Pulmonary Wegener's Granulomatosis*

was the increase in polymorphonuclear leukocytes at
the time of the differential cell count, but this is not a
feature peculiar to WG, and its diagnostic value is
therefore limited.
Pleural involvement in WG rarely results in much
effusion, and no published information on pleural fluid
is available.’ In our four cases with pleural fluid
analysis, there was an exudate with a predominance
(of polymorphonuclear leukocytes.
Lung functioon tests have been used in the staging
and the follow up o)f patients with pulmonary WG,
and the most common abnormality was airflow (ob-
struction.#{176} Detailed lung function tests were rarely
available in the present study, thus allowing rio com-
ment (on this point.
The alveolar hemorrhagic syndrome has become a
more frequently recognized pulmonary manifestation
ofWC,’7”5’21 and it occurred in 8 percent of patients
in this series. Patients present with hemoptysis, and
blood originating from distal airways is seen at bron-
choscopy. The chest x-ray film shows diffuse bilateral
alveolar infiltrates. Alveolar hemorrhage is generally
acute and even fulminant in soome cases. Death fre-
quently occurs in untreated patients or if treatment is
delayed.’8 On the oother hand, resolution is generally
complete with early treatment and may sometimes
occur spontaneously.2#{176} Alveolar hemorrhagic WG may
be confused with Gooodpasture syndrome especially
since the latter also is usually assoociated with renal
disease. However, patients with WG have in addition
extrapulmonary and extrarenal disease. They also have
antineutrophil antibodies, whereas they lack the anti-
basement membrane antibodies found in Gooodpas-
ture’s syndrome. The pathologic diagnosis of WC at
the time of the lung biopsy often is difficult when
typical lesions are absent and capillaritis is overshad-
owed by alveolar 0708
We fcound no striking differences between the pill-
monary manifestations of “limited” WG and those oof
“classic” WC (ie, with renal invoolvement). The cooncept
O)f tile limited form o)f WG has been proposed princi-
pally to differentiate patients with and without renal
invoolvemellt, the former having the poorer proogno-
sis.#{176}27Iii this series, only OOC oof the 14 patients whoo
died with active disease had “limited” WC.
Usual laboratory tests are oof little help in the
diagnosis
matoorytation
of WG,
syndrome
rate , anenlia,
since
witil
they only point
a raised erythroocyte
hyperleukoocytosis
to) an inflamsedimen-
and hyperthroombocytosis.)6
Circulating immune co)mplexes
were Present iii at least 16 percent oofour patients and
at least 19 percent of Fauci’s,#{176} and they (occasionally
have been reported by o)thers. The roole of immune
complexes in WG is uncertain but may he similar to
that in other vasculitic syndromes. Several recent
studies have shown that anti-neutroophil antibodies are
Downloaded From: http://hwmaint.chestpubs.org/ on 08/11/2013
present in the serum oof patients with active WG and
could be invoolved in the pathogenesis of the dis-
ease.’#{176} In the present series, antineutro)phil antihod-
ies were present in ten out ofthe 12 patients tested.
We shall not discuss the clinical manifestations
outside the respiratory tract o)ther than to make the
point that the multi-organ involvement can guide the
diagnosis of the pulmonary disease. The prognoosis of
WG has been transformed by therapy with corticooste-
rooids and cycloophosphamide.3 The case fatality rate of
7 percent reported by Fauci3 was especially low hut
related to) patients enrolled in a treatment protocoi at
a referral center, thus excluding patients dying before
or just after diagnosis in other institutions. In the
present and two other series,6’ the case fatality rate
ranged from 20.8 to) 28 percent. In the present series,
seven of 77 (9 percent) patients died without treat-
ment. The fact that three patients died shortly after a
diagncstic thoracotomy underlines the risk (of this
procedure in patients with severe pulmonary WC. On
the other hand, treated patients generally improve
without significant sequelae, although the hazards of
immunoosuppressive drugs are a major limiting factor
foir long-term survivai.’ Cotrimoxazole has been
advocated as an alternative treatment, especially when
WG is limited to the lung.’#{176}”#{176}’#{176}
Our study shows that patients die from WG mainly
as a result of delays in diagnoosis. It is therefore
necessary to recognize the protean clinical and imag-
ing presentation cof WG so that the approopriate diag-
noostic investigations are done and the patient is treated
earl)
ACKNOWLEDGMENT: Ve thank L. D. Cnoer for reviewing the
translation o)f this paper and NI. C. Thevenet four secretarial
assistamice.
APPENDIX
The foollowing French clinicians participated in the Clinicoopathoulogic
Research
contributing one
Crotop’s
our moore
study
patients:
on Wegener’s
C. Akotmn,
granuloumnatosis
Paris; J. P Bernard,
b)V
Lvun; J. Bignon, Cr#{233}teil; F. Blanc-Jouvan, Grenoble; F. Bo)nns010d,
Limoges;
Caries,
P A. Boudes, Paris;
Tooulouuse; J. Cerrina,
J. Bran,
Paris; J. C.
Rouemm;
Dalphin,
P Camus, Dijon;
Besan#{231}’omi; M .
P
Dc
Lajartre,
A. Dhers,
Nantes;
Macon;
P. Delaval,
P 1)ugioe,
Rennes;
Crasse;
P Deteix, Clermont-Ferramod;
J. NI. Durand, Marseille; A.
Emnoomiot, Saint-Etienne; P Godard, Moontpo’llier; J. C. Cto#{233}rimi,
Lyon; A. llaloumomi,
Kerhrouimc’h, Brest;
Nantes;
A.
B. Ilerer,
Krivitzk;
Paris;
Paris:
P llyvernat,
J. Lacromiiojmme,
Lyon;
Paris:
J. F.
0.
L5UO(110e, TO)(Ol000Se; NI. Laville, Lyon; F. X. Lehas, Le Mans; NI. C.
Level, Verolumi; C. Mayaud, Paris; B. Milleron, Paris; F. Natali,
Paris; C. Nootovet, Rouuemo; R. Panente, Paris; F. Patte, Poitiers; J. N.
Prevost. Rooiocn; 0. Rigatid, Crenooble: D. Roohert, Lyon; E. Rowgel.
Straslouomrg; B. Saoovezie, Clermoont-Ferrand; C . Tenipelhouff,
Rooanmme; J. M. V#{235}rgnoomm, Saint-Etiemone.
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sificatioun. Mayoo Clin Proc 1975; 51:777-8 1
3 Fauci AS, Haynes BF, Katz P, Wolff SM. Vegener granioloomso-
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