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Histologic spectrum of idiopathic interstitial pneumonias.Proc
Am Thorac Soc. 2006 Jun;3(4):322-9
Histopathologic
classification plays a key role in separating multiple forms of
idiopathic interstitial pneumonia into clinically meaningful
categories with important differences in natural history, prognosis,
and treatment. Microscopic criteria in diagnosis of these entities
include the pattern and microanatomic distribution of inflammation,
fibroblast proliferation, collagen deposition, and architectural
remodeling. Usual interstitial pneumonia (UIP) defines idiopathic
pulmonary fibrosis and is the most common of the idiopathic
interstitial pneumonias. UIP has distinctive morphologic features that
allow precise diagnosis in classical cases. Other forms of idiopathic
interstitial pneumonia include desquamative interstitial pneumonia,
respiratory bronchiolitis-associated interstitial lung disease, acute
interstitial pneumonia, and nonspecific interstitial pneumonia. These
latter categories differ from UIP in that the histopathologic findings
do not, by themselves, allow specific diagnosis in most cases and
require careful correlation with clinical and radiologic findings.
Desquamative
interstitial pneumonia and respiratory bronchiolitis-associated
interstitial lung disease.Chest.
2005 Jan;127(1):178-84.
BACKGROUND:
Desquamative interstitial pneumonia (DIP) and respiratory
bronchiolitis-associated interstitial lung disease (RB-ILD) are
uncommon forms of interstitial lung disease and have been incompletely
characterized. STUDY OBJECTIVES: To further characterize the clinical
features and course of subjects with DIP and RB-ILD. DESIGN:
Retrospective study. SETTING: Tertiary care, referral medical center.
PATIENTS: Twenty-three subjects with DIP and 12 subjects with RB-ILD
seen over a 12-year period between 1990 and 2001. INTERVENTIONS: None.
RESULTS: The study population included 19 men (54%) and 16 women
(46%). The mean (+/- SD) age at diagnosis was 46 +/- 10 and 43 +/- 7
years, respectively, for patients with DIP and RB-ILD. All subjects
were either current or previous smokers except for three subjects with
DIP. The diagnosis was confirmed in all cases by surgical lung biopsy.
Bronchoscopy with transbronchial lung biopsy had been performed in 12
patients and was nondiagnostic in all. The most common pulmonary
function abnormality was a reduced diffusing capacity of the lung for
carbon monoxide. A CT scan of the chest revealed ground-glass
opacities bilaterally in most patients who had DIP and RB-ILD. No
differences were observed between subjects with DIP and RB-ILD with
respect to clinical features, radiologic findings, or pulmonary
function test results. The clinical course was characterized by
relative stability in the majority of patients in both groups and a
partial response to corticosteroid therapy. Five deaths were observed,
including three resulting from progressive diffuse lung disease, all
in subjects with DIP. CONCLUSIONS: We concluded that DIP and RB-ILD
are chronic disease processes that in most patients are related to
smoking. Persistent abnormalities can be seen on pulmonary function
testing and radiologic studies despite smoking cessation and
corticosteroid therapy. Corticosteroid therapy appeared to be
associated with modest clinical benefit but usually not with
resolution of disease. Progressive disease with eventual death can
occur in subjects with DIP, especially with continued cigarette
smoking.
Desquamative
interstitial pneumonia, respiratory bronchiolitis and their
relationship to smoking. Histopathology.
