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Diseases of the Lung
Parenchyma
Structure of the Alveolar Wall
Cell Types
Type IEpithelialCell
Type 2 EpithelialCell
AlveolarMacrophage
Fibroblast
Interstitium
Diffuse Interstitial Pulmonary Fibrosis
Pathology
pqthogenesis
ClinicalFeatures
.PulmonaryFunction
VeiltilatoryGapacityand,Mf!fhanics,t
Clinical Features
PulmonaryFunction
HypersensitivityPneumonitis
Pathology
Clinical Features
PulmonaryFunction
I
InterstitialDisease CausedbyDrugs,
I
Poisons, andRadiation
Collagen Diseases
LymphangitisCarcinomatosa
of the Pleura
Pneumothorax
SpontaneousPneumothorax
Tension Pneumothqrax
eneumothoraxComplicating Lung
81
j

Disease
--
--
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-
-
82 PART II Function of
Restrictive diseases are those in which the expansion of the lung is restricted either
because of alterations in the lung parenchyma or because of disease of the pleura,
the chest wall, or the neuromuscular apparatus. They are characterized by a
reduced vital capacity and a small resting lung volume (usually), but the airway
resistance (related to lung volume) is not increased. These diseases are therefore
different from the obstructive diseases in their pure form, although mixed restric-
tive and obstructive conditions can occur.
C1 Il!SEASES OF THE LUNG PARENCHYMA
This term refers to the alveolar tissue of the lung. A brief review of the structure
of this tissue is pertinent.
Strudure of the Alyeolar Wall
Figure 5-1 shows an electron micrograph of a pulmonary capillary in an alveolar
wall. (25) The various structures through which oxygen passes on its way from the
alveolar gas to the hemoglobin of the red blood cell are the layer of pulmonary sur-
factant (not shown in this preparation), alveolar epithelium, interstitium, capillary
endothelium, plasma, and erythrocyte.
Cell Types
The various cell types have different functions and different responses to injury.
Type 1 Epithelial Cell
This is the chief structural cell of the alveolar wall; its long protoplasmic exten-
sions pave almost the whole alveolar surface (Figure 5-0. The main function of
this cell is mechanical support. It rarely divides and is not very active metaboli-
cally. When type 1 cells are injured, they are replaced by type 2 cells, which later
transform into type 1 cells.
Type 2 Epithelial Cell
This is a nearly globular cell (Figure 5-2) (26) that gives little structural support
to the alveolar wall but is metabolically active. The electron micrograph shows
the lamellated bodies that contain phospholipid. This is formed in the endo-
plasmic reticulum, passed through the Golgi apparatus, and eventually extruded
into the alveolar space to form surfactant. (See Respiratory Physiology: The
Essentials, 7
th
ed., p. 99.) After injury to the alveolar wall, these cells rapidly
divide to line the surface and then later transform into type 1 cells. A type 3 cell
has also been described, but it is rare and its function is unknown.
AlveQlar Macrophage
This scavenger cell roams around the alveolar wall phagocytosing foreign particles
and bacteria. The cell contains lysozymes that digest engulfed foreign matter.
Fibroblast
This cell synthesizes collagen and elastin, which are components of the interstitium
of the alveolar wall. After various disease insults, large amounts of these materials
may be laid down. This results in interstitial fibrosis.
COL--
RBC-.....
FIGURE 5-1 Electron Mic
collagen; PL, plasma; RBe, r
(From Weibel ER. Morpholoi'
53:419-495.)
Interstitium
This fills the space betw(
lium. Figure 5-1 shows th:
only of the fused baserne
On the other side of the
fibrils of type I collagen. '
across the endothelium,
exchange (see Figure 6-1
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sion of the lung is restricted either
. because of disease of the pleura,
They are characterized by a
olume (usually), but the airway
;ed. These diseases are therefore
ire form, although mixed restric-
ICHYMA
A brief review of the structure
llonary capillary in an alveolar
ygen passes on its way from the
are the layer of pulmonary sur-
ithelium, interstitium, capillary
8. different responses to injury.
1; its long protoplasmic exten-
Ire 5-0. The main function of
ld is not very active metaboli-
:ed by type 2 cells, which later
t gives little structural support
he electron micrograph shows
This is formed in the endo-
ratus, and eventually extruded
:e Respiratory Physiology: The
eolar wall, these cells rapidly
into type 1 cells. A type 3 cell
ion is unknown.
phagocytosing foreign particles
st engulfed foreign matter.
components of the interstitium
Irge amounts of these materials
CHAPTER 5 Restrictive Diseases 83
PL
FIGURE 5-1 Electron Micrograph ofPortion ofan Alveolar Wall. EP, epithelium; COL,
collagen; PL, plasma; RBe, red blood cell; EN, endothelial cell; IN, interstitium (x 6300).
(From Weibel ER. Morphological basis ofalveolar-capillary gas exchange. Physio/ Rev 1973;
53:419-495.)
Interstitium
This fills the space between the alveolar epithelium and the capillary endothe-
lium. Figure 5-1 shows that it is thin on one side of the capillary, where it consists
only of the fused basement membranes of the epithelial and endothelial layers.
On the other side of the capillary, the interstitium is usually wider and includes
fibrils of type I collagen. The thick side is chiefly concerned with fluid exchange
across the endothelium, whereas the thin side is responsible for most of the gas
exchange (see Figure 6-0.
84 PART II Function ofthe Diseasecntirig:-
FIGURE 5-2 Electron Micrograph of Type 2 Epithelial Cell (x 10,000). Note the lamel-
lated bodies (LB), large nucleus, and microvilli (arrows), which are mainly concentrated
around the edge of the cell, and cytoplasm rich in organelles. The inset at top right is a scan-
ning electron micrograph showing the surface view of a type 2 cell With its characteristic
distribution of microvilli (x 3400). (From Weibel ER, Gil J. Structure-function relationships at
the alveolar level. In: West JB. ed. Bioengineering Aspects of the Lung. New York: Marcel
Dekker, 1977.)
Interstitial tissue is found elsewhere in the lung, notably in the perivascular
and peribronchial spaces around the larger blood vessels and airways and in the
interlobular septa. The interstitium of the alveolar wall is continuous with that
