1231


[medical radiology] systemic vasculitis || respiratory tract vasculitis

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