2004 Sep;45(3):275-82.
AIMS:
Respiratory bronchiolitis (RB) and desquamative interstitial pneumonia
(DIP) are closely associated histological patterns of interstitial
pneumonia, although there are no studies on the extent of individual
histological parameters. Furthermore, the term smoking
related-interstitial lung disease (SR-ILD) has been proposed as a term
to encompass patients with both these histological patterns who give a
history of smoking, though it is not well defined how this term
relates to historical cases of DIP. The aim of this study was to
compare histological parameters in cases of DIP and RB and then to
review in detail clinical, imaging and histological data for DIP in
relation to a history of smoking. METHODS AND RESULTS: Forty-nine
cases were reviewed, 24 with RB and 25 with DIP; five cases of DIP
were re-classified as RB on review due to bronchocentricity of the
infiltrate. There was a significantly greater extent of interstitial
fibrosis (P = 0.02), lymphoid follicles (P < 0.001) and eosinophilic
infiltration (P < 0.0001) in patients with DIP compared with RB. In
addition, the extents of these three parameters were significantly
interrelated. Patients with DIP had a lower incidence of smoking (60%)
when compared with patients with RB-ILD (93%) (P < 0.005). Further
analysis of smokers versus never-smokers with DIP showed no difference
in histological parameters, extent of haemosiderin deposition or the
number of CD1a+ macrophages between the two groups, nor were there any
differences in clinical data to suggest other aetiologies. Follow-up
high-resolution computed tomography data from patients with DIP
suggested that a pattern of fibrotic non-specific interstitial
pneumonia (NSIP) may develop in the long term in both smokers and
never-smokers. CONCLUSION: There are significant differences in the
extent of interstitial fibrosis, lymphoid follicles and eosinophilic
infiltration between DIP and RB, as well as a much lower incidence of
smoking in patients with DIP. Whether the lower reported incidence of
smoking in DIP reflects referral bias or conservatism in giving a
history of smoking remains uncertain, as neither histological
parameters nor clinical data indicate a difference between smokers and
never-smokers with DIP. Nevertheless, some cases of DIP are likely to
remain idiopathic and unrelated to RB, though still have a good
prognosis. Furthermore, they may evolve into a pattern resembling
fibrotic NSIP. Therefore, whilst SR-ILD is appropriate in the correct
clinical setting, the distinction between the histological patterns of
RB and DIP remains appropriate.
Comparative
analysis of respiratory bronchiolitis-associated interstitial lung
disease and desquamative interstitial pneumonia.Zhonghua
Jie He He Hu Xi Za Zhi. 2004
Jun;27(6):373-7.
OBJECTIVE: To
investigate the clinicopathologic characteristics of respiratory
bronchiolitis-associated interstitial lung disease (RBILD) and its
relationship to desquamative interstitial pneumonia (DIP). METHODS:
The clinical and pathological data of one patient with RBILD confirmed
by video-assisted thoracoscopic lung biopsy were reviewed
retrospectively, and compared with one patient with DIP. RESULTS: Both
patients were 57 year old male, and they had a history of cigarette
smoking for 24 and 30 years respectively. The clinical manifestations
were cough and sputum production, breathlessness with exertion, and
crackles on chest examination. Lung function test showed a mild
abnormality with mixed obstructive-restrictive and restrictive pattern
respectively. The chest radiograph showed scattered small nodules and
patchy densities. High resolution computer tomography showed scattered
interstitial infiltrates and reticular changes in the middle and lower
lung fields, but patchy ground-glass attenuation was found only in
DIP. The pathological examination showed the presence of clusters of
pigmented macrophages within the lumens of respiratory bronchioles,
alveolar ducts and peribronchiolar alveolar spaces, with patchy
submucosal and peribronchiolar infiltration of lymphocytes and
histiocytes. Mild peribronchiolar fibrosis was found in RBILD.
Compared with RBILD, the lesions of DIP were more severe and
widespread. Both the patients responded favorably to glucocorticoids.
They were followed for more than three years. CONCLUSION: RBILD could
be confused with DIP in clinical manifestations, but can be separated
by open lung biopsy. Considering their similarities, these two lesions
may be regarded as a same disease entity.
DIP (desquamative
interstitial pneumonia): as a tobacco-associated disease -- case
report.
Rev Port Pneumol. 2004
Sep-Oct;10(5):431-5.
DIP (desquamative
interstitial pneumonia) is an interstitial lung disease with diffuse
and uniform accumulation of alveolar macrophages. There is a strong
association with tobacco since 90% of the patients are smokers. The
interstitial lung diseases related to tobacco are diverse and include
tumours, emphysema, chronic bronchitis, RBILD (Respiratory
Bronchilites associated Interstitial Lung Disease), DIP and Langerhans
Cell Histiocitosis. The authors present a case of DIP. A brief
theorycal revision and discussion of a case is made facing the
association with tobacco.
A case of
completely resolved pneumatocoeles in desquamative interstitial
pneumonia.
Respirology. 2003 Sep;8(3):389-95.
Desquamative
interstitial pneumonia (DIP), also known as alveolar macrophage
pneumonia (AMP), represents a subset of idiopathic interstitial
pneumonia that responds better to steroids and has a more favourable
prognosis than usual interstitial pneumonia. Recently, we encountered
a case of DIP with the formation of multiple pulmonary cysts during
corticosteroid maintenance treatment. After the introduction of
cyclophosphamide, the cysts gradually disappeared. This complete
resolution is believed to have resulted from the clearance of
check-valve-like bronchiolar obstructions that may be another
interesting terminal airway pathology in DIP.