in the perivascular spaces (see Figure 6-1) and is the route by which fluid drains
from the capillaries to the lymphatics.
Diffuse Interstitial Pulmonary Fibrosis
The nomenclature this condition is confusing. Synonyms include idiopathic
pulmonary fibrosis, interstitial pneumonia, and cryptogenic fibrosing alveolitis.
Some physicians reserve the term "fibrosis" for the late stages of the disease. The
changes in pulmonary function are described in detail because they are typical of
many of the other conditions alluded to later in this chapter.
Pathology
The principal feature is
Initially there is infiltratio
appear and lay down thic
may be dispersed irregular!
consisting of macrophages
in the early stages of the c
alveolar architecture is df
cystic spaces formed by d
honeycomb lung.
Pathogenesis
This is unknown, althoug
reaction.
FIGURE 5-3 Electron Micrc
the thick bundles of collagel
PC plasma. Compare Figure 5-
membrane in idiopathic intCi
cases. Am Rev Respir Dis 19E
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Cell (x 10(000).Note the lamel-
), which are mainly concentrated
les. The inset at top right is a scan-
I type 2 cell with its characteristic
. Structure-function relationships at
:ts of the Lung. New York: Marcel
g, notably inthe perivascular
vessels and airways andinthe
rwall is continuouswith that
he route by whichfluid drains
;is
Synonyms include idiopathic
yptogenic fibrosing alveolitis.
latestagesofthedisease. The
tailbecausetheyaretypicalof
lis chapter.
'CHA!>TER 5 Restrictive Diseases 85
Pathology
The principal feature is thickening of the interstitium of the alveolar walL
Initiallythereis infiltrationwithlymphocytesandplasmacells.Later,fibroblasts
appear and lay down thick collagen bundles (Figure 5-3). (27) These changes
maybedispersedirregularlywithinthelung. Insomepatients, acellularexudate
consistingofmacrophagesandothermononuclearcells is seenwithinthealveoli
in the earlystagesofthedisease. Thisis called "desquamation."Eventually, the
alveolar architecture is destroyed and the scarring results in multiple air-filled
cystic spaces formed by dilated terminal and respiratory bronchioles, so-called
honeycomblung.
Pathogenesis
This is unknown, although insome cases there is evidenceofan im)TIunologic
reaction.
FIGURE 5-3 Electron Micrograph from a Patient with Diffuse Interstitial Fibrosis. Note
the thick bundles of collagen. COL, collagen; ALV, alveolar space; RBC, red blood cell;
PL, plasma. Compare Figure 5-1. (From Gracey DR, Divertie MD, Brown AL, Jr. Alveoiar-capillary
membrane In idiopathic interstitial pulmonary fibrosis. Electron microscopic study of 14
cases. Am Rev Respir Dis 1968;98:16-21.)
i
:
I
-
-
- - --
--
--
86 PART II Function of
Clinical Features
The disease is not common and tends to affect adults in late middle age. The
patientoftenpresentswithdyspnea withrapid, shallow breathing. Thedyspnea
typicallybecomesmore significantonexercise (compareFigure 3-4).Anirritat-
ing, unproductivecough is oftenpresent.
Onexamination, mildcyanosismaybeseenatrestinseverecases.Ittypically
worsens onexercise. Finecrepitationsare usually heard throughout both lungs,
especially toward theendofinspiration. Fingerclubbing is common. Thechest
radiograph shows a reticular orreticulonodularpattern, especially at thebases.
Patchyshadowsnearthediaphragmmay be caused by basalcollapse. Lateinthe
disease, a honeycomb appearance is often seen; this is caused by multiple air-
spacessurrounded by thickenedtissue.
Cor pulmonale or pneumonia may complicate the picture, and the patient
may develop respiratory failure terminally. The diseases often progress insidi-
ously, althoughanacuteformdoesoccur,asoriginallydescribedby Hammanand
Rich. (28)
Pulmonary Fundion
Ventilatory Capacity and Mechanics
Spirometry typically reveals a restrictive pattern (see Figure 1-2). The FYC is
markedlyreducedbutthegas isexhaledrapidlyso thatalthoughtheFEY
l
is low,
theFEY/FYC %may exceed thenormal value. Thealmost square shapeofthe
forced expiratory spirogram is in striking contrast to the obstructive pattern
(compareFigure4-15).TheFEF
z5
-
7
5% is normalorhigh.Theflow-volumecurve
does notshow thescooped-outshapeofobstructivedisease, andtheflow rate is
oftenhigher thannormal when related to absolute lung volume. This is shown
inFigure 1-5, where itcanbeseenthatthedownslopeofthecurvefor restrictive
disease lies above thenormalcurve.
All lung volumes are reduced, including the TLC, FRC, and RY, but the
relativeproportionsaremoreorlesspreserved.Thepressure-volumecurveofthe
lung is flattened and displaced downward (see Figure 3-1), so thatatany given
volume, the transpulmonary pressure is abnormally high. Themaximumelastic
recoil pressure that can be generated at TLC is typically higher than normaL
Airway resistance is normalorlow whenrelated to lungvolume.
All these results are consistentwith the pathologic appearance offibrosis
ofthealveolarwalls (Figures 2-5 and 5-3). Thefibrous tissue reduces thedis-
tensibilityofthe lung just as a scaronthe skin reduces its extensibility. As a
result, thelungvolumesaresmall, andabnormally largepressuresarerequired
to distend the lung. The airways may not be specifically involved. but they
tend to narrow as lung volume is reduced. However, airway resistance at a
given lung volume is normal or even decreased because the retractiIe forces
exerted on the airway walls by the surrounding parenchyma are abnormally
high (Figure 5-4). Thepathologiccorrelate ofthis is thehoneycomb appear-
ancecausedbythedilatedterminalandrespiratorybronchiolessurroundedby
thickenedscar tissue.
Normal
FIGURE 5-4 AirwayCaliber
airways tend to collapse becal
traction may be excessive, wit
volume.
Gas Exchange
ThearterialP
02
andPC02 a
emia is usually mild atrest
the POz oftenfalls dramati(
disease, both thephysiolog
Therelative contributi(
eVA/Q) inequalityto thel-
is natural to argue thatthe
slow thediffusionofoxyge
thethicknessofthebarrier
addition, theincreasinghy
anism ofimpaired diffusiOl
cell inthepulmonarycapil
However, we now know
hypoxemia inthese condit
diffusion inthattheP0
2
of
itstransitthrough thecapil
Features of Pulmonary FUI
Dyspnea with shallow, rap
Reductions inall lung
FEV/FVC ratio normal or
Airway resistance normall
Reduced lung
Very negative intrapleural i
ArteriaLhypoxemia chiefly'
Diffusion impairmentpos!
Normal or lowarterial Pc6
Reduced diffusing
pulmonaryvase
i

