BOOP: what is old, what ... J .

EDITORIAL
Eur Reaplr J
1991 , 4, 771 - 773
BOOP: what is old, what is new?
U. Costabel, J. Guzman
Old is the disease, and old is the description of the
pathological lesion. New is the term bronchiolitis obliterans organizing pneumonia (BOOP), and new is the
recognition of the characteristic clinical and radiological
findings associated with the histological lesion, i.e. the
recognition of a clinicopathological entity. How can this
entity be defined?
Firstly, it can be idiopathic or can be produced by a
variety of immunological, toxic, and inflammatory
processes. Idiopathic BOOP [1, 2] or cryptogenic organizing pneumonia (COP) [3] can be defined as a clinicopathological entity of unknown origin characterized by:
I) the cJjnical presentation with a preceding flu-like illness and a short history of progressive dyspnoea associated with; 2) patchy infiltrates on chest radiogram and/
or computerized tomographic (CT) scan; . and 3) the
pathohistological pattern of intraluminal organization
predominantly within the alveolar ducts.
BOOP is a disease with a restrictive ventilatory defect
involving the lung parenchyma. Therefore, the disease
is best classified as part of the spectrum of infiltrative or
interstitial lung diseases [2, 4]. It is not a predominant
or pure disorder of the small airways, as the first two
words of the name BOOP may suggest. What is now
recognized as BOOP or COP is, according to EPLER and
CoLBY [1], the same reaction that was labelled bronchiolitis interstitial pneumonia (BIP) by L IEBOW and
CARRINGTON [5] in their classification of the chronic interstitial pneumonias.
The pure bronchiolitis obliterans (without organization)
is a totally different disease, both clinically and pathologically. It is a true disorder of the small airways with
stenotic, scarred, constrictive bronchiolitis (without intraluminal plugs or polyps) leading to airflow obstruction;
the chest radiogram is normal or shows hyperinflation
and occasionally small nodules (6-9]. It appears to be a
much rarer disease than BOOP. The majority of the 52
cases in the first extensive roentgenologic-pathologic
study of bronchiolitis obliterans, published by GosJNK et
al. in 1973 [6], would be classified as BOOP today.
The German pathologist LANoB [10] was fi rst to
describe the pathological lesion of BOOP as early as
1901, in two cases. What he observed and termed
"Bronchitis et Bronchiolitis obliterans" was not the small
airways disease of scarred constrictive bronchiolitis with
Abt. Pneumologie/Allergologie, Ruhrlandklinlk. D-4300 Essen-16,
Germany.
airflow obstruction; it was exactly the lesion now called
BOOP or cryptogenic organizing pneumonia. Clinically,
his two cases presented with a history of fever, cough
and increasing dyspnoea of eight days and six months
duration, respectively. On auscultation, crackles were
heard in both patients. Both died on the second day in
hospital. The postmortem findings were described by
Lango i.n detail, and he found organizing exudates with
plugs of granulation and young connective tissue that
were located within small bronchi, bronchioli, and alveoli. He already recognized that the plugs always extend from the walls of bronchioles into the alveolar lumen,
and never grow from the alveolar wall itself.
In 1983, DAVISON et al. [3] in London, recognized the
association of the distinct clinical features with the
pathological lesion in this condition. In their report on
eight cases of unknown aetiology, they suggested the
term "cryptogenic organizing pneumonitis" in order to
avoid confusion with postinfective organizing pneumonia. They also reported the dramatic response to prednisolone and the frequent relapse when the dose was
reduced too quickly.
In 1985, EPLE.R et al. [2] extended Davison's observations in their report on 50 idiopathic cases with this
clinical syndrome. This article from Boston popularized
the disorder and gave important information such as the
typical clinical presentation, the typical patchy infiltrates
on chest radjographs, and the fact that long-term
treatment (3- 12 months) with steroids is necessary
to prevent relaps e of the disease. Alrea dy in
1983, EPLBR and CoLBY [1 ] were first to use the
term "BOOP" in an Editorial on the spectrum of
bronchiolitis obliterans with a proposal for a clinical
classification of this disease.
Since 1985, there were first preliminary reports on the
bronchoalveolar lavage (BAL) cell types and the
constant decrease in the CD4/CD8 ratio of BAL lymphocytes [11, 12] as well as on er findings [13, 14]. It was
only in 1989, that first observations appeared on the
peripheral location of the patchy infiltrates in BOOP
[15, 16]. It is our experience that the er scan is
more sensitive in recognizing the peripheral character of
the patchy infiltrates than the conventional chest radiogram, and that they are frequently shaped like triangles
which appears to be the characteristic er feature ofBOOP
infiltrates [17).