BAL findings
in idiopathic nonspecific interstitial pneumonia and usual
interstitial pneumonia.
Eur Respir J. 2003
Aug;22(2):239-44.
Idiopathic
pulmonary fibrosis (IPF), which has the histological pattern of usual
interstitial pneumonia (UIP), is a progressive interstitial lung
disease with a poor prognosis. Idiopathic interstitial pneumonias with
a histological pattern of nonspecific interstitial pneumonia (NSIP)
have a better prognosis than UIP, and may present with a clinical
picture identical to IPF. The authors hypothesised that
bronchoalveolar lavage (BAL) findings may distinguish between UIP and
NSIP, and have prognostic value within disease subgroups. BAL findings
were studied retrospectively in 54 patients with histologically proven
(surgical biopsy) idiopathic UIP (n=35) or fibrotic NSIP (n=19), all
presenting clinically as IPF. These findings were also compared with
the BAL profile of patients with other categories of idiopathic
interstitial pneumonias. BAL total and differential cell counts did
not differ between the two groups. Survival was better in NSIP. In
neither group were BAL findings predictive of survival or changes in
lung function at 1 yr, even after adjustment for disease severity,
smoking and treatment. BAL differential counts in fibrotic NSIP
differed from respiratory bronchiolitis-associated interstitial lung
disease, but not from desquamative interstitial pneumonia or cellular
NSIP. The authors conclude that bronchoalveolar lavage findings do not
discriminate between usual interstitial pneumonia and nonspecific
interstitial pneumonia in patients presenting with clinical features
of idiopathic pulmonary fibrosis, and have no prognostic value, once
the distinction between the two has been made histologically. Desquamative
interstitial pneumonia--case report and review of literature.
Nihon Kokyuki Gakkai Zasshi.
2002 Feb;40(2):160-5.
A 50-year-old
man was admitted to our hospital because diffuse reticulonodular
shadows in the bilateral lung fields had deteriorated on chest
radiographs during a regular checkup. The bronchoalveolar lavage (BAL)
fluid revealed an increase in the total number of cells, including
slightly elevated levels of eosinophils and neutrophils. The
thoracoscopic lung biopsy specimens showed findings compatible with
desquamative interstitial pneumonia (DIP). Corticosteroid therapy was
done, and improvement of chest CT findings and pulmonary function were
seen. We reviewed the clinical features in seventeen patients with
biopsy-proven DIP reported in Japan. All patients have good prognoses
and a high frequency of steroid responsiveness. Moreover, many
patients were positive for antinuclear antibodies in serological
tests, and there was a tendency toward increasing eosinophils in BAL
fluids.
Desquamative interstitial pneumonia and respiratory bronchiolitis-associated
interstitial lung disease.Semin
Respir Crit Care Med. 2001
Aug;22(4):387-98.
Our understanding
of the various types and patterns of diffuse lung disease that might
result in fibrosis has evolved considerably over the last 50 years.
Many entities now regarded as distinct had been previously "lumped''
together as a single disease, "lung fibrosis,'' and more recently
misdiagnosed as idiopathic pulmonary fibrosis (IPF, synonymous with
cryptogenic fibrosing alveolitis). In 1965 desquamative interstitial
pneumonia (DIP) was first described, and later it was clearly
demonstrated that the clinical and pathological features of DIP and
IPF were different, particularly in terms of survival and response to
therapy. They are not part of the same disease spectrum nor does DIP
evolve into usual interstitial pneumonia (UIP). Later, in the
mid-1980s, RBILD was described as a distinct clinicopathologic
syndrome with features consistent with an interstitial lung disease
among current or former smokers. In the recent histopathological
classification of idiopathic interstitial pneumonia (IIP), DIP and
RBILD have been included as separate entities, although there is some
evidence that suggests they may lie at the two ends of a single
spectrum. The debate bears similarities with the debate about DIP and
UIP and is as yet unresolved. This article will give a broad and
current overview of these two rarer forms of IIP, including issues
that relate to diagnosis, imaging, histopathology, treatment, and
prognosis.
Respiratory
bronchiolitis, respiratory bronchiolitis-associated interstitial lung
disease, and desquamative interstitial pneumonia: different entities
or part of the spectrum of the same disease process?AJR
Am J Roentgenol. 1999 Dec;173(6):1617-22.