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ts in late middle age. The
)w breathing. Thedyspnea
are Figure 3-4).Anirritat-
inseverecases.It typically
lrd throughout both lungs,
ling is common.Thechest
rn. especially at the bases.
,basalcollapse. Lateinthe
is caused by multiple air-
Le picture, and the patient
often progress insidi-
describedbyHammanand
e Figure 1-2). The FVC is
It althoughtheFEV1 is low,
almost square shape ofthe
to the obstructive pattern
gh.TheHow-volume curve
:iisease, and theHow rate is
ung volume. This is shown
eofthecurvefor restrictive
.C, FRC, and RY, but the
ressure-volumecurveofthe
3-1),so thatatany given
1igh. Themaximumelastic
lically higher than normal.
ungvolume.
ogic appearance offibrosis
rous tissue reduces thedis-
luces its extensibility. As a
largepressuresarerequired
ifically involved, but they
ver, airway resistance at a
:cause the retractile forces
arenchyma are abnormally
is thehoneycomb appear
bronchiolessurroundedby
5 Restrictive Diseases 87


Normal Emphysema Fibrosis
FIGURE 5-4 Airway Caliber in Emphysema and Interstitial Fibrosis. In emphysema, the
ailWays tend to collapse because of the loss of radial traction. By contrast, in fibrosis, radial
traction may be excessive, with the result that ailWay caliber is large when related to lung
volume,
Gas Exchange
ThearterialPaz andP
eoz
are typicallyreducedandthepHis normal.Thehypox-
emia is usually mild at rest until the disease is advanced. However, onexercise,
the Paz oftenfalls dramatically andcyanosismaybe evident. Inwellestablished
disease, boththephysiologicdeadspace andphysiologicshuntare increased.
The relative contributionofdiffusion impairmentand ventilation-perfusion
(VA/OJ inequalitytothehypoxemiaofthese patientshas longbeendebated. It
is natural toargue thatthehistologicappearancesshowninFigures 2-5 and5-3
slow thediffusionofoxygenfrom thealveolargas tothecapillarybloodbecause
thethicknessofthebarriermaybe increasedmanyfold (compareFigure5-l).Jn
addition, theincreasinghypoxemiaduringexercise is consistentwiththemech-
anism ofimpaired diffusion because exercise reduces the time spent by the red
cell in thepulmonarycapillary (Figure 2-4).
However, we now know that impaired diffusion is not thechiefcause ofthe
hypoxemia in theseconditions. First, the normal lunghasenormousreservesof
diffusion inthatthePaz ofthebloodnearlyreachesthatinalveolargas early in
itstransitthroughthecapillary (seeFigure2-4). Inaddition,thesepatientshave
Features of Pulmonary Fundion in
,'" "
Dyspnea with shallow, rapid breathing
Reductions in all lung volumes
FEV/FVC ratio normal or even increased" ,
AilWay resistance nOf\llal or low when relatedio.lung'volume
Reduced lung compliance
Very negative intrapleural pressure arTLC , "
Arterial hypoxemia chiefly 9l.1e to VA/el
Diffusion impairment possibly contributing 'tothe:hYR0l'emiaduring exercise
. '. ,.', .".,.{_.,.fil
Normal or low artenal Pco;,> I.: ..+. ," , '
Reduced diffusing capac:;ity for carbon
Increased pulmonary vascular resistance,
_M.___ __ ...
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--
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--
88 PART II Function oftheDiseased lung,
substantial inequalityofventilationandbloodflow withinthe lung. Howcould
theynot,withthedisorganizationofarchitectureshown inFigures 2-5 and5-3?
The inequalities have been demonstrated by single-breath nitrogen washouts
and measurementsoftopographicalfunctionwithradioactivegases.
Toapportionblamefor thehypoxemiabetweenthetwopossiblemechanisms,
it is necessary to measure the degree of\IA/Q inequality and determine how
muchofthehypoxemia is attributable to this. Thishas beendone by using the
multiple inert gas eliminationtechnique in a series ofpatientswith interstitial
lung disease. (29) Figure 5-5 shows that at rest the hypoxemia could be ade-
quatelyexplained by thedegree of\lA/Q inequality in thesepatients. However,
Figure 5-6 shows that on exercise, the observed alveolar P
02
was conSistently
below the value predicted from the measured amount of\lAIC!. inequality, and
thusanadditionalcause ofhypoxemia musthave beenpresent. Most likely this
was diffusion impairment in these patients. However, hypoxemia caused by
diffusion impairmentwas onlyevidentonexercise, andeventhen it accounted
for onlyaboutone-thirdofthealveolar-arterialdifferencefor Poz.
The low arterial P
e
o
2
in these patients (typically in the middle or low 30s)
occurs despite theevident\lAIC!. inequality and is caused by increased ventila-
tionto thealveoli (compareFigure 2-9).Thecauseoftheincreasedventilation
is uncertain.Thereis some evidencethatthecontrolofventilationis abnormal
because ofstimulationofreceptorswithin thelung (see latertext). Stimulation
oftheperipheralchemoreceprorsby thearterialhypoxemiamayalso beafactor.
ThearterialpHis usuallynormalatrestbutmay increaseconsiderablyonexercise
as a result ofthe hyperventilation and consequentrespiratory alkalosis (compare
Figure3-4),althoughmetabolicacidosiscausedby lacticaddaccumulationmay
also occur. Interminalrespiratoryfailure, thepHmay fall.
Thediffusing capacityfor carbonmonoxideis oftenstrikinglyreduced inthese
patientstotheneighborhoodof5ml/minpermmHg(normalvalue25-30depend-
ing on age and stature). This may be a useful diagnostic pointer: ifthe diffusing
capacityis notlow, thediagnosisshouldberegardedwithsuspicion.Thereduction
iscaused inpartbythethickeningof theblood-gasbarrier(Figure2-5).Inaddition,
thebloodvolumeofthepulmonarycapillariesdecreasesbecausemanyofthevessels
are obliterated by thefibrotic process. A further factor in the lower measured dif-
fusing capacity is probably theV",/Q inequality, whichcauses unevenemptyingof
thelung.Thediffusingcapacityshouldnotbetakentoreflectonlythepropertiesof
theblood-gasbarrier.
Exercise
Patients with mild diffuse interstitial fibrosis may show much more evidence of
impaired pulmonary function on exercise than at rest. The changes shown in
Figure 3-4B are typical, although this patienthad hypersensitivity pneumonitis
(seelatertext).NotethatthemaximalO
2
intakeandCOz outputwere severely
limitedcomparedwiththenormalvaluesofFigure3-4A.Theincreaseinventi-
lationonexercisewas greatlyexaggerated.Thisexaggerationwaschietlycaused
by thehigh rateofbreathing, which rose to over 60breaths per minuteduring
ma:dmalexercise.
120
100
Oi
J:
E
..
80
0'"
0.
60
iii
.;:
Q)
1::
111
1:1
40
Q)
...
(J
'6
I!! 20