To the best of our knowledge, there have so far
been 41 cases of idiopathic BOOP reported in Europe
m
U. COSTABEL, 1. GUZMAN
[17-21 ], including the three cases described in this issue
of the Journal by Au!oRE-MARTIN et al. [21]. One of the
latter cases showed cavitation which is a rare finding in
BOOP. The authors concluded that transbronchial biopsy
(TBB) is not useful for diagnosis. Our own experience is
different, since we were able to get appropriate tissue for
diagnosis by fluoroscopy guided TBB in two of seven
patients where this procedure was applied [17].
The clinicopathological entity of BOOP has been observed in the context of collagen-vascular diseases [1, 2,
22, 23) and other auto-immune diseases like chronic
thyroiditis [24]; secondary to treatment with drugs like
gold [25-31], cephalosporin [32-34], amiodarone [17,
35], and other drugs [35-38]; following the inhalation of
cocaine [39]; after cytomegalovirus pneumonia in allogeneic bone marrow transplantation [40]; and associated
with human immunodeficiency virus (HIV)-infection [41].
Furthermore, histological lesions with a BOOP pattern
(not necessarily combined with the distinct clinical and
radiographic presentation of the BOOP entity) are nonspecific and may be found in influenza and other organizing infections [1, 6, 40, 42], hypersensitivity
pneumonitis [43, 44], chronic eosinophilic pneumonia
[15, 43, 45, 46], irradiation pneumonitis [47], organizing diffuse alveolar damage, distal to bronchial
obstruction, associated with chronic aspiration, lung
abscesses, and Wegener's disease [48].
There is also a localized form of the histologic BOOP
pattern that may best be termed "focal organizing pneumonia". Usually, such solitary lesions are incidental
findings in asymptomatic patients where biopsy or resection was performed for suspected carcinoma [1). Thus,
idiopathic BOOP or COP can only be diagnosed after
exclusion of BOOP secondary to an underlying disorder
and after ruling out conditions or entities that show
histological lesions with a BOOP pattern.
We believe that there is no way back to one name for
this clinicopathological entity. We have to live with
BOOP or COP as we have to live with different names
for another entity, namely idiopathic pulmonary fibrosis
(IPF) also called usual interstitial pneumonia (UIP)
(Liebow); cryptogenic fibrosing alveolitis (CFA) (TumerWarwick); diffuse fibrosing alveolitis (DFA) (Scadding);
the list of synonyms for IPF is not exhaustive! What we
have to recognize, however, is the clear distinction between BOOP or COP as an interstitial or infiltrative lung
disease and pure obliterative bronchiolitis (BO) as a
disease of the small airways with airflow obstruction.
The differences are clearly presented by ou Bms and
GEODES [49) in their Editorial in this issue of the Journal.
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BOOP: quoi de neuf, quoi de vieux? U. Costabel, J. Guzman.
REsUME: Un anatomo-pathologiste allemand, W. Lange, a
d6critla L6slon anatomo-pathologique de cette affection en 1901.
Quoi qu'il ail d6nomm6 scs deux cas "bronchite et bronchlolite
oblit6rante'', il ne s'agissait pas de la maladie des petites voies
a6riennes avec une bronchiolite constrictive cicatricelle
accompagn6e d'obstruction du courant a6rien. Jl s'agissait
exactement des 16sions appe16es aujourd'hui BOOP ou encore
pneumonic cryptog6nique scl6rosante. Ses deux cas se
caract~risaient par de la mvre, de la toux, une dyspn6e
croissante, et des cr~pitements dans les deux champs
pulmonaires. Les deux patients souffraient d'une maJadie
rapidement progressive. Les examens autopsiques ont monlr~
des exsudats en voie d'organisalion, avec des bouchons de
granulations et du tissu conjonctif jeune situ6 dans les petitcs
brooches, les bronchioles et les alv~oles. En 1983, Davison et
collaborateurs ont reconnu l'association de signes cliniques
caract6ristiques avec la 16sion pathologique de cette maladie.
En 1985, Epler et coUaborateurs ont ~tendu les observations de
Davison dans leur relation de 50 cas idiopathiques.
Le C.T. scan est plus sensible pour reconnattre le caract~re
ptriph~rique des infiltrats irr~guliers que ne l'est le clich6
thoracique conventlonnel. Les 42 cas pubU~s en Europe sont
ensuite pass6s en revue.
Eur Respir J., 1991, 4, 771-773.