OBJECTIVE: Our
objective was to assess high-resolution CT findings of respiratory
bronchiolitis, respiratory bronchiolitis-associated interstitial lung
disease, and desquamative interstitial pneumonia and to determine
whether these three entities could be reliably differentiated by
radiologic criteria. MATERIALS AND METHODS: CT scans (1- to 3-mm
collimation) were reviewed in 40 patients with pathologically proven
respiratory bronchiolitis (n = 16), respiratory bronchiolitis-associated
interstitial lung disease (n = 8), or desquamative interstitial
pneumonia (n = 16). All patients with respiratory bronchiolitis and
respiratory bronchiolitis-associated interstitial lung disease were
cigarette smokers, and 85% of the patients with desquamative
interstitial pneumonia had a history of smoking. CT scans were
independently reviewed by two radiologists who assessed the pattern
and distribution of abnormalities. RESULTS: The predominant
abnormalities in respiratory bronchiolitis were centrilobular nodules
(12 [75%] of 16 patients) and ground-glass attenuation (six [38%] of
16). No single abnormality predominated in the respiratory
bronchiolitis-associated interstitial lung disease group; findings
included ground-glass attenuation (four [50%] of eight), centrilobular
nodules (three [38%] of eight), and mild fibrosis (two [25%] of
eight). All patients with desquamative interstitial pneumonia showed
ground-glass attenuation, and 10 (63%) of the 16 showed evidence of
fibrosis. CONCLUSION: The significant overlap between the CT findings
of respiratory bronchiolitis, respiratory bronchiolitis-associated
interstitial lung disease, and desquamative interstitial pneumonia is
consistent with the concept that they represent different degrees of
severity of small airway and parenchymal reaction to cigarette smoke.
Spontaneous
remission of desquamative interstitial pneumonia.Intern
Med. 1997 Oct;36(10):728-31
We report a case
of spontaneous remission of desquamative interstitial pneumonia (DIP)
in a 50-year-old male. The histological diagnosis of DIP was based on
open lung biopsy. A chest X-ray revealed reticulo-nodular shadows in
the bilateral lung fields, and the patient had mild dyspnea on
exertion. Without treatment, these shadows decreased gradually and
disappeared after several months. The patient recovered completely
within one year, and recurrence of the disease has not been observed
for 4 years. Recently, DIP has rarely been described, and the
spontaneous remission of DIP has not been reported since Carrington et
al in 1978 (1).
Fibrosing alveolitis and desquamative interstitial
pneumonitis.Pediatr
Pulmonol. 1994 Jun;17(6):359-65.
We report the
experience with and evaluation of treatment strategies in fibrosing
alveolitis and desquamative interstitial pneumonitis (FA/DIP) over the
last 16 years by a review of all cases referred to a tertiary referral
center. There were 25 cases, 16 boys and 9 girls (mean age at onset,
2.3 years; range, 7 days to 11.6 years). In each case the diagnosis
was confirmed by open lung biopsy at a mean age of 3.3 years (range, 7
weeks to 15.1 years). Presently features were tachypnea (19), cyanosis
(15), cough (12), exertional dyspnea (7), recurrent chest infections
+/- wheezing (9), and clubbing (8). Four patients recovered without
antiinflammatory medication. The others received specific treatment.
Of 11 patients given only prednisolone, six improved, two did not, and
three died despite treatment. Of five patients receiving only
chloroquine, four responded. Five patients received both prednisolone
and chloroquine; one died, two responded well. There was poor progress
in the remaining two. Of the 10 patients receiving chloroquine six
(60%) showed a good response. A younger presentation carried a worse
prognosis, but chest radiology at presentation and outcome were not
interrelated. Those with mild histological changes all survived, but
severe desquamation or fibrosis at biopsy was not related to outcome.
In four cases there was a family history (16%). Patients with FA/DIP
probably represent a disease spectrum of multiple etiology with a
variable prognosis and response to treatment.
Desquamative
interstitial pneumonia: a case presentation. Md
Med J. 1993 Nov;42(11):1119-22
A 25-year-old
African-American woman presented in the emergency room of a community
hospital complaining of shortness of breath. The patient was admitted
with a diagnosis of atypical pneumonia. Her respiration worsened
despite intravenous erythromycin, nebulized albuterol, 40% oxygen via
ventimask, and guaifenesin. An open lung biopsy revealed pulmonary
tissue showing interstitial fibrosis with lymphocytes and histiocytes
scattered within the fibrous tissue. Numerous alveoli contained
mononuclear cells as seen in desquamative interstitial pneumonia. The
patient did not respond to methylprednisolone and died on the
eighteenth hospital day.
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