0.
0
0
FIGURE 5-5 Study of the
Interstitial lungDisease.This
of Inequalityagreed well
emia could be explained by thl
120
100
Oi
J:
E
..
80
0
'"
0.
:s
60
Gi
1::
ra
1:1
40
S
(J
'6
Q)
0.
...
20


0
FIGURE 5-6 ResultsObtainE
Undertheseconditionstheme,
the pattem of VA/Q inequality.
sumably diffusion Impairment.
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-
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-
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-
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)w withinthe lung. Howcould
showninFigures 2-5 and5-3?
ngle-breath nitrogen washouts
1 radioactivegases.
nthetwopossiblemechanisms,
inequality and determine how
his has beendone by using the
'ies ofpatients with interstitial
the hypoxemia could be ade-
ity in these patients. However,
alveolar Poz was consistently
110unt ofVA/o. inequality, and
beenpresent. Most likely this
)wever, hypoxemia caused by
ie, andeven thenit accounted
ifferencefor Poz'
ally in the middle or low 305)
is caused by increased venti la-
lse ofthe increased ventilation
,trol ofventilationis abnormal
19 (see latertext). Stimulation
ypoxemiamay alsobeafactor.
lcrease considerablyonexercise
_respiratory alkalosis (compare
I lacticacid accumulationmay
mayfall.
ftenstrikingly reduced in these
g(normalvalue25-30depend-
pointer: ifthe diffusing
1withsuspicion.Thereduction
larrier(Figure2-5). Inaddition,
3sesbecausemanyofthevessels
:torin the lower measured dif-
ichcauses unevenemptyingof
toreflectonlythepropertiesof
showmuchmore evidence of
t rest. The changes shown in
hypersensitivity pneumonitis
andCOzoutputwere severely
3-4A.Theincreaseinventi-
[aggerationwas chieflycaused
60 breaths per minute during
0 20 40 60 80 100 120
MeasuredarterialP02(mmHg)
FIGURE 5-5 Study of the Mechanism of Hypoxemia in a Series of Patients with
Interstitial Lung Disease. This figure showsthatthe arterial POL predicted from the pattern
ofVA/a. inequalityagreed well with the measured arterial po). Thus at rest, all ofthe hypox-
emia could be explained bythe uneven ventilation and blood flow.
g 80
0'"
D..
60


I.\l
"
J!!
40
u
'0
20
D..
0
CHAPTER 5 Restrictive Diseases 89
120
I
IRESTI
100 r
_/A
Regression
line
-/

A
Inspiredgas
Air
,'0'0<:'
50%02
120 ,-----; ----.- --..... -
c; 100
l:
E
g 80
o 0;;-
Regression 0,lf/0

line Jf'

60

;'

I.\l
"
40


D..
20
.0 16""-__'--__'--__'--__'--__'--_---'
o 20 40 60 80 106 1.20
MeasuredarterialP0
2
(mmHg)
FIGURE 5-6 ResultsObtainedonExerciseintheSame Patientsas Shown in Figure5-5.
Undertheseconditionsthemeasuredarterial P0
2
was systematicallybelowthatpredictedfrom
the pattern of VA/a. inequality_ ThiS Indicates an additional mechanism for hypoxemia" pre-
sumably diffusion impairment
90 PART II Function of tHe
As a result of the high ventilation, which was out proportion to the
increase in O
2
uptake and CO
z
output, the alveolar and arterial fell and the
alveolar Paz rose. However, as noted earlier, the arterial fell, thus increasing
the alveolar-arterial difference for Paz. This result can be explained partly by the
impaired diffusion characteristics of the lung (Figure 5-6). However, most of the
hypoxemia on exercise was caused by VA/Q inequality.
One factor that tends to reduce the arterial Paz on exercise is the abnormally
small rise in cardiac output. These patients typically have an increased pul-
monary vascular resistance. This is particularly evident on exercise, during which
the pulmonary artery pressure may rise substantially. The high resistance is
caused by obliteration of much of the pulmonary capillary bed by the interstitial
fibrosis (see Figure 2-5). Another factor is hypertrophy of vascular smooth muscle
and consequent narrowing of the small arteries. It is important ro appreciate that
an abnormally low cardiac output in the presence of VA/Q inequality can cause
hypoxemia. One way of looking at this is that a low cardiac output results in a
low Paz in the mixed venous blood (see Chapter 9). As a consequence, a lung
unit with a given VA/Q will oxygenate the blood less than when the mixed
venous Paz is normal.
The importance of this factor can be seen if we consider some results obtained
in out laboratory in a patient with interstitial lung disease. During exercise that
raised the O
2
uptake from about 300 to 700 ml/min, the arterial Paz fell from
50 to 35 mm Hg. The rise in cardiac output was only from 4.6 to 5.7 L/min; the
normal value for this level of exercise is approximately 10 L/min. As a result, the
Paz in the mixed venous blood fell to 17 mm (normal value is approximately
35 mm Hg). Calculations show that if the cardiac output had increased to
10 L/min (and the pattern ofVA/Q inequality remained unchanged), the arterial
Paz would have been some 10 mm Hg higher.
If the diffusing capacity for carbon monoxide is measured in these patients
during exercise, it typically remains low, whereas it may double or triple in normal
subjects.
Conuol of VenUlaUon
We have already seen that these patients typically have shallow rapid breathing,
especially on exercise. The reason for this is not certain, but it is possible that the
pattern is caused by reflexes originating in pulmonary irritant receptors or J (jux-
tacapillary) receptors. former lie in the bronchi or in the epithelial lining
and may be stimulated by the increased traction on the airways caused by the
increased elastic recoil of the lung (Figure 5-4). The Jreceptors are in the alve-
olar walls and could be stimulated by the fibrotic changes in the interstitium. No
direct evidence of increased activity of either receptor is yet available in humans,
but work in experimental animals suggests that it could cause rapid shallow
breathing.
The rapid s4allow pattern of breathing reduces the respiratory work in
patients with reduced lung compliance. However, it also increases ventilation of
the anatomic dead space at the expense of the alveoli, so a compromise must be
reached.
Other Types of Pare
The changes in pulmonary
dealt with at some length
forms of parenchymal rest
briefly here, and difference:
(Figure 5-7).
Sarcoidosis
This disease is characterize
characteristic histologic apl
Pathology
The characteristic lesion is
large histiocytes with
lymph nodes, lungs, skin,
monary fibrotic ch,
Pathogenesis
This is unknown, although
is that an unknown antige
results in activation of a T I
activated mactophage may
blasts, thus explaining the (
FIGURE 5-7 Chest Radiogra
the small contracted lung and
appearance in Figure 4-BA).
-
-- ...........
-
-=$

---
-
I
) out of proportion to the
nd arterial Peoz fell and the
rial Poz fell, thus increasing
IIbe explained partly by the
5-6). However, most of the
ty.
IIexercise is the abnormally
Jly have an increased pul-
lt on exercise, during which
Hy. The high resistance is
)illary bed by the interstitial
.y of vascular smooth muscle
mportant to appreciate that
;VAIQ inequality can cause
I cardiac output results in a
I. As a consequence, a lung
less than when the mixed
nsider some results obtained
[isease. During exerCise that
'1., the arterial Poz fell from
y from 4.6 to5.7 L/min; the
ly 10 L/min. As a result, the
Irmal value is approximately
lC output had increased to
led unchanged), the arterial
measured in these patients
ay double or triple in normal
:lYe shallow rapid breathing,
lin, but it is possible that the
y irritant receptors Of J(jux-
.i or in the epithelial lining
1 the airways caused by the
:Jreceptors are in the alve-
.nges in the interstitium. No
)f is yet available in humans,
could cause rapid shallow
es the respiratory work iJ,1
also increases ventilation of
IIi, so a cOIJ1promise must be
Dise\,!ses 91
Other Types of Parenchymal Restridive Disease
The changes ih pulmonary function in diffuse interstitial pulmonary fibrosis were
dealt with at some length because this disease serves as a prototype for other
forms of parenchymal restrictive disease. These diseases are now considered
briefly and differences in their pattern of pulmonary function are discussed
(Figure 5-7).
i
I
Sarcoidosis
This disease is characterized by the presence of granulomatous tissue having a
characteristic histologic appearance. It often occurs in several organs.
Pathology
The characteristic lesion is the noncaseating epithelioid granuloma composed of
large hist'iocytes with giant cells and lymphocytes. This lesion may occur in the
lymph nodes, lungs, skin, eyes, lrver, spleen, and elsewhere. In advanced pul-
monary disease, fibrotic changes in the alveolJlr walls are seen.
Pathogenesis
This is unknown, although an immunologic basis appears likely. One possibility
is that an unknown antigen is recognized by an alveolar macrophage, and this
results in activation of a T lymphocyte and the production of interleukin-2. The
activated macrophage may also release various products that stimulate fibro-
blasts, thus explaining the deposition of fibrous tissue in the interstitium.
I
I'
I'
I
FIGURE Chest Radiograph ofaPatientwith Interstitial Pulmonary Fibro?is. Note
the small contracted lung and rib cage and:the raised diaphragms (compare the normal
appearance in Figure 4-SA).
92 PART II
Clinical Features
F ~ U : r stages of sarcoidosis can be identified. (30)
Stage 0: These patients have no obvious intrathoracic involvement,
although a CT scan may show enlarged mediastinal lymph nodes
(lymphadenopathy) .
Stage 1: There is bilateral hilar adenopathy often with right paratracheal
adenopathy. This is often accompanied by erythema nodosum of the legs.
Arthritis, uveitis, and parotic gland enlargement may also occur. There are
no disturbances of pulmonary function.
Stage 2: The pulmonary parenchyma is also involved, the most common
radiologic appearance being widespread mottled shadows, most significant
in the mid and upper zones. Symptoms include breathlessness and a dry,
4nproductive cough.
Stage 3: Here there are pulmonary infiltrates without adenopathy. Fibrosis is
seen predominantly in the upper lobes, a!ld cavities or bullae may be present.
Pulmonary Function
There is no impairment of function in stages 0 and 1 of the disease. In stages
2 and 3, typical changes of the restrictive type are seen, although the radiographic
appeara,nce sometimes suggests more interference with function than actually exists.
Ultimately, significant pulmonary fibrosis may develop, with a severe restric-
tive pattern of function. All Jung volumes are small, but the FEY/ PVC % is
preserved. Lung compliance is strikingly reduced, the pressure-volume curve
being flattened and shifted downward and to the right (see Figure 3-1). The rest-
ing arterial POI is low and often falls considerably on exercise. The arterial P
COI
is normal or low, although, terminally, it may rise as respiratory failure super-
venes. The diffusing capacity for carbon monoxide (transfer factor) is reduced
significantly. Cor pulmonale may develop in advanced disease.
Hypersensitivity Pneumonitis
This is also known as extrinsic allergic alveolitis. It is a hypersensitivity reaction
affecting the lung pa:r;enchyma that occurs in response to inhaled organic dusts.
A good example is farmer's lung. The exposure is usually occupational and heavy.
The disease is an example of type 3 hypersensitivity (or combination of types
3 and 4), and precipitins can be demonstrated in the serum.
The term "extrinsic" implies that the etiologic agent is external and can be
identified, in contrast to "intrinsic" fibrosing alveoli tis (diffuse interstitial fibrosis
discussed above), where the cause is unknown. Farmer's lung is due to the spores
of thermophilic Actinomyces in moldy hay. Bird breeder's lung is caused by avian
antigens from feathers and excreta. Air conditioner's lung and bagassosis (in sugar
cane workers) are also recognized ..
Pathology
The alveolar walls are thickened and infiltrated with lymphocytes, plasma cells,
and occasional eosinophils together with collections of histiocytes that, in some
areas, form sma.U granulomas.. The small bronchioles are usually affected and
there may be exudate in the lumen. Fibrotic changes occur in advanced cases.
Clinical Features
The disease occurs in either
dyspnea, fever, shivering, am
for 24-48 hours. The patienl
throughout both lung fields. '
prior acute attacks. These pa
a period of years. In the ac
frequently a military nodula
the upper lobes is common.
Pulmonary Function
In well-developed disease, t
reduced lung volumes, low
normal or low arterial Peol'
variable degrees of airway 01
Interstitial Disease Caw
Various drugs may cause al
interstitial fibrosis. These
chronic myeloid leukemia)
rhythmic agent amiodarone
plastic drugs can also cause
toxic changes with subsequ
the weedkiller paraquat r e ~
fibrosis. Therapeutic radiat
lung is included in the field
Collagen Diseases
Interstitial fibrosis wi th a 1
with systemic sclerosis (gel
out of proportion to the (
Other connective tissue disc
erythematosus and rheumat
Lymphangitis Carcinom
This re(ers to the spread of C(
complicate carcinomas, chie
the typical restrictive patte
DISEASES OF TI
Pneumothorax
Air can enter the pleural s
the chest wall as the re;
intrapleural space is norm
forces of the lung and che:
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iiiiiiiiiiiiiii

.IS intrathoracic involvement,
ed mediastinal lymph nodes
'{ often with right paratracheal
erythema nodosum of the legs.
ment may also occur. There are
o involved, the most common
ttIed shadows, most significant
:lude breathlessness and a dry,
without adenopathy. Fibrosis is
lVities or bullae may be present.
lOd 1 of the disease. In stages
;een, although the radiographic
th function than actually exists.
develop, with a severe restric-
nall, but the FEV/ FVC % is
:1, the pressure-volume curve
ight (see Figure 3-0. The rest-
on exercise. The arterial Peoz
;e as respiratory failure super-
Je (transfer factor) is reduced
Iced disease.
t is a hypersensitivity reaction
Jnse to inhaled organic dusts.
;ually occupational and heavy.
'ity (or combination of types
he serum.
agent is external and can be
itis (diffuse interstitial fibrosis
ner's lung is due to the spores
:eder's lung is caused by avian
s lung and bagassosis (in sugar
th lymphocytes, plasma cells,
IS ofhistiocytes that, in some
)les are usually affected and
es occur in advanced cases.
"'P:!APTER 5 Restrictive Diseases 93
Clinical Features
The disease occurs in either acute or chronic forms. In the former, symptoms of
dyspnea, fever, shivering, and cough appear 4-6 hours after exposure and continue
for 24-48 hours. The patient is frequently dyspneic at rest, with fine crepitations
throughout both lung fields. The disease may also occur in a chronic form without
illi
i
prior acute attacks. These patients present with progressive dyspnea, usually over
a period of years. In the acute form, the chest radiograph may be normal, but
frequently a military nodular infiltrate is present. In the chronic form, fibrosis of
the upper lobes is common.
Pulmonary Fundion
In well-developed disease, the typical restrictive pattern is seen. This includes
reduced lung volumes, low compliance, hypoxemia that worsens on exercise,
normal or low arterial P
eoz
, and a reduced diffusing capacity. In the early stages,
variable degrees of airway obstruction may be present.
Interstitial Disease Caused by Drugs, Poisons, and Radiation
Various drugs may cause an acute pulmonary reaction, which can proceed to
interstitial fibrosis. These drugs include busulfan (used in the treatment of
chronic myeloid leukemia), the antibiotic nitrofurantoin, the cardiac antiar-
rhythmic agent amiodarone, and the cytostatic drug bleomycin. Other antineo-
plastic drugs can also cause fibrosis. Oxygen in high concentrations causes acute
toxic changes with subsequent interstitial fibrosis (see Figure 5-3). Ingestion of
the weedkiller paraquat results in the rapid development of lethal interstitial
fibrosis. Therapeutic radiation causes acute pneumonitis followed by fibrosis if
lung is included in the field.
Collagen Diseases
Interstitial fibrosis with a typical restrictive pattern may be found in patients
with systemic sclerosis (generalized scleroderma). Dyspnea is often severe and
out of proportion to the changes in radiologic appearance or lung function.
Other connective r.issue diseases that may produce fibrosis include systemic lupus
erythematosus and rheumatoid arthritis.
Lymphangitis Carclnomatosa
This refers to the spread of carcinoma tissue through pulmonary lymphatics and may
complicate carcinomas, chiefly of the stomach or breast. Dyspnea is prominent, and
the typical restrictive pattern of lung function may be seen.
DISEASES Of THn: PLEURA
Pneumothorax
Air can enter the pleural space either from the lung or, less commonly, through
the chest wall as the result of a penetrating wound. The pressure in the
intrapleural space is normally subatmospheric as a result of the elastic recoil
forces of the lung and chest walL When air enters the space, the lung collapses
--
and the rib cage springs out. (See Respiratory Physiology: The Essentials, 7
th
ed.,
p. 104.) These changes are evidenton a chestradiograph (Figure 5-8), which
showspartialorcompletecollapseofthelung,overexpansionoftheribcage and
depressionofthediaphragmontheaffectedside, andsometimesdisplacementof
mediastinumawayfrom thepneumothorax.Thesechangesare mostevident
if the pneumothorax is large, particularly ifa tension pneumothorax is present
(see text).
Spontaneous Pneumothorax
Thismostcommonformofpneumothoraxiscausedby theruptureofasmallbleb
on the surface ofthe lung nearthe apex. Ittypically occurs intall young males
and may berelated to the highmechanical stresses thatoccur inthe upperzone
oftheuprightlung(seeFigure3-5).Thepresentingsymptomis oftensuddenpain
ononesideaccompaniedbydyspnea.Onauscultation, breathsoundsarereduced
ontheaffectedside andthediagnosis is readilyconfirmedby aradiograph.
Thepneumothoraxgraduallyabsorbsbecause thesumofthepartialpressures in
thevenous bloodis considerably less thanatmosphericpressure. Recurrentattacks
mayneedsurgicaltreatmenttopromoteadhesionsbetweenthetwopleuralsurfaces.
FIGURE 5-8 ChestRadiograph ShowingaLarge Right-Sided SpontaneousPneumothorax.
Notethe small, collapsed rightlung, depressionofthe right hemidiaphragm,and overexpansion
ofthe rib cage on the right.
Spontaneous PneumothoraJ
Typically occursinyoung pE
Accompanied by dyspnea, r
Graduallyabsorbed bythe t
Recurrent attacks may requi
Tension pneumothoraxis a
Tension Pneumothorax
In a small proportion of s
between thelungand the pI
quence,airentersthespace
tion.Theresultis alargepm
exceedatmosphericpressure
Thismedicalemergencyi
cardia,andsignsofmediastir
theapexbeat.Theradiograr
ingthepressurebyinserting:
toanunderwaterseal thatal
Pneumothorax Complier.
Thisoccursinavarietyofc(
cystinadvancedfibroticdise
titationwithhighairwaypre
Pulmonary Function
Aswouldbeexpected,apnt
pulmonaryfunctiontestsare
radiograph is so informative
Pleural Effusion
This refers tofluid rather d
own right, but it frequent!
shouldalways be sought.
The patientoften report
pleUriticpainfromtheunde
and include reduced movel
breathsounds, anddullness
Pleural effusions can be
whethertheirproteinconter
(LDH) concentrationtends
with malignancies and infe
failure andotheredematous
treatment should be direct
impairedas inpneumothora
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siology: The Essentials, 7
th
ed.,
diograph (Figure 5-8), which
rexpansion of the rib cage and
nd sometimes displacement of
lese changes are most evident
;ion pneumothorax is present
Iby the rupture of a small bleb
lly occurs in tall young males
; that occur in the upper zone
symptom is often sudden pain
on, breath sounds are reduced
lfirmed by a radiograph.
sum of the partial pressures in
ric pressure. Recurrent attacks
tween the two pleural surfaces.
led SpontaneousPneumothorax.
omidiaphragm, andoverexpansion
iI ,C8APTER5 Restrictive Diseases 95
Spontaneous Pneumothorax
Typically occurs in youngpeople in their20s
Accompanied bydyspnea, pain
Grad_ually absorbedby the blood
Recurrent attacks may require surgery
Tension pneumothorax is amedical emerg.ency.",
".
Tension Pneumothorax
In a small proportion of spontaneous pneumothoraces, the communication
between the lung and the pleural space functions as a check valve. As a conse-
quence, air enters the space during inspiration but cannot escape during expira-
tion. The result is a large pneumothorax in which the pressure may considerably
exceed atmospheric pressure and thus interfere with venous return tothe thorax.
This medical emergency is recognized by increasing respiratory distress, tachy-
cardia, and signs of mediastinal shift, such as tracheal deviation and movement of
the apex beat. The radiograph is usually diagnostic. Treatment consists of reliev-
ing the pressure by inserting a tube through the chest walL This tube is connected
to an underwater seal that allows air to escape from the chest but not toenter it.
Pneumothorax Complicating Lung Disease
This occurs in a variety of conditions, including rupture of a bulla in COPD or a
cyst in advanced fibrotic disease. Italso sometimes occurs during mechanical ven-
tilation high airway pressures (see Chapter 10, "Mechanical Ventilation").
Pulmonary Function
As would be expected, a pneumothorax reduces the FEV! and FVC. In practice,
pulmonary function tests are rarely helpful in treating these patients because the
radiograph is so infortnative.
Pleural Effusion
This refers to fluid rather than air in the pleural space. It is not a disease iri its
own right, but it frequently accompanies serious disease and an explanation
should always be sought.
The patient often reports dyspnea if the effusion is large and there may be
pleuritic pain from the disease. The chest signs are often informative
and reduced movement of the chest on the affected side, absence of
breath sounds, and dullness to percussion. The radiograph is diagnostic.
Pleural effusions can be divided into exudates and ttansudates according to
whether their protein content is high or low. In addition, the lactic dehydrogenase
(LDH) concentration tends tobe higher in transudates. Exudates typically occur
with malignancies and infections, whereas transudates complicate severe heart
failure and other edematous states. Itis often necessary to aspirate an effusion, but
treatment should be directed at the underlying cause. Pulmonary function is
impaired as in pneumothorax, but the measurements are not required in practice.
i
I
.
96 PART II Function oftheDiseasedlung __',
Variantsofpleuraleffusion include empyema (pyothorax), hemothorax, and
chylothorax, whichrefer tothepresenceofpus, blood, and lymph, respectively,
inthepleuralspace,
PleuralThickening
Occasionally, a long-standing pleural effusion results in a rigid, contracted
fibrotic pleura thatsplints the lung and prevents its expansion. This can result
inasevererestrictive typeoffunctional impairment,particularlyifthedisease is
bilateral.Surgicalstrippingmaybenecessary.
DISEASES OF TtgE CHEST WALL
Scoliosis
Bonydeformityofthe cancauserestrictivedisease.Scoliosisreferstolateral
curvatureofthespineandkyphosis toposteriorcurvature.Scoliosisis moreserious,
especially ifthe angulation is high inthe vertebralcolumn. Itis frequently asso-
ciated with a backward protuberance of the ribs, giving the appearance ofan
added kyphosis. Inmost cases, the cause is unknown, although the condition is
occasionally caused by bonytuberculosisorneuromusculardisease.
Thepatientinitiallyreportsdyspneaonexertion; breathingtendstobe rapid
andshallow. Hypoxemialaterdevelops,andeventuallycarbondioxideretention
and corpulmonalemaysupervene. Bronchitisis commonifthepatientsmokes.
Pulmonaryfunctionteststypicallyshowareductioninalllungvolumes. Airway
resistance is nearlynonnalifrelated tolungvolume. However, thereis inequality
ofventilation, partly ofairway closure in regions. Partsofthe
lungarecompressed andthereareoftenareasofatelectasis.
The hypoxemia is caused by ventilation-perfusion inequality. In advanced
disease, a reduced ventilatoryresponse toCO
2
can bedemonstrated. This
reduction reflects the increased work of breathing caused by deformity ofthe
chestwall.Notonlyis thechestwall stiff, butalso therespiratorymusclesoperate
inefficiently. pulmonary vascular bed is restricted, causing a rise in pul-
monary artery pressure, which is exaggerated by the alveolar hypoxia, Venous
congestionand peripheraledema may develop. patientmay succumb toan
intercurrentpulmonary infectionorrespiratoryfailure.
AnkylosingSpondylitis
Inthisdisease unknownetiology,thereis agradualbutrelentlessonsetofimmo-
bilityofthevertebraljointsandfixation oftheribs. Asaresult, themovementof
the chest wall is grossly reduced. There is a reduction ofFVC andTLC, but the
FEV/FYC percent and the airway resistance are normal. The compliance ofthe
chestwall mayfall andthereis oftensome unevenventilation,probablysecondary
tothereducedlungvolume.Thelungitselfremainsnonnalinnearlyallcases, and
diaphragmaticmovementis preserved. Respiratoryfailure does notoccur.

Diseases affecting the musc
poliomyelitis,Guillain-Barre
gravis,and musculardystroph
canlead todyspneaand
adeepbreathis reflectedina
Itshouldbe rememberedt
diaphragm, and wit!
until the diaphragm is inv(
severelycompromised.Thepl
theFVCandthebloodgases."
the patient can develop are;
"MechanicalVentilation") IT
KEY CONCEPTS
1. Diffuse interstitialpulm
characterized by dyspr
reduced lungcomplian
2. Thealveolarwallsshm
ofcapillaries.
1. Airwayresistanceis no
abnormallyhighflow r
airway.
t 4. Diffusion ofoxygenac
f
eningand mayresultir

tilation perfusion inei
exchange.
5. Otherrestrictive disor
wall, orneuromuscular
QUESTIONS
1. Thetype II alveolarer
A. Provides mostofth
B. Cannotmultiply.
C. Is formed whenat'
D. Secretessurfactant.
E. Is metabolically im
2. Histologicchanges in
include:
A. Infiltrationofthec
B. Breakdownofman
C. Mucousglandhyp<
D. Mucousplugging0
E. Increased volume (
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yothorax), hemothorax, and
lod, and lymph, respectively,
suIts in a rigid, contracted
sexpansion. This can result
:, particularlyifthedisease is
:ase. Scoliosisrefers tolateral
ture. Scoliosis is moreserious,
:olumn. Itis frequently asso-
the appearance ofan
n, although thecondition is
Isculardisease.
;breathingtends to be rapid
Jlycarbondioxideretention
amon ifthepatientsmokes.
:1 inalllungvolumes.Airway
However, thereis inequality
Jendent regions. Partsofthe
:ctasis.
on inequality. In advanced
)ftenbedemonstrated. This
caused by deformity of the
respiratory musclesoperate
:ted, causing a rise in pul-
e alveolar hypoxia. Venous
patientmaysuccumb to an
reo
Jutrelentlessonsetofimmo-
\.s aresult, themovementof
1 ofFVC and TLC, but the
nal. The compliance ofthe
ltilation, probablysecondary
)fmalinnearlyallcases, and
luredoes notoccur.
CHAPTER 5 Restrictive Diseases 97
NEUROMUS(UlAR DISORDERS
Diseases affecting the muscles of respiration or their nerve supply include
poliomyelitis,Guillain-Barresyndrome, amyotrophiclateralsclerosis, myasthenia
gravis,andmusculardystrophies (seeTable2-1 andFigure 2-3). Allthesediseases
canlead todyspneaandrespiratoryfailure. Theinabilityofthepatienttotake in
adeepbreathis reflectedinareducedFVC,TLC, inspiratorycapacity, andFEV
j
.
Itshould be remembered thatthemost importantmuscleofrespirationis the
diaphragm, and patients with progressive disease often do not report dyspnea
until the diaphragm is involved. By then their ventilatory reserve may be
severelycompromised. Theprogressofthediseasecanbe monitoredby measuring
theFVCandthebloodgases.Themaximalinspiratoryandexpiratorypressuresthat
the patient can develop are also reduced. Assisted ventilation (see Chapter 10,
"MechanicalVentilation") may becomenecessary.
KEY CONCEPTS
1. Diffuse interstitialpulmonaryfibrosis is anexampleofrestrictivelungdisease
characterized by dyspnea, reduced exercise tolerance, small lungs, and
reduced lungcompliance.
2. Thealveolarwallsshowmarked infiltrationwithcollagenanddestruction
ofcapillaries.
3. Airwayresistance is notincreased; indeedaforced expirationcanresult in
abnormallyhighflow rates becauseofthe increased radial tractiononthe
airway.
4. Diffusion ofoxygen across the blood-gas barrier is impeded by the thick-
eningand may result inhypoxemia,especiallyonexercise. However, ven-
tilation perfusion inequality is the major factor in the impaired gas
exchange.
5. Other restrictive disorders are caused by diseases of the pleura or chest
wall, orneuromusculardisease.
QUESTIONS ___
1. Thetype II alveolarepithelial cell:
A. Providesmostofthestructuralsupportfor thenormalalveolarwall.
B. Cannotmultiply.
C. Is formed whena type Iepithelialcelldivides.
D. Secretessurfactant.
E. Is metabolicallyinactive.
2. Histologic changesindiffuse interstitialpulmonaryfibrosis typically
include:
A. Infiltrationofthealveolarwallwith lymphocytes and plasmacells.
B. Breakdownofmanyalveolarwalls.
C. Mucous glandhypertrophy inthebronchi.
D. Mucouspluggingofairways.
E. Increasedvolumeofthepulmonarycapillarybed.
I
98 PART II Functipn of
3. Featuresofdiffuse interstitialpulmonaryfibrosis include:
A. Coughproductiveofcopioussputum.
B. Hemoptysis.
C. Rhonchiinbothlungs.
D. Dyspneaespeciallyonexercise.
E. Depresseddiaphragmsontheradiograph.
4. Pulmonaryfunctiontests indiffuse interstitialpulmonaryfibrosis typically
.
A. IncreasedFEY1"
B. IncreasedFYC.
C. IncreasedFEY\/FYC %.
D. IncreasedTLC.
E. Increasedairwayresistancewhenrelatedto lung volume.
5. Thearterialhypoxemiaofapatientwithdiffuse interstitialpulmonary
fibrosis:
A. Typically worsens onexercise.
B. Is chieflycaused by diffusion impairment.
C. Is associatedwitha large increaseindiffusingcapacityduringexercise.
D. Is usually associatedwithcarbondioxide retention.
E. Is improvedduringexercise becauseoftheabrormallylarge increase
incardiacoutput.
6. Ina patientwithdiffuse interstitialfibrosis ofthelung, themaximal
expiratoryflow rateatagivenlung volume may behigherthanina
normalsubjectbecause:
A. Expiratorymuscles havea large mechanicaladvantage.
B. Airwayshavea smalldiameter.
C. Dynamiccompressionoftheairways is more likely thanina normal
subject.
D. Radialtractionontheairways is increased.
E. Airwayresistance is increased.
7. Thediffusingcapacityfor carbonmonoxide ina patientwithdiffuse
interstitiallungdisease:
A. Is typicallysubstantially increased.
B. Showsanabnormallylarge increaseduringexercise.
C. Is unaffectedby thickeningoftheblood-gas barrier.
D. Is reduced inpartbecauseofobliterationofpulmonarycapillaries.
E. Falls onlylate inthedisease.
8. Featuresofpneumothoraxinclude:
A. Itreduces thevolumeofthechestwallontheaffectedside.
B. Itcauses anincreasedbloodflow intheaffected lung.
C. Whenpresentinthetensionform itis a medicalemergency.
D. Spontaneouspneumothorax is mainlyseeninolderwomen.
E. TheFYCis increased.
PulmonaryEdema
Pathophysiology
Pathogenesis
Increased Capillary Hydl
Pressure
Increased Capillary Pem
Reduced Lymph Drainaf
Decreased Interstitial Pn
Decreased Colloid Osmol
Uncertain Etiology
,Clinical.Features
I pulmoparyFunction
I'\
Exchange
of Ventilation
",;,
u/monaryCirculatlOn
",{') (
The pathophysiology
edema is not a dis east
diseases and can he Ii.
diagnosed and may hI
is poorly understood